CARDIO mksap 2019-2020 New ACP Guidlines

CARDIO mksap 2019-2020 New ACP Guidlines

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2. Welcome to the Cardiovascular Medicine
Sestion of MKSAP 191
In these pages, you will find updated information on risk factors for cardiovascular disease, diagnostic testing. coronary
artery disease, heart failure, arrhythmias, valvular heart disease, myocardial disease, pericardial disease. diseases ofthe aorta.
peripheral artery disease, and other clinical challenges. All of these topics are uniquely focused on the needs of generalists and
subspecialists outside of cardiologr.
MKSAP 19 strives to provide the clinical knowledge its learners need to navigate their longitudinal learning paths. MKSAP 19's
core content contains essential, newly researched inlormation in 11 subspecialty areas of internal medicine created by
dozens ol expert generalists and subspecialists. Development of MKSAP 19's syllabus and its 1200 all ne
r, peer reviewed.
psychometrically validated multiple choice questions (MCQs) has been informed by ABIM Certification and Maintenance of
Certification (MOC) requirements, emerging internal medicine knowledge, and our learners' feedback. MKSAP 19 contin
ues to include High Value Core (HVC) recommendations and MCQs, based on the concept of balancing clinical benefit with
costs and harms. Hospital based internists can continue to trust that MKSAP's comprehensive hospitalist content, integrated
throughout the syllabus, and hospitalist focused MCQs, specially designated with the blue hospitalist icon (E). continue to
align with the ABIM's Focused Practice in Hospital Medicine MOC exam blueprint and enhance Iearning for hospital-based
practitioners.
More than ever before, MKSAP 19 Digital focuses on individualized learning and convenience. In addition to custom quizzes
and interlinked questions and syllabus sections, MKSAP 19 Digital's new learning dashboard enables users to create a self
directed learning plan, with topic specific links to resources within MKSAP and ACP Online. Multimedia formats, including
whiteboard animations and clinical videos, will benefit our audiovisual learners, while MKSAP's Earn-as-You Go CME/MOC
feature now allows subscribers to earn CME/MOC as they answer individual questions. In addition to Extension Questions
and New Info Updates, MKSAP 19 Complete and Complete Green continue to offer Virtual Dx and Flashcards and now oiler
brand new enhancements: MKSAP Quick Qs, a set of concise questions mapped to high-frequency/high importance areas
of the ABIM blueprint mirroring boards-style MCQs, and an embedded digital version of Board Basics lor easy access
exam prep.
Language can be imprecise and imperfect, but MKSAP 19's Editors and contributors commit to using language and images
that support ACP's commitment to being an anti-racist organization that supports diversity, equity, and inclusion throughout
health care and health education. ACP also continues to ensure diversity among MKSAP's physician contributors. When
appropriate, the MKSAP Editors also rely on MKSAP 19 Digital's expanded use of multimedia enhancements, including video
and audio. to explore and more fully explain issues surrounding the presentation of MKSAP 19 clinical content as it relates to
race and ethnicity. MKSAP 19 users are encouraged to contact the Editors at mksap-editorspacponline.org to help us identify
opportunities for improvement in this area.
On behall ol the many internists and editorial staff who have helped us create our new edition, we are honored that you have
chosen to use MKSAP 19 to meet your lifelong learning needs.
Sincerely,
Davoren Chick, MD, FACP
Editor-in-Chief
Senior Vice President
Medical Education Division
American College of Physicians
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3. Cardiovascu lar Medicine
Committee
Andrew Wang, MD, Section Editor
Professor of Medicine
Vice Chief for Clinical Affairs
Division of Cardiologz
Duke University Medical Center
Durham. North Carolina
GregoryW Barsness, MD
Assistant Professor of Medicine
Consultant, Departments of Cardiovascular Diseases
and Radiologr
Mayo Clinic College of Medicine and Sciences
Rochester, Minnesota
R. Michael Benitez, MD
Professor of Medicine
Associate Chief (Clinical)
Division of Cardiovascular Medicine
University of Maryland School of Medicine
Baltimore, Maryland
Gerald S. Bloomfield, MD, MPH
Associate Professor of Medicine and Global Health
Division of Cardiologz
Duke University
Durham, North Carolina
HeidiM. Connolly, MD
Professor of Medicine
Department of Cardiovascular Diseases
Mayo Clinic College of Medicine and Sciences
Rochester, Minnesota
W. Schuyler Jones, MD
Associate Professor of Medicine
Division of Cardiologr
Duke University and Duke Clinical Research lnstitute
Durham, North Carolina
Stuart D. Russell, MD, EACP
Professor of Medicine
Regional Director of Heart Failure
Division of Cardiologr
Duke University School of Medicine
Durham, North Carolina
BenjaminA. Steinberg, MD, MHS
Assistant Professor of Medicine
Cardiovascular Medicine Division
University of Utah
Salt Lake City, Utah
Sreekanth Vemulapalli, MD
Director, Cardiac Diagnostic Unit and Echocardiography
Laboratories
Associate Director for Healthcare Delivery Transformation
Division of Cardiologz
Duke University Medical Center
Durham. North Carolina
Editor-in-Chief
Davoren Chick, MD, FACP
Senior Vice President, Medical Education
American College ol Physicians
Philadelphia. Pennsylvania
Senior Deputy Editor
Patrick C. Alguire, MD, FACP
American College of Physicians
Philadelphia, Pennsylvania
Deputy Editor
Richard S. Eisenstaedt, MD, MACP
Chair, Department of Medicine
Abington Hospital, Jefferson Health
Abington, Pennsylvania
Clinical Professor of Medicine
Sidney Kimmel Medical College at Thomas Jefferson
University
Philadelph ia. Pen nsylvan ia
Cardiovascular Medicine Reviewers
Ahmed Sami Abuzaid, MD, FACP, RPVI
Yousaf Ali, MD, MS
Floyd W. Burke, MD, FACP
Joseph P. Carrozza, ir., It4D, FACP
Benjamin S. Citrin, MD, FACP
Islam Y. Elgendy, MD, FACP
David L. Fischman, MD, FACP
D. R. Shanti Gunawardena, MD, PhD, FACP
Richard Hahn, MD, FACP
James Marsh, MD, FACP
Patrick G. o'Malley, MD, MPH, FACP
Kashif J. Piracha, MD, FACP
Harish Raj Seetha Rammohan, MD, MRCP, FACP
Rahul Sharma, MD, FACP
Saurabh Sharma, MD, FACP
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4. Each single MKSAP 19 self assessment question qualifies for
one quarter of a CME credit hour or ABIM MOC point.
MKSAP 19 Subscribers can enter their self assessment
question answers and submit for CME/MOC in two ways:
1. Users of MKSAP 19 Complete who prefer to use their
print books and a paper answer sheet to study and
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corresponding online answer sheets, which are avail
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may then submit their answers to qualify for CME cred-
its or MOC points (see below for information on Opting
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2. Users of MKSAP 19 Digital can enter their answers within
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The MOC Opt-in Form will be presented during a user's
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ABIM Maintenance of Certification
Successful completion of the CME activity, which includes
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ticipant to earn up to 300 medical knowledge MOC points
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It is the policy of the American College of Physicians (ACP)
to ensure balance, independence, objectivity, and scientific
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consistent with the policies of the ACP and the Accreditation
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To mitigate all conflicts of interest and influences of vested
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and lnclusion within MKSAP 19
MKSAP 19's Editors and contributors commit to using
Ianguage and images that support ACP's commitment to
being an anti racist organization that suppofts diversity.
equig, and inclusion throughout health care and health
education. ACP also continues to ensure diversity among
MKSAP's physician-contributors. When appropriate. the
MKSAP Editors will also rely on MKSAP 19 Digital's erpanded
use of multimedia enhancements, including video and audio,
to explore and more fully explain issues surounding the pre-
sentation of MKSAP 19 clinical content as it relates to race
and ethnicity. MKSAP 19 users are encouraged to contact
the Editors at mksap editors4aacponline.org to help us iden-
tiff opportunities for improvement in this area.
vl

5. Hospital-Based Medicine
For the convenience ofsubscribers who provide care in
hospital settings, comprehensive hospital-focused content
aligned with the ABIM Focused Practice in Hospital Medicine
trlueprint is integrated throughout the syllabus, and self
assessment questions that are specific to the hospital setting
are specially designated with the blue hospitalist icon (E).
High Value Care Key Points
Key Points in the text that relate to High Value Care
concepts (that is, concepts that discuss balancing clinical
benefit with costs and harms) are designated by the HVC
icon [HVC].
Educational Disclaimer
The editors and publisher of MKSAP 19 recognize that
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nities for error. Despite our best efforts, some errors may
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Readers are advised, however, to ensure that the recom
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MKSAP 19 ISBN: 978 1 938245 75 6
Cardiovascular Medicine ISBN : 978-1-938245 -84-8
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Errata and Revisions
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Table of Contents
Epidemiology and Risk Factors
Overview
Risk Factors for Cardiovascular Disease
Lifestyle
Dyslipidemia
Hypertension
Diabetes Mellitus
Obesiry and Metabolic Syndrome .
Inherited Fhctors
Ethnicity.
Calculating Cardiovascular Risk . . . . . .
Specific Risk Groups.
Women
Chronic Kidney Disease
Systemic lnflammation and HIV. .
Diagnostic Testing in Grdiology
Clinical History and Physical Examination . . .
Diagnostic Testing for Atherosclerotic
CoronaryArteryDisease . . . . .
Cardiac Stress Testing
Visualization of the Coronary Anatomy . .
CoronaryArtery Calcium Scoring. . . . . . .
Risks of Diagnostic Testing for Coronary
ArteryDisease.....
Diagnostic Testing for Structural Heart Disease
Diagnostic Testing for Cardiac Arrhythmias . .
CoronaryArtery Disease
Stable Angina Pectoris
Diagnosis and Evaluation. . . . .
General Approach to Treatment of Stable
Angina Pectoris
Coronary Revascularization. . .
Acute Coronary Syndromes
General Considerations. . . . . .
ST Elevation Myocardial I nfarction
Non-ST Elevation Acute Coronary Syndromes
Medical Therapy for Acute Coronary Syndromes
Acute Coronary Syndromes Not Associated
With Obstructive Coronary Artery Disease . . .
Care After an Acute Coronary Syndrome . . . . .
Management of Coronary Artery Disease
in Specific Populations.
Patients With Asymptomatic Vascular Disease
Patients Older Than 75 Years . .
Women
Patients With Diabetes Mellitus
Heart Failure
Pathophysiologz of Heart Failure . . . .30
Screening ..........30
Diagnosis and Evaluation of Heart Failure . . . . 30
Clinical Evaluation .....30
Diagnosis ......31
Evaluationforlschemia ........31
Classification ..........32
Management .......32
Heart Failure With Reduced Ejection Fraction . . . .32
Heart Failure With Preserved Ejection Fraction . . . 37
AcuteDecompensatedHeartFailure .....38
AdvancedRefractoryHeartFailure ......40
SpecificPopulations ....47
SpecificCardiomyopathies.. ........4l
TakotsuboCardiomyopathy... ......... 4l
AcuteMyocarditis.... .........41
GiantCellMyocarditis. .........42
Sarcoidosis .....42
Tachycardia-Mediated Cardiomyopathy. . . . . . . . . 42
Arrhythmias
Introduction.
Approach to the Patient With Bradycardia. . . . . . .
Clinical PresentationandEvaluation ...... .
SinusBradycardia...
Atrioventricular Block
Treatment.
Approach to the Patient With Tachycardia. . . . . . .
Clinical PresentationandEvaluation ...... .
Antiarrhythmic Drugs
SinusTachycardia...
Supraventricular Tachycardias. . . .
Clinical Presentation.
Atrioventricular Nodal Reentrant Tachycardia
Atrioventricular Reciprocating Tachycardia. .
Premature Atrial Contractions and
AtrialTachycardia...
Atrial Fibrillation. . .
Clinical Presentation.
Acute Management .
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8. Long TermManagement .......52
Management in Patients With Heart Failure . . . . .54
SubclinicalAtrialFibrillation... ........ 54
AtrialFlutter .......55
VentricularArrhythmias ....55
PrematureVentricularContractions ..... 55
VentricularTachycardia ........56
Inherited Syndromes Characterized by
SuddenCardiacDeath. .....57
SuddenCardiacArrest. .....59
EpidemiolograndRiskFactors .........59
AcuteManagement. ....59
Device Therapy for Prevention ofSudden Death. . . 61
Pericardial Effusion and Cardiac Tamponade
Pericardial Effusion.
Cardiac Tamponade
Constrictive Pericard itis
Clinical Presentation and Evaluation . . .
Management
Adult Congenital Heart Disease
Introduction. .......90
PatentForamenOvale. ......91
AtrialSeptalDefect. ........91
PathophysiologzandGenetics .......... 91
Clinical Presentation. ..........92
DiagnosticEvaluation ..........92
Treatment. .....92
Follow up After Atrial Septal Delect Closure . . . . .92
VentricularSeptal Defect ....94
Pathophysiolory .... ..........91
Clinical Presentation. ..........94
DiagnosticEvaluation ..........94
Treatment. .....94
Follow up After Ventricular Septal Defect Closure. . . 94
PatentDuctusArteriosus ....95
Pathophysiolory .... ..........95
Clinical Presentation. ..........95
DiagnosticEvaluation ..........95
Treatment. .....95
PulmonaryStenosis ........95
Pathophysiolory .... ..........95
Clinical Presentation. ... ...... .95
DiagnosticEvaluation ..........95
Treatment. .....95
Follow-up After Pulmonary Stenosis Repair . . . . .96
AorticCoarctation.. ........96
Pathophysiolory .... ..........96
ClinicalPresentation. ..........96
DiagnosticEvaluation ..........96
Treatment. .....96
Follow-up After Aortic Coarctation Repair. . . . . . . 97
TetraloryofFallot ..........97
Diagnostic Evaluation After Repair of
Tetralogrof Fallot ......98
Treatment of Tetralogz of Fallot Residua . . . . . . . . 98
Adults With Cyanotic Congenital Heart Disease . . . . . . 98
GeneralManagement ..........98
EisenmengerSyndrome ........98
Diseases of the Aorta
lntroduction.
Thoracic Aortic Aneurysm. . . . .
Screening and Surveillance
Treatment.
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101
B6
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Valvular Heart Disease
General Principles.
Aortic Stenosis. . . . .
Clinical Presentation and Evaluation
Management
Aortic Regurgitation .
Clinical Presentation and Evaluation
Management
Bicuspid Aorlic Valve Disease
MitralStenosis.....
Clinical Presentation and Evaluation
Management
Mitral Regurgitation .
Clinical Presentation and Evaluation
Management
Tricuspid Valve Disease
Prosthetic Valves .
Infective Endocarditis.
Diagnosis and Management. . . . . . .
Prophylaxis
Myocardial Disease
HypertrophicCardiomyopathy . . . . . . .. .
Clinical Presentation.
Evaluation
RiskStratification...
Management
Cardiac Amyloidosis
Restrictive Cardiomyopathy. . . .
Clinical Presentation and Evaluation
Management
Cardiac Tumors
Pericardial Disease
Acute Pericarditis . .
Clinical Presentation and Evaluation
Management
x
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9. Abdominal Aortic Aneurysm. . . .
Screening and Surveillance . . .
Treatment.
AorticAtheroma...
Acute Aortic Syndromes.
Pathophysiolory ....
Diagnosis and Evaluation. . . . .
Treatment.
Role of Genetic Testing and Family Screening
Peripheral Artery Disease
Epidemiologr and Screening
Clinical Presentation.
Evaluation
History and Physical Examination
Diagnostic Testing
Medical Therapy
Cardiovascular Risk Reduction . . .
Symptom Relief . .
Interventional Therapy .
Acute Limb Ischemia
Gdiovascular Disease in Gncer Survivorc
Cardiotoxicity of Radiation Therapy to the Thorax . . . .111
CardiotoxicityofChemotherapy.... ........112
Pregnancy and Cardiovascular Disease
Cardiovascular Changes During Pregnancy. . . . .
Prepregnancy Evaluation
Management of Cardiovascular Disease
During Pregnancy.
Peripartum Cardiomyopathy. . .
Other Cardiovascular Disorders . . .
Cardiovascular Medication Use
During Pregnancy.
Anticoagulation Therapy During Pregnancy
Bibliography
Self-Assessment Test.
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11. Cardiovascular Medicine
High Value Care Recommendations
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The American College of Physicians, in collaboration with
multiple other organizations, is engaged in a worldwide
initiative to promote the practice of High Value Care (HVC).
The goals of the HVC initiative are to improve health care
outcomes by providing care ofproven benefit and reduc
ing costs by avoiding unnecessary and even harmful inter-
ventions. The initiative comprises several programs that
integrate the important concept of health care value (bal
ancing clinical benefit with costs and harms) for a given
intervention into a broad range of educational materials
to address the needs oftrainees, practicing physicians,
and patients.
F{VC content has been integrated into MKSAP 19 in sev-
eral important ways. MKSAP 19 includes HVC identified
key points in the text, HVC focused multiple-choice ques
tions, and, in MKSAP Digital, an HVC custom quiz. From
the text and questions, we have generated the following
list of HVC recommendations that meet the definition
below of high value care and bring us closer to our goal
of improving patient outcomes while conserving finite
resources.
High Value Care Recommendation: A recommendation to
choose diagnostic and management strategies for patients
in specific clinical situations that balance clinical benefit
with cost and harms with the goal of improving patient
outcomes.
Below are the High Value Care Recommendations for the
Cardiovascular Medicine section of MKSAP 19.
o Cardiac stress testing is not routinely recommended in
asymptomatic patients with diabetes mellitus to detect
subclinical coronary artery disease.
o With few exceptions, fasting and nonfasting total cho-
lesterol and HDL cholesterol levels have fairly similar
prognostic value and association with cardiovascular
outcomes (see Item 19).
r No study has demonstrated the benefit of intensifying
lipid management, such as with the initiation of ezeti-
mibe, when LDL cholesterol level is lower than 70 mgldL
(t.Bt mmol/L) (see Item 21).
o There is no role for dual antiplatelet therapy with aspirin
and clopidogrel in patients with chronic stable angina in
the absence ofrevascularization (see Item 41).
. For patients with new-onset heart failure, directly initiat-
ing valsartan-sacubitril rather than a pretreatment period
with an ACE inhibitor or angiotensin receptor blocker, is
a safe and effective stratery in patients with heart failure
with reduced ejection fraction (see Item 1).
o Current guidelines recommend against routine surveil-
lance echocardiography in patients with chronic heart
failure in the absence of a change in clinical status or
planned intervention.
r Serial B-Wpe natriuretic peptide measurements should not
be used to guide care of patients with chronic heart failure.
o Routine invasive pulmonary artery catheterization for
hemodynamic monitoring is not recommended in patients
with decompensated heart failure.
. Two key elements are associated with a successful transi-
tion to home following hospitalization for heart failure: a
follow-up phone call within 2 to 3 days of discharge and
an office visit within 7 to 14 days of hospital discharge
(see ltem 52).
. [n patients with atrial fibrillation, rivaroxaban is nonin
ferior to warfarin in the prevention of stroke or systemic
embolism and is associated with less intracranial and
fatal bleeding (see Item 11).
o Premature ventricular contractions (PVCs) without high-
risk features (syncope, family history of premature sud-
den cardiac death, structural heart disease) are managed
with reassurance; treatment is reserved for bothersome
symptoms or frequent PVCs.
o Device pocket infection is a clinical diagnosis; aspiration
of a cardiac implantable device pocket should never be
performed for diagnostic purposes (see ltem 64).
. In patients with valvular heart disease who are at high
risk for infective endocarditis, antibiotic prophylaxis is
not recommended for nondental procedures, such as
transesophageal echocardiography, esophagogastroduo -
denoscopy, colonoscopy, or cystoscopy, in the absence of
active infection (see Item 108).
. In patients with suspected infective endocarditis (lE),
transthoracic echocardiography (TTE) is recommended
as the initial imaging study in most clinical situations;
in all patients with known or suspected IE and nondi-
agnostic TTE results, or if complications have developed
or are clinically suspected or if intracardiac device leads
are present, transesophageal echocardiography is recom
mended (see Item 18).
. Genetic testing is not indicated in first-degree relatives
of patients with hypertrophic cardiomyopathy unless a
pathogenic genetic variant is identified in the index patient.
r No treatment or follow-up is needed in asymptomatic
patients with a patent foramen ovale.
t
L
\
1
xltl

12. . In patients with peripheral artery disease and intermit-
tent claudication, supervised exercise training is the most
effective treatment for improving maximal walking dis-
tance and pain-free walking distance (see Item 86).
. There is no evidence to support the use ofdual antiplate-
let therapy with aspirin and clopidogrel over antiplatelet
monotherapy in patients with peripheral artery disease
(see Item 3).
o Neither pentoxifflline nor chelation therapy with ethy-
lenediaminetetraacetic acid has any benefit for the
treatment of symptomatic peripheral artery disease
(see Item 86).
!
,|
i
l
I
l
,
1
'l
xtv

13. Ca rd iovascu la r Med ici ne
Epidemiology and
Risk Factors
Overuiew
Cardiovascular disease (CVD) encompasses many conditions,
including coronary artery disease (CAD), stroke, congenital
heart disease, rhythm disorders, subclinical atherosclerosis,
heart failure, valvular disease, venous disease, and peripheral
artery disease. CVD remains the leading cause of death in the
United States; however, fiom 2004 to 2016, the age adjusted
death rate fbr CVD fell by 22'l.. Despite this improvement, CVD
was responsible lbr nearly 31'/, ol all deaths in the United
States in 2016. Globally, a third of all deaths were attributed to
CVD in 2015.
Nearly 507, of U.S. adults currently have some form of
CVD, including hypertension. Pre'u'alence increases with age,
:rnd more than 75'l, of persons aged 60 to 79 years have CVD.
Lifetime risk for CVD is estimated to be one in three for
women and two in three for men according to data from the
Framingham Heart Study.
CVD was responsible for 4.8 million U.S. hospital dis
charges in 2014. The estimated total cost of CVD fbr 2014 to
2015 was approxin.rately $350 billion. The projected total cost
of CVD between 2015 and 2035 is estimated to remain stable
lor most persons but to increase sharply for adults aged
65 years or older.
An estimated 6.2 million U.S. adults older than 20 years
l.rave a diagnosis of heart lailure, a final common pathway for
many cardiovascular conditions. The prevalence of'heart fail
ure is projected to increase by 46"/,, between 2012 and 2030.
Risk Factors for
Ca rd iovascu la r Disease
Lifestyle
The selen metrics of ideal cardiovascular health are optimal
lipid, btood pressure, and glucose levels; healthy dieti appro
priate energy intake; physical activity; and avoidance of
tobacco; however. very few people meet these metrics.
Promotion of a healthy lifestyle throughout life remains the
most inlportant way to prevent atherosclerotic CVD.
Sedentary lifestyle, poor diet, and obesity all contribute
to increased cardiovascular risk. According to the CDC, only
22.5'X, of aclults meet aerobic and strengthening recommen
datior.rs. Average daily lruit and vegetable consumption in
the United States is less than 1 cup of fruit (recommended,
1.5 2 cups) and less than 1.5 cups ot vegetables (recommended,
2-3 cups). The National Diabetes Prevention Program found
that in persons at high risk for diabetes mellitus, interventions
such as changes in diet, exercise, and weight loss of 5'7, to 7'1,
reduced the risk for developing diabetes by 58'2, but did not
reduce CVD events.
Tobacco use is the leading preventable cause of disease,
disability, and death in the United States. and tobacco expo
sure is a significant risk factor for CVD. The prevalence of
tobacco use continues to decliner 16.7"/,' of men and 13.67, of
women were current smokers in 2015. The risk lor heart fail
ure and death fbr most former smokers is similar to that of'
never smokers alter greater than 15 years of tobacco cessa
tion. Smoking status should be assessed at every visit, and
cessation counseling and pharmacologic therapy should be
offered to active smokers (see I\4KSAP 19 General Internal
Medicine 1).
Psychosocial factors, including depression, anger, and
anxiety, are associated with worse cardiclvascular outcomes.
Depression has been linked with higher risk for cardiovascular
events. Psychosocial stressors also affect the course of'treat
ment and adherence to a healthy lifestyle after an event.
Although it is important to detect and treat these disorders,
there is no evidence that such detection and treatment impacts
cardiovascular risk itself.
Dyslipidemia
Twelve percent of adults older than age 20 years have total
cholesterol levels greater than 240 mgldL (6.22 mmol/L).
Elevated semm cholesterol levels are associated with increased
cardiovascular risk, and reducing cholesterol le'uels has been
shown to lower overall risk. Elevated LDL cholesterol and low
HDL cholesterol levels also are independently associated witlr
increased risk fbr CVD. Lipid lowering therapies lor primary
and secondary prevention of CVD are discussed in MKSAP 19
General lnternal Medicine 1.
Hypertension
Hypertension (blood pressure >130/80 mm Hg) alfects approx-
imately 46% of persons aged 20 years or older. Before age
45 years, the prevalence of hypertension is higher in men than
in womenr however, after age 65 years, the prevalence is higher
in women. The population attributable risk of hypertension for
stroke is 17.9"1,. Treatment of hypertension reduces risk for
stroke, heart failure, and kidney disease. Early recognition and
effective management of hypertension could reduce CVD mor
tality by 30.4% among men and 38.0'X, among women.
1

14. Epidemiology and Risk Factors
Diabetes Mellitus
Diabetes increases cardiovascular risk and is an independent
indication for statin therapy. Sixty eight percent of individu
als with diabetes eventually die of heart disease. Diabetes
increases ischemic stroke incidence at all ages. but this risk
is most prominent (risk ratio >5) before age 65 years. In per
sons with diabetes, CAD is likely to be more extensive. and
the incidence of multivessel disease is increased. patients
with diabetes also have worse outcomes when hospitalized
for any CVD.
Obesity and Metabolic Syndrome
In 2016, 39.6% of U.S. adults had class I obesity (BMI, 30 35).
and7.7"/o had class III obesity (BMI, >40). Obesity may increase
the risk for CVD events. even in the absence of n.retabolic risk
factors.
Metabolic syndrome is characterized by the presence of at
Ieast three of the following conditions: elevated glucose level,
central obesity, low HDL cholesterol level. elevated triglyceride
level, and elevated blood pressure. The hallmark feature of
metabolic syndrome is glucose intolerance. Approximately
34"1, of adults meet the criteria for metabolic syndrome. The
highest prevalence is among Hispanic and Latino persons
(34'7,). Metabolic syndrome is associated with increased risk
for CVD and diabetes, with risk increasing as the number of
component conditions increases.
lnherited Factors
In addition to the risk for CVD as predicted by the traditional
risk elements, inherited factors may increase the risk
for CVD. A history of premature CAD (males younger than
55 years, females younger than 65 years) in parents doubles
the risk fbr myocardial infarction (MI) in men and increases
risk in women by 7O"/,,. Stroke in a first-degree relative
increases risk for stroke by 50'/.. A parental history of atrial
fibrillation increases odds of this condition by B0'){,. In addi
tion to the shared environment (i.e., lifestyle), genetics may
contribute to increased risk in family members, although no
genetic profile has been shown to explain a significant per
centage of CVD incidence.
Ethnicity
There are significant racial and ethnic disparities in the risk
and prevalence for CVD in the United States. The prevalence of
CVD is highest among American Indian or Alaska Native
(74.6y.\, white (11.5",{,), Black (10.0'U,), and Asian (7.791,) races.
Those with Hispanic or Latino ethnicity have a lower preva
Ience of CVD (8.2%) than non llispanic persons (11.7"1,).
Prevalence of hypertension is highest among non Hispanic
Black men (58.6')(,) and lowest among Asian women (So.q'L).
The risk for diabetes is highest among American Indians and
Alaska Natives (15.1'l.).
GIobally, the prevalence ofcardiovascular risk factors and
subsequent CVD is increasing because of changes in tobacco
use and eating habits, lifestyle, and social lactors. contributing
to a dramatic increase in obesitv and diabetes.
I(EY POIXI
. Beyond age and gender, risk for cardiovascular disease
is strongly attributed to modifiable risk factors. includ
ing dyslipidemia, smoking, diabetes mellitus. hyperten
sion, obesity, inadequate exercise, and poor diet.
Calculating Cardiovascular Risk
Cardiovascular risk scores can be used to assess an individual
patient's risk for major cardiovascular events and to identify
preventive interventions. Traditionally the Framingham risk
score. r.thich includes the data inputs of age. sl,stolic blood
pressure. total cholesterol level, HDL cholesterol level. smok
ing status, and presence ofdiabetes. has been used to estimate
the 10 year risk for a major coronary heart disease event
(MI or coronary death). The Reynolds risk score (http: uu'u,.
reynoldsriskscore.org/) is a sex-specific calculator and
includes family history and high sensitivity C reactive protein
Ievel. Another tool, the Multi Ethnic Stud)' of Atherosclerosis
(MESA) risk score (https:irr.lrvn:mesa nhlbi.org CAC Tools.
aspx), uses coronary artery calcium score and traditional risk
factors to calculate risk.
The Americar.r College of Cardiology American Heart
Association (ACC/AHA) Pooled Cohort Equations (PCE) is a
risk assessment instrument derived from several diverse
community based cohorts. The ACC/AHA CVD risk calculator
based on the PCE (https:irtools.acc.org ASCVD Risk Estimator
Plus) may be used to calculate 10-year atherosclerotic CVD risk.
thereby identifying persons u,ho would benefit lrom preven
tive measures. including statin therapy The PCE is the single
most robust tool for estimating 10 year risk in U.S. adults aged
40 to 75 years and is recommended by the U.S. Preventive
Services Task Force and other organizations to assess athero
sclerotic CVD risk.
According to the ACCIAHA. in patients with borderline
(5'x, to <7.5'7,) or intermediate (>7.5')(, to <20'l,) l0-year athero
sclerotic CVD risk based on the PCE. it is reasonable to further
refine individual risk based on the presence of risk enhancing
f.actors. such as chronic kidney disease (CKD). selected testing
(e.g., high-sensitivity C reactive protein level. ankle brachial
index), and lamily history of premature CAD. The U.S.
Preventive Services Task Force concluded that there is insuf-
ficient evidence to recommend using nontraditional risk fac
tors fbr cardiovascular risk calculation.
I(EY POIIIT
. Validated risk prediction tools, such as the Pooled
Cohort Equations, can be used to identiff persons at
risk for the development of cardiovascular disease r,r,ho
would benefit from preventive measures, including
statin therapy.
2

15. 5
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Specific Risk Groups
Women
Hyperlipiden.ria, type 2 diabetes. obesity, and tobacco use con
ler greater risk for CAD in women compared with men.
Beyond those more traditional risk factors. some prior obstet
ric complications, including preeclampsia, gestational hyper
tension or diabetes, and preternr or lort, birth weight delivery
increase CVD risk in women. CVD risk is also increased in
women with early menopause or polycystic ovary syndrome.
CVD remains the leading cause of death in women, result-
ing in more deaths than those caused by cancer. diabetes, and
kidney disease combined. Approximately two thirds of women
who clie of MI are asymptonlatic or have symptoms unrecog
nized as cardiac in origin. Chest pain is the most common
presenting anginal symptom in both men and women, but
wonren are more likely to report chest pain that is nonexer
tional, occurs during sleep, or is induced by mental stress. In
patients with MI, chest pain is reported less frequently by
women than by men. ln addition, :rfter an acute coronary
syndrome, women undergo f'ewer interventions, have more
con.rplications. and have higher unadjusted mortality. Mortality
within the first year after a first I\41 is 23% in women versus
18'7, in men. Acute MI mortality is highest among Black
\{,omen. Black women also have a higher prevalence of CVD
(10..5'){,) compared with White women (s.l'I,) and Hispanic
romen (8.0'7,). Despite this illness burden, women have been
underrepresented in clinical treatment trials, making it chal-
lenging to extrapolate treatment decisions.
Chronic Kidney Disease
CKD is associated with higher incidence of CVD and worse
cardiovascular outconles. In persons with CKD versus without
CKI). the incidence rate (per 1000 person years) for CAD is
24.5 versus 8.4 and fbr stroke is 13.4 versus 4.8. The risk for
CVD related death is 5 to 30 times higher in patients under
going dialysis than in those with similar risk factors and pre
served kidney function.
Systemic lnflammation and HIV
The risk for CVD is higher in patients with systemic inflamma.
tory conditions, such as systemic lupus erythematosus and
rheumatoid arthritis. The risk tbr CAD is nearly 607, higher in
patients with rheun.ratoid arthritis and is nearly doubled in
patients with systemic lupus erythematosus. The increased
risk in these patients may be a result of the inflammatory
process, a prothrombotic state, insulin resistance, and use of
glucocorticoids or other immunosuppressive therapy in addi
tion to traditional cardiovascular risk factors.
Certain HIV related risk enhancing factors may cor.rf'er a
1.5 to 2 times higher risk fbr CVD than the calculated risk
using the ACC/AHA CVD risk calculator. HIV risk enhancing
factors include history of prolonged HIV virernia and/or delay
in antiretroviral therapy initiation, low current or nadir
CD4 cell count (<350/pL), and HIV treatment failure or
Diagnostic Testing in CardiologY
nonadherence. Patients with well controlled HIV (undetecta-
ble viral load, normal CD4 cell count) may still be at increased
risk. especially in the setting of concomitant metabolic syn
drome, lipodystrophyrlipoatrophy, fatty Iiver disease, or hepa
titis C virus co infection; this increased risk may be related
to the effects of antiretroviral therapy. Patients with HIV
risk enhancing factors should be considered for intensive
therapeutic lifestyle modifi cations, lipid-lowering therapy, or
referral to a specialist.
Diagnostic Testing
in Cardiology
Clinical History and
Physical Examination
The diagnostic cornerstone of cardiovascular disease is the
clinical history and physical examination. A careful history
that includes symptom characteristics, timing, and duration;
factors that exacerbate or relieve symptoms; and functional
capaci$/ is critical to ensuring a focused and appropriate diag
nostic evaluation. Abnormal findings on the cardiovascular
examination also may raise suspicion for specific cardiac con
ditions and guide test selection.
Cardiovascular testing can provide both diagnostic and
prognostic infbrmation, and its use should be guided by symp
ton.rs, the pretest likelihood of disease, whether testing results
will alter patient management, and shared decision making
with patients.
Diagnostic Testing for
Atherosclerotic Corona ry
Artery Disease
Diagnostic testing for coronary artery disease (CAD) can be
categorized as providing functional and/or anatomic evidence
of:rtherosclerotic burden. Functional studies reveal the pres
ence of ischemia (exercise ECG, single photon emission CT
[SPECT], PET), the extent and severity of ischemia (SPECT,
PET), information on coronary blood llow (PE't fractional flow
reserve [FFR]-CT), and developmer.rt of wall motion abnor
malities (echocardiography, cardiac magnetic resonance
[CMR] imaging). Anatomic infbrmation is obtained from inva
sive angiography, coronary CT angiography (CTA), and coro
nary artery calcium (CAC) scoring. Cardiac diagnostic testing
modalities are summarized in Table 1.
Cardiac Stress Testing
Cardiac stress testing is commonly performed to stratify risk in
those with or suspected of having CAD. Appropriate, cost
efl'ective stress testing is based on the history. examination
findings, and pretest probability of CAD (see Coronary Artery
Disease). The pretest probability takes into account age, sex,
3

16. Diagnostic Testing in Cardiology
TABLE 1, Diagnostic Testing for Coronary Artery Disease
Diagnostic Test Utility
Exercise Stress Testing
Exercise ECG
Advantages
Data acquired on exercise
capacity, blood pressure and
heart rate response, and
provoked symptoms
Exercise data acquired along
with wall motion images to
assess for ischemia
Allows evaluation of valve
function and pulmonary
pressures
Relatively portable and less costly
than nuclear protocols
Entire study is completed in <1 h
Gating (image acquisition
coordinated with the cardiac
cycle); use o{ higher-energy
agents, such as technetium; and
techniques used to correct for
attenuation provide improved
specificity
Late reperfusion imaging allows
evaluation of myocardial viability
if thallium is used
Limitations
Not usefulwhen baseline ECG is
abnormal (LVH, LBBB, paced
rhythm, preexcitation, >1 -mm
ST-segment depression)
lmage quality is suboptimal in
some patients but can be
improved with microbubble
contrast
lmage interpretation is difficult
when baseline wall motion
abnormalities are present
Diagnostic accuracy decreases
with single-vessel disease or
delayed stress image acquisition
Attenuation artifacts can be
caused by breast tissue or
diaphragm interference;
attenuation correction and
software programs can improve
image interpretation
Radiation exposure
Contraindications are severe
baseline hypertension, unstable
angina, severe tachyarrhythmias,
hypertrophic cardiomyopathy,
severe aortic stenosis, and large
aortic aneurysm
Contraindications a re active
bronchospastic airway disease,
theophylline use, sick sinus
syndrome, hypotension, and high-
degree AV block
Theophylline and caffeine must be
withheld 12-24 h bef ore the test
Adenosine or dipyridamole may
cause chest pain, dyspnea, or
flushing
Radiation exposure
Contraindications are severe
baseline hypertension, unstable
angina, severe tachyarrhythmias,
hypertrophic card iomyopathy,
severe aortic stenosis, and large
aortic aneurysm
Radiation exposure
lnitial diagnostic test in most
patients suspected of having
CAD
Recommended when baseline
ECG findings are abnormal or
when information on a particular
area of myocardium at risk is
needed
Recommended when baseline
ECG findings are abnormalor
when information on a particular
area of myocardium at risk is
needed
With LBBB, conduction delay in
the septum may cause false-
positive abnormalities;
vasodilator stress can improve
the accuracy of perfusion
imaging
Stress
echocardiog raphy
SPECT myocardial
perfusion imaging
t
Pharmacologic Stress Testing
Dobutamine
echocardiography
Vasod ilator myoca rdial
perfusion imaging
(adenosi ne,
dipyridamole,
regadenoson)
Dobutamine
myocardial perfusion
imaging
Recommended in patients who
cannot exercise or when
information on an area of
myocardium at risk is needed
Recommended in patients who
cannot exercise
Minimizes septal abnormalities
frequently seen with myocardial
perfusion imaging in patients
with LBBB
Recommended in patients who
cannot exercise and have
contraindications to vasodilator
stress
Recommended when
information on an area of
myocardium at risk is needed
Because the patient is supine,
images are acquired
continuously, allowing the test to
be stopped as soon as ischemia
is evident
Stepwise increases in
dobutamine dose allow staged
assessment o{ wall motion
abnormalities
Vasodilator stress testing may
minimize effect of B-blockade
on
perfusion defect size
lmaging with vasodilator stress
can be performed sooner after
myocardial infarction
Has sensitivity and specificity
similar to those of exercise or
vasodilator perfusion imaging for
diagnosis of myocardial ischemia
(Continued on the ne* page)
4

17. Diagnostic Testing in CardiologY
Other Tests
TABLE 1 . Diagnostic Testing for Coronary Artery Disease (Continued)
Diagnostic Test utility Advantages
Study duration is shorter and
radiation dose is lowerthan with
conventional myocardial
perfusion imaging
Absolute myocardial blood flow
can be measured
Can be combined with CAC
scori ng
Accurate test for myocardial
ischemia or viability
Can be combined with functional
assessment of coronary stenosis
(FFR, IFR)
Percutaneous revascularization
can be performed after
diagnostic study
CAC scores are predictive of
cardiovascular risk in selected
patients
Coronary artery vessel lumen and
atherosclerotic lesions can be
visualized in detail
Limitations
Not widely available
More expensive than other
imaging modalities
Used with pharmacologic stress
only (no exercise protocol)
Radiation exposure
Some patients experience
claustrophobia
May be contraindicated in
with an older pacemaker, I
other implanted device
Certain gadolinium-based
contrast agents are
contraindicated in patients with
CKD"
Sinus rhythm and a slower heart
rate are needed for improved
image quality
Limited avail and
lnvasive
Risks associated with vascular access
and radiocontrast exposure (kidney
dysfunaion, allergy, bleeding)
Radiation exposure
Does not provide data on
coronary luminal narrowing
Radiation exposure
Requires high-resolution (64-slice)
CT instruments
Does not provide detailed images
of distal vessel anatomy
Catheterization will be needed if
intervention is planned
Ability to quantify lesion severity
can be limited by significant
calcification or presence of
coronary stent
Radiation and radiocontrast
exposure
PET/CT
Dobutamine or
adenosine CMR
imaging
Coronary angiography
CAC scoring
Coronary CT
angiography
Provides best perfusion images
in patients with increased BMI
Provides data on myocardial
perfusion, function, and viability
Provides excellent spatial
resolution for visualization of wall
motion abnormalities during
dobutamine infusion
ldentifies perfusion abnormalities
during adenosine infusion with
gadolinium as contrast agent
Provides data on infarction and
viability using gadolinium
contrast
ldentifies anomalous coronary
artery origin
Provides anatomic diagnosis of
the presence and severity of
CAD
Allows for evaluation of coronary
anatomy
May inform preventive treatment
decisions for patients with
intermediate or borderline 10-y
risk for cardiovascular events
Useful for selected patients with
intermediate risk for CAD
ldentifies anomalous coronary
a rteries
patients
CD, or
AV=atrioventricular;CAC=coronaryarterycacium;CAD=coronaryarterydisease;CKD=chronickidneydisease;CMR=cardiacmagneticresonance;FFR=fractionalflow
emission CT.
"Group
I gadolinium based contrast agents are contraindicated in patients with end stage kidney disease or estimated glomerular filtration rate less than 30 mUmin/1.73 m'.
Group ll gadolinium-based contrast agents are not contraindicated in patients with CKD. There are insufficient data to make a recommendation for group lll gadolinium-based
contrast agents in patients with CKD.
and symptoms. Stress testing is most effectively used in
patients with an intermediate pretest probability of CAD (10%
to 90'7,), in whom a positive test result significantly increases
disease likelihood and a negative test result significantly
decreases likelihood. Stress testing in persons with a low
likelihood of disease, such as young patients with atypical
symptoms, often yields false positive results, potentially
resulting in unnecessary testing, inaccurate diagnoses, and
harms. In patients with a high pretest probabiliry invasive
angiography rather than stress testing is appropriate.
Assessment of the patient's ability to exercise is important
in determining the most appropriate stress test. Exercise ECG
5

18. Diagnostic Testing in Cardiology
is the initial test of choice in patients with normal findings on
baseline ECG. If there are baseline ECG abnormalities (e.g.,
ST segment depression >1 mm. Ieft bundle branch block. left
ventricular hypertrophy. paced rhythnl, or preexcitation).
ST segment changes induced u/ith exercise are not specific fbr
CAD. Stress testing with adjunctive imaging or anatomic
assessment with coronary CTA is indicated in these instances.
The decision to \,\,ithhold cardiac medications. such as
nitrates and p blockers, before stress testing should be indi
vidualized. In patients who are undergoing exercise stress
testing to diagnose CAD, cardiac medications that impair heart
rate response (p btockers) should be withheld fbr at least
2,1 hours before testir.rg because these agents may lead to an
inadequate peak heart rate. If the purpose of the stress test is
to evaluate symptoms, determine efficacy oltherapy, or deter
mine prognosis in a patient with kno 11 CAD, then patients
should continue their cardioactive medication regimen.
Exercise ECG
Stress testing to evaluate for CAD should always be performed
with exercise unless exercise is contraindicated or the patient
is unable. Exercise stress testing protocols use treadmill or
bicycle ergometry to increase workload in a stepr,r,ise manner.
which allows adequate time fbr development of maximal
metabolic demand. A standard Bruce protocol increases the
speed and grade of the treadmill e."ery 3 minutes. Achieving
B5'1, of the age predicted maximal heart rate is adequate for
identifying obstructive CAD; honever. patients should exer
cise until limited by symptoms. Because heart rate and blood
pressure are the major determinants of myocardial oxygen
demand, achieving a rate pressure product (heart rate x sys
tolic blood pressure) of at least 25,000 is considered an ade
quate workload. Stress testing should be terminated when the
BASELINE
EXERCISE STAGE I
0O:0O 1.0
49 tfo ST @ l0ooa\'
8085 po!r,
4
0.?
4.1
1.6
0,
patient has exerted maximal effort, requests to stop. or expe
riences significant symptoms (angina or light headedness).
The test also should be stopped for exertional hypotension,
significant hypertension (>200i 110 mnr Hg). ST-segment ele
vation or significant ST-segment depression. or significant
arrhythmias.
Ischemia is identified on exercise ECG b1' the develop
ment of horizontal or downsloping ST segment depression of
at least 1 nrm occurring 80 milliseconds after the J poirrt
(Figure l). ST segment depression cannot localize ischemia or
identi['the culprit coronary artery. Exercise induced h1'po
tension or absence ofblood pressure augmentation can indi
cate signifi cant obstructive disease.
Erercise stress testing additionalll' provides prognostic
value in patients with known or suspected CAD. Exercise
capacit)'is a pouerful predictor of,outcomes: indir,iduals una
ble to achieve 5 metabolic equivalents. or the first stage of a
Bruce protocol, have higher all-cause mortaliR: Heart rate
recovery after cessation ofexercise pror,ides incremental prog
nostic information. A heart rate drop of less than 12 min in the
first minute after exercise termination is associated r,r,ith
higher mortality. Information obtained from erercise stress
testing also may be used in risk prediction models. The Duke
Treadmill Score incorporates duration of exercise. de'u'elop-
ment of symptoms. and degree of ST segment depression
to calculate 5-year all cause mortalin in patients \vithout
known CAD.
Stress Testing With Adjunctive Imaging
ln patients with obstructive CAD. reduced blood flow and
myocardial ischemia result in a progression of myocardial
abnormalities, termed the ischemic cascade. Initiallll ischemia
induces changes in perfusion. follor,r,ed b1' diastolic and (at a
MA.Y ST
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tIGURE 1. ECGrecordedatbaseline(/eft) andduring(rght) exercisestresstesting.Thepresenceof 2mmdownslopingST-segmentdepressionsinleadsl, ll, Ill,andaVF
and leads V3 through Vu during exercise is diagnostic of ischemia but does not localize specific vascular distribution.
6

19. Diagnostic Testing in Cardiology
Stress SPECT
At Rest After Stressor tnterpretation
Normal
Stress-induced
myocardial ischemia
lnfarct or hibernating
myocardium
Small or no distinct zone
of ischemia, possible
balanced ischemia or
multivessel CAD
Normal
Normal
Perfusion
defea
Normal
Normal
Perfusion defect
Same perfusion
defect
LV dilation
Stress Echocardiography
At Rest After Stressor lnterpretation
Normal
Wall motion
abnormality
Same regional
wall motion
abnormalities
LV dilation
Normal
Stress-induced
myocardial ischemia
lnfarct or hibernating
myocardium
Small or no distinct zone
of ischemia, possible
balanced ischemia or
multivessel CAD
Normal
Normal
Regional wall
motion
abnormalities
Normal
TABLE 2. lnterpretation of Stress Testing With
lmaging Results
CAD = coronary artery disease; LV = left ventricular; SPECT = single photon
emission Cl
later stage) systolic dysfunction, ECG changes, and lastly,
angina. The addition of imaging studies to stress testing
increases diagnostic sensitivity by detecting earlier signs of
and localizing ischemia.
Stress testing with imaging is indicated in patients with
an inability to exercise, baseline ECG abnormalities that limit
interpretation of the exercise ECG, or indeterminate findings
on the exercise ECG. Depending on the modality used, stress
testing with imaging compares wall motion, perfusion, and/
or metabolism at baseline and after stress (Table 2). Imaging
with SPECT, PET, or CMR can be used to detect reduced myo-
cardial perfusion as early evidence of ischemia. Systolic dys-
function, indicated by wall motion abnormalities during
stress, can be detected by echocardiography or CMR imaging.
Imaging choice should consider characteristics of the patient
and modalify as well as local availabilily and expertise (see
Table 1).
Exercise is preferred over pharmacologic stressors.
Dobutamine, like exercise, increases myocardial oxygen
demand and elicits ischemia because of insufficient perfusion
to the affected myocardium. Vasodilators, such as dipyrida-
mole, regadenoson, and adenosine, produce hyperemia and a
flow disparity between myocardium supplied by unobstructed
vessels and myocardium supplied by stenotic vessels because
of the inability of the distal vasculature to dilate further. In
patients with left bundle branch block undergoing myocardial
perfusion imaging, vasodilator induced stress is preferred to
exercise or dobutamine because of the potential for false
positive septal perfusion abnormalities.
Stress Echoc ardiographA
Exercise stress echocardiography provides information on
ischemia, hemodynamic significance of valvular abnormali-
ties, and pulmonary pressures during exercise. Exercise is
performed with supine or upright bicycle ergometry which
allows for continuous imaging, or with a treadmill protocol,
which requires acquisition of poststress images within
90 seconds. The development of new wall motion abnormali-
ties indicates ischemia in the visualized territory. Resting wall
motion abnormalities that do not improve at peak exercise
may indicate infarcted or hibernating myocardium (chronic
but potentially reversible ischemic dysfunction).
With pharmacologic stress echocardiography, dobu
tamine is progressively infused (up to 40 pg/kg/min) to achieve
85% of age predicted maximal heart rate. Atropine is adminis-
tered ifthe target heart rate is not achieved. The sensitivity of
stress echocardiography may be reduced in the setting of
baseline wall motion abnormalities, systolic dysfunction,
or single vessel disease.
Myocardial Perfusion lmaging and Viability Testing
Myocardial perfusion imaging (MPI), also known as nuclear
stress testing, uses dif'ferences in myocardial blood flow to
detect ischemia. In SPECT MPI, a radiotracer is injected at rest
and at peak exercise/vasodilation, and the radiotracer is taken
up by the myocardium relative to blood flow. Rest images are
compared with images obtained after stress. Perfusion defects
observed after stress indicate flor.r, limiting CAD (Figure 2).
F I G U R E 2. Selected images from a nuclear perfusion single-photon emission CI
stress study. Normal perfusion is indicated by orange to white coloring, whereas poor
perfusion is indicated by purple to blue coloring. Short-axis views (/) of the heart with
stres (top row) and alresl(boftom row) show a radiotracer defect in the septal, anterior,
and inferior walls that is filled on the rest images. Long-axis views (8) demonstrate
anterior, apical, and inferior filling defects with stres (top row) that is perfused at rest
(bottom row). These findings are consistent with reversible myocardial ischemia.
7

20. TABLE 3. lnterpretation of Myocardial Viability
Study Results
Diagnostic Testing in Cardiology
SPECT Viabitity Testing
Rest Study
Repeated After
4-24 h (With
Thallium)
Perfusion defect Perfusion defect
Perfusion defect Reperfusion of
area
PETViability Testing
Baseline Metabolism
Perfusion defea Metabolically
active
Echocardiographic Viability Testing
lnitial Study
(at Rest)
Wall motion
abnormality
Low dose:
improvement of
function
Higher dose:
worsening of
function
Interpretation
Fixed defea:
infara, no viability
Viable
myocardium
lnterpretation
Viable
myocardium
Biphasic response
indicates viable
myocardium
Regions with fixed defects can indicate infarcted or hibernat
ing myocardium, and viability assessment can help distin
guish between the two (Table 3). Gated images can provide an
assessment of left ventricular systolic function.
SPECT imaging also can quantify the extent and severity
of disease. providing additional prognostic information. High
risk features, such as multiple regions of hypoperfusion,
reduction or lack ofaugmentation in poststress ejection frac
tion, transient cavity dilatation, and wall motion abnormali
ties. are associated with a worse prognosis.
Technetium based MPI has higher sensitivity and speci-
ficity than thallium-based studies and provides belter image
quality. Technetium based agents bind to the mitochondria,
allowing for delayed imaging. In contrast, thallium uptake
requires active metabolism, which can be useful in assessing
myocardial viability.
Cardiac PET provides excellent diagnostic and prognostic
information for patients known or suspected to have CAD. PET
provides better temporal and spatial resolution than does
SPECT, which is helpful in patients with obesity or nondiag-
nostic SPECT results. CT may be used with PET to provide
information on the presence of coronary artery calcification.
PET radiotracers have a short half'-life, resulting in lower radia
tion exposure and necessitating the use of vasodilators.
Vasodilator stress allows for assessment of peak stress ejection
fraction, quantification of absolute myocardial blood flow and
evaluation of myocardial metabolism. The utility of PET in
cardiac patients is limited by availability of the technologz.
Cardiouascular Magnetic Resononce lmaging
CMR imaging may be used with dobutamine to assess r,r'all
motion abnormalities or with vasodilators to assess perfusion.
It is commonll'performed to evaluate the degree of infarction.
ViabiliR' can be determined by evaluating the extent of m1,o-
cardial fibrosis within the left ventricle. Measures of right and
left ventricular function can be obtained u'ith gating. CMR
imaging is limited by operator expertise. length of tinte for
image acquisition. and availability.
XEY POIilIS
r Cardiac stress testing is best used in patients with an
intermediate pretest probability of coronary artery
disease.
o Exercise ECG is the preferred stress test in patients who
can exercise and have normal findings on a baseline
ECG, because exercise provides additional prognostic
information on functional capacity and hemodynamic
response.
. Stress testing with imaging is indicated in patients with
an inability to exercise, contraindications to exercise.
baseline ECG abnormalities that preclude interpretation
of exercise ECC results, or indeterminate findings on
exercise ECG.
Visualization of the Coronary Anatomy
Anatomic assessment of the epicardial coronary arteries can
be performed with noninvasive coronary CTA or invasive lngi
ography. Both tests require administration of contrast agents
and expose the patient to radiation. Abilit),to interpret CTA
can be limited in patients u'ith extensive calcification and
small distal arteries.
In select symptomatic patients. CTA is another option fbr
ruling out CAD. ln the PROMISE trial. 10,000 symptomatic
patients suspected of having CAD were evaluated r.t,ith an ini
tial strate$/ of either anatomic testing n'ith CTA or functional
testing. In patients n,ith an intermediate pretest probabilitl'of
CAD, the composite cardiovascular event rate r\Ias lor,r' (<1'll,
per year) in both groups, and outcomes (death. ml,ocardial
infarction, hospitalization for unstable angina. or major proce
dural complication) at 2 years did not differ betvreen groups.
Coronary CTA also may play a role in the evaluation of
acute chest pain in the emergency department. CTA is appro
priate in patients suspected of having an acute aortic syn
drome or coronary embolism. CTA may be helpful in patients
with low or intermediate likelihood of non ST elevation
acute coronary syndrome who have a lou, TIMI risk score.
negatire troponin assay. or nonischemic findings on ECG. It
also may be useful in patients with an equivocal diagnosis ol
non ST elevation acute coronary s)'ndrome u'ho have an
equivocal initial troponin level or single troponin elevation
without further symptoms of acute coronary syndrome or in
patients who have ischemic symptoms that resolved hours
before undergoing testing. Caref'ul consideration of patient
SPECT = sing e photon emiss on CT
Response to
Dobutamine
lnterpretationBaseline
8

21. f'actors is essential to avoid additional unnecessary testing,
potential harms, and unnecessary costs.
Invasive coronary angiography provides two dimensional
images of the coronary vessel Iumen through the injection of
nonionic contrast material using long, thin (<2-mm) catheters
percutaneously. The femoral or radial artery is used to obtain
arterial access, and radiation exposure is required. Invasive
angiography should be considered in highly symptomatic
patients with abnormal findings on stress testing, selected
patients with acute coronary syndrome, and patients with
ischemic symptoms refractory to medical therapy.
FFR, when added to angiography or CTA, measures the
hemodynamic significance of a lesion and helps determine the
need for intervention. FFR is the ratio ofblood flow distal to
the stenosis to blood flow proximal to the stenosis at maximal
t'low. It is typically measured during cardiac catheterization
by placing a pressure wire across the stenosis and inducing
conditions of maximal hyperemia, usually with adenosine.
lnstantaneous wave free ratio is a similar invasive functional
assessment ofcoronary stenoses that does not utilize vasodila
tor administration. FFR CT is an FDA approved noninvasive
diagnostic test that provides both anatomic and functional
coronary data; it has higher specificity for the diagnosis of
obstructive CAD than does CTA alone. FFR CT is performed
using computational fluid dynamics on CT derived data sets.
Limited availabiliff of FFR CT and potential delays in analysis
affect its use in patients with acute symptoms.
XEY POIl{I
. Coronary angiography and CT angiography (ClA) pro-
vide anatomic infbrmation regarding the extent and
severity ofcoronary artery disease; however, the diag
nostic value of CTA may be limited in cases of extensive
calcification.
Coronary Artery Calcium Scoring
Calcification of the coronary arteries is indicative ol ath-
erosclerosis and may be quantified with CT. Although CAC
scoring provides infbrmation regarding the burden of dis
ease, it cannot determine the degree of obstruction. CAC
measurement has been used for diagnosis and risk assess
ment in both symptomatic and asymptomatic patients.
Assessment ol CAC in asymptomatic patients should be
limited to those at intermediate cardiovascular risk and to
select patients at borderline risk in whom CAC results may
influence primary prevention therapy (see MKSAP 19
Ceneral Internal Medicine l). CAC score also is incorpo-
rated in the MESA Risk Score to predict 10 year coronary
heart disease risk (www.mesa nhlbi.org/MESACHDRisk/
MesaRiskScore/RiskScore.aspx).
CAC scores are categorized as follows: 0, no disease; 1 to
99. mild disease; 100 to 400, moderate disease; and greater
than 400, severe disease. Normal reference values depend on
age, ethnicity, and sex. The absence ofCAC is associated with
a low risk for cardiovascular events.
Diagnostic Testing in Cardiology
lmY P0t 1{r
. The absence of coronary artery calcification is associated
with a low risk for cardiovascular events.
Risks of Diagnostic Testing for
Coronary Artery Disease
Cardiac diagnostic testing carries risks related to exerciser expo
sure to pharmacologic stress testing agents, radiation, or contrast
agents; and vascular access for invasive procedures. Additionally,
inappropriate initial testing may lead to unnecessary down-
stream testing with added physical and flnancial costs.
There is a very small risk fbr myocardial infarction or
death (1/2500 patients) with exercise stress testing. Absolute
contraindications to exercise include unstable angina or acute
myocardial inflarction, uncontrolled arrhythmias, decompen-
sated heart failure, acute pulmonary embolism or deep venous
thrombosis, acute pericarditis or myocarditis, acute aortic
dissection, and symptomatic severe aortic stenosis. Relative
contraindications are left main coronary artery stenosis,
hypertrophic cardiomyopathy with severe obstruction, elec-
trolyte abnormalities, high degree atrioventricular block, and
significant arrhythmias.
Side effects of vasodilator stress agents (most commonly,
adenosine and regadenoson) include chest pain, headache,
and flushing. Atrioventricular block and bronchospasm also
may occur. Theophylline may be given after testing to reverse
these effects. Vasodilator stress testing is contraindicated in
patients with reactive airways disease with active wheezing,
systolic blood pressure of less than 90 mm Hg, sick sinus syn
drome, or high degree atrioventricular block.
SPECT, PET, CAC scoring, coronary CTA. and coronary
angiography all expose the patient to radiation. The level of
radiation exposure depends on the procedure, equipment,
choice ofradiopharmaceutical agent and dose, operator tech
nique, and patient characteristics (e.g., body size).
Contrast agents used in invasive angiography, coronary
CTA, and CMR imaging also pose a risk to the patient. Rarely,
CMR imaging that requires gadolinium contrast may cause
nephrogenic systemic fibrosis in patients with end stage kid
ney disease (see MKSAP 19 Nephrology). lodinated contrast
material used in CT or angiography may result in acute kidney
injury. Microbubble contrast agents are used to enhance the
endocardial borders in echocardiography and rarely can cause
hypersensitivity reactions.
Coronary angiography can be complicated by vascular
access problems, bleeding complications, coronary artery dis
section, aortic dissection, and plaque disruption or thrombus
leading to peripheral emboli, stroke, or myocardial infarction.
Femoral artery access can be complicated by retroperitoneal
hemorrhage, which should be suspected in patients with
hypotension, back or flank pain, and/or a drop in hemoglobin
level. Pseudoaneurysms at the arterial puncture sites occur
more commonly with f'emoral artery access and may manifest
as a large hematoma or new bruit at the access site.
9

22. Diagnostic Testing in Cardiology
I(EV POITIT
o Vasodilator stress testing is contraindicated in patients
with reactive airways disease with active wheezing, sys
tolic blood pressure of less than 90 mm Hg, sick sinus
syndrome, or high degree atrioventricular block.
Diagnostic Testing for Structural
Heart Disease
Diagnostic testing for structural heart disease should be consid
ered in patients with suggestive history and physical examina-
tion findings, such as a grade 316 or higher systolic murmur, a
late or holosystolic murmul a diastolic or continuous murmur,
or a murmur with accompanying symptoms. Repeat imaging is
indicated in patients with structural heart disease when there is
a change in the clinical presentation. tunctional status, or exam
ination findings; routine surveillance imaging is guided by
lesion severity (see Valvular Heart Disease). Imaging modalities
to evaluate for structural heart disease are listed in Table 4.
The mainstay of noninvasive imaging fbr structural heart
abnormalities is transthoracic echocardiography (TTE). TTE
evaluates right and left chamber size. thickness. and function.
including wall motion. TTE also provides information on val
vular patholory (including endocarditis), diastolic function,
hemodynamics, and the pericardium. TTE can be used rt,ith
intravenous agitated saline contrast. normally cleared by the
pulmonary circulation, to document the presence of an intra-
cardiac shunt. Dobutamine infusion during TTE may be used
in patients with low gradient aortic stenosis in the setting of
reduced ejection fraction to help diflerentiate between severe
aortic stenosis and pseud<lstenosis.
Transesophageal echocardiography (TEE) is commonly
used to evaluate for inf'ective endocarditis and its complica-
tions (e.g., abscess). TEE also may be used to better visualize
valvular pathologr, particularly when surgical or percutaneous
intervention is plannedr to evaluate specilic structures that
cannot be well visualized on TTE (e.g., prosthetic heart valves)
or in patients with poor transthoracic imaging; to ev:rlurrte
acute aortic abnormalities: and to rule out left atrial thrombus
befbre cardioversion (Figure 3). TEE requires moderate seda
tion and placement of the probe in the distal esophagus and
stomach. Contraindications include esophageal strictures or
active esophageal varices. Esophageal injury. including perfb
ration and bleeding, is a potential complication.
CMR imaging can be used for evaluation of myocardial
and pericardial disease, including inflammatory or infiltrative
processes. CMR is particularly useful in diagnosing hyper
trophic cardiomyopathy when there is eccentric or apical
hypertrophy that is diflicult to visualize on echocardiography.
Although cardiac amyloidosis can be diagnosed with
echocardiography or CMR imaging, 99m technetium pyro
phosphate scintigraphy (eetrlTc-PYP) has re emerged as a useful
means fbr diflerentiating between amyloid subtypes, which
guides patient management.
eemTc
PYP imaging specifically
F I G U R E 3. Transesophageal echocardiogram showing an absence of thrombus
in the left atrial appendage (LAA). The transducer is posteraor t0 the heart, and the
left atrium (LA) and LAA (arow) are more easily seen than with transthoraci(
echocardiography. LV = left ventricle.
identifies cardiac amyloidosis due to misfolded transthyretin
proteins (ATTR), with a specificity greater than 99'1,.
f,EY POIXIS
. The mainstay of noninvasive imaging for structural
heart abnormalities is transthoracic echocardiography,
which is used to evaluate patients'*'ith valvular abnor
malities. congenital heart disease. pericardial disease, or
ventricular dysfunction.
. ggm-Technetium pyrophosphate scintigraphy can be
used to differentiate between amyloid subtypes and guide
patient management.
Diagnostic Testing for
Cardiac Arrhythmias
The initial study in patients llith palpitations. pres)'ncope. or
syncope when an arrhythmia is suspected should be 12 lead
resting ECG. The ECC may show evidence o1'preexcitation.
ectopic rhythms. atrioventricular block. or intraventricular
conduction delay, providing insight into the cause of the
symptoms. Echocardiography should be perfbrmed in ptltients
suspected of having structural heart disease.
The intermittent and fleeting nature of'arrhllhmias can
make diagnosis difficult. Diagnostic studies are selected on the
basis of symptom frequency and the duration and timing of the
recording (Table 5). [f symptoms occur daill: a 2.1- or 1B hour
ambulatory ECG monitor (Holter monitor) ma1'be used. Long
term ambulatory ECC monitors can be worn fbr up to 30 days
if symptoms are less fiequent. lntiequent s),mptomatic e\ents
may be captured $,ith an external patient triggered event
recorder if the event lasts long enough for the patient to trigger
the device. A looping event recorder captures seleral seconds of
the ECG signal befbre the device is triggered: it is useful for
(Text continued on page 13)
10

23. TABLE 4. Diagnostic Testing for Structural Heart Disease
Diagnostic Testing in Cardiology
Transthoracic
echocardiography
Transesophageal
echoca rd iog ra phy
Th ree-dimensional
echocardiography
Radionuclide
angiography (MUGA)
Cardiac catheterization
(left and right)
Coronary CT
angiography
CMR imaging
Chest CT with contrast
99m-Technetium
pyrophosphate
scintigraphy
Heart failure
Cardiomyopathy
Valvular disease
Congenital heart disease
Pulmonary hypertension
Aortic disease
Pericardial disease
Endocarditis
Prosthetic valve dysfunction
Evaluation of embolic source
Aortic disease
Left atrialthrombus
Mitral valve disease
ASD (percutaneous ASD
closure)
Cardiac masses
Evaluation of LV systolic
function
Coronary artery disease
Congenital heart disease
Valve assessment
Shunt assessment
Coronary artery disease
Coronary anatomy assessment
Congenital heart disease
Congenital heart disease
Myocardial disease
(infiltrative disease,
myocarditis, hypertrophic
cardiomyopathy)
RV cardiomyopathy (ARVC)
Ouantitation of LV mass and
function
Diagnostic Test Major lndications Advantages
Accurate diagnosis of structural
heart disease and its severity
Ouantitation of LV size
.
nd function,
pulmonary pressures, valve function,
and intracardiac shunts
Widely available, portable, fast
High-quality images, especially of
posterior cardiac structures
Most accurate test for evaluation of
endocarditis, prosthetic valves, and
left atrial thrombus
lmproved tomographic imaging
Used during cardiac procedures for
device placement
lmproved assessment of LV global/
regional systolic function
Ouantitative EF measurements
Accurate for serial LVEF measurements
(e.9., to evaluate for cardiotoxicity
from chemotherapy)
Direct measurement of intracardiac
pressures, gradients, and shunts
Contrast angiography provides
visualization of complex cardiac
anatomy
Allows percutaneous intervention
for structural heart disease
Visualization of complex cardiac
anatomy
High-resolution tomographic images
High-resolution tomographic
imaging and blood-flow data
Ouantitative RVvolume and EF
measurements
No ionizing radiation or contrast
material
Enables three-dimensional
reconstruction of cardiac anatomy
Limitations
Operator-dependent data acquisition;
interpretation requires expertise
Variability in instrumentation
lmage quality limits diagnosis in
some patients (COPD, large body
habitus)
May require microbubble contrast
agents
Requires esophageal intubation,
typically with moderate sedation
Adjunct to two-dimensional imaging
Limited by availability and expertise
Radiation exposure
Provides no data on other cardiac
structures
lnvasive
Radiation and radiocontrast
exposure
lmages are not tomographic, limiting
evaluation of complex three-
dimensional anatomy
Radiation and radiocontrast exposure
lmage acquisition improved with
sinus rhythm and slower heart rate
Limited by availability and expertise
Patient claustrophobia
May be contraindicated in patients
with an older pacemakel lCD, or
other implanted devices
Certain gadolinium-based contrast
agents are contraindicated in
patients with CKD"
Sinus rhythm and slower heart rate are
needed for improved image quality
Radiation and radiocontrast
exposure
Aortic disease
Cardiac masses
Pericardial disease
High-resolution tomographic images
Enables three-d imensional
reconstruction of vascular structures
Amyloid transthyretin (ATTR) High specificity for ATTR amyloidosis Radiation exposure
cardiac amyloidosis
ARVC = arrhythmogenic right ventricular cardiomyopathy; ASD = atrial septal defecU CKD = chronic kidney disease; CMR = cardiac magnetic resonance; EF = ejection fradion;
"Group
I gadolinium-based contrast agents are contraindicated in patients with end-stage kidney disease or estimated glomerular filtration rate less than 30 mUmin/1 .73 mr.
Group ll gadolinium-based contrast agents are not contraindicated in patients with CKD. There is insufficient data to malu u recommendation for group lll gadolinium_based
contrast agents in CKD.
11

24. TABTE 5. Diagnostic Testing for Suspected or Known Cardiac Arrhythmias
Diagnostic Testing in Cardiology
Diagnostic Test or Device lndications
Resting ECG
Advantages
12-lead ECG recorded during
the arrhythmia often identifies
the specific arrhythmia
Records every beat for 24 h,
48 h, or up to 30 d for later
ana lysis
Patient log allows correlation
with symptoms
Recorded data may be
transmitted to central
monitoring service for rapid
notification
Allows diagnosis of exercise-
related arrhythmias
Allows assessment of impact of
arrhythmia on blood pressure
and symptoms
Small, pocket-sized recorder
is held to the chest when
symptoms are present
Recorded data may be
transmitted to central
monitoring service for rapid
notification
Continuous ECG signal is
recorded (with the previous
30 s to 2 min saved)when the
patient activates the recording
mode
Arrhythmia onset is recorded
Long-term continuous ECG
monitori ng with patient-
triggered or heart rate-
tri g gered episode stora ge
Specific heart rate or ORS
parameters can be setto
initiate recording of data
Auto-trig gered and patient-
triggered capture of arrhythmic
events
Up to 96 h of retrievable
memory
Some patch models connect
wirelessly to a mobile phone
Origin and mechanism of an
arrhythmia can be precisely
defined
Catheter ablation of the
abnormal heart rhythm or
implantation of a cardiac
electronic device (e.9.,
pacemaker or cardioverter-
defibrillator) may be
performed concomitantly
Limitations
Most arrhythmias are
intermittent and not recorded
on resting ECG
Short-term: Not helpful when
arrhythmia occurs infrequently;
ECG leads limit patient
activities (24- and 48-h
monitors)
Long-term:Adhesive
attachment to chest; detection
of rhythm abnormalities that
are asymptomatic or not
clinically significant
Physician supervision needed
during testing
Most arrhythmias are not
exercise related
Symptomatic arrhyth mias must
last long enough for patient to
activate the device
Arrhythmia onset is not recorded
Not use{ul for syncope or
extremely brief arrhythmias
ECG leads limit patient
activities
Used for 1-4 wk in most cases
Device records only when
activated by patient
lnvasive procedure with minor
ri sks
Device is functionalfor 1 3 y
(device may need to be
explanted)
ECG leads limit patient
activities unless a patch device
is used
Resource intensive
lnvasive procedure with some
risk
Some arrhythmias may not be
inducible, particularly i{ the
patient is sedated
lnitial diagnostic test in all
patients
Short-term: Frequent (at least
daily) asymptomatic or
symptomatic arrhythmias for
24- and 4B-h monitors
Long-term: lnfrequent
asym ptomatic or sym ptomatic
arrhythmias for monitoring up
to30d
Arrhyth mias provoked by
exercise
I nfrequent symptomatic
arrhythmias that last >1 '2 min
lnfrequent, brief symptomatic
arrhythmias
Syncope
Very i nfrequent asymptomatic
or symptomatic arrhythmias
(e.g., infrequent syncope)
Continuous outpatient ECG
recording for precise
quantification or capture of
rare arrhythmia
lnducing, identifying, and
clarifying the mechanism o{
arrhythmia as well as potential
treatment (catheter a blation)
Continuous ambulatory ECG
(Holter monitor)
Exercise ECG
Patient-triggered event
recorder
Looping event recorder
(wearable)
Looping event recorder
(implantable)
Mobile cardiac outpatient
telemetry
Electrophysiology study
12

25. syncope or presyncope associated with arrhyhmias. An
implanted loop recorder may be warranted in patients with
very infiequent events.
Exercise stress testing frequer.rtly is used in patients with
suspected or known arrhythmia to evaluate for chronotropic
incompetence. ischemia, and exercise induced arrhy'thmia.
Most patients do not require invasive electrophysiologr
testing. Electrophysiology testing may be indicated in patients
in rn hom the diagnosis remains indeterminate or in settings in
which catheter based intenentions mav be needed to treat
refractory arrhyhmias.
I(EY POIlIT
o The initial study in patients with palpitations, presyn-
cope, or syncope when an arrhythmia is suspected
should be 12-lead resting ECG.
Coronary Artery Disease
o Duration
o Aggra\ating factors (exertion. anrietl'. meals)
. Relieving factors
o Associated symptoms (shortness of breath. nausea. clil
phoresis)
Some demographic groups. including u'ot,'.tctt rti-tti
patients \'ith diabetes mellitus. may present onll'u'itl-r iln l.licril
s)'mptoms. including exertional d1'spnea. nausea. or e\aKqci-
ated fatigue.
The physical examin:rtion includes an eraluation oi tilt
cardiovascular system and a search for findings su&qcstills
conditions that mimic angina, including heart f.ailure. pr-rlmo
nary hypertension, valvular heart disease (particularll aortic
stenosis), and hypertrophic cardiomyopathy
The first step in diagnostic testing is to determine the pretcst
probability (or likelihood) of coronary artery diseasc' ((..\i))
(Table 6). A baseline resting ECG is required to eraiuate tbr ot'tg r
ing ischemia and to guide the choice of stress test (Figure +1.
Stress testing is most useful in patients $'ith an ir.rtemtedi.iic
probabiliry* of CAD; houever. u'hen the pretest probabilir, ot'C.\i)
is high. testing may provide prognostic infbrmation. Other ctiirg
noses sl-rould be pursued in patiellts u'ith normal stress test finci
ings. lf stress testingyields abnormal results. additional er,aluation
should be considered (see Diagnostic Testing in Cardiolog').
I(EY POIf,TS
r Stable angina is characterized by reproducible chest dis
comfbrt precipitated by exertion or emotional stress
without appreciable worsening over a period of at least
2 months.
o The initial evaluation of angina includes a locused his
tory eliciting qualiry location, radiation. and duration
ofangina; aggravating and relieving factors; and associ
ated symptoms.
Coronary Artery Disease
Stable Angina Pectoris
Diagnosis and Evaluation
Stable angina pectoris is reproducible angina (discon.rfort or
pressure of the chest, neck, or arms) of at least 2 months'dura
tion that is precipitated by a stable level of exertion or emotional
stress and is relieved with rest. Unstable angina is new onset
angina or angina occurring at a relatively low level of exefiion.
occurring at rest. or accelerating in fiequency or severity.
Unstable angina is associated u,ith increased short term risk for
acute myocardial infarction (Ml).
The evaluation of angina includes a focused history elicit
ing information on the follort,ing anginal characteristics:
o
Qualit!
o Location
o Radiation
TABLE 6. Pretest Likelihood of Coronary Artery Disease in Symptomatic Patients According to Age and Sex'
Pretest Likelihood
Nonanginal Chest Painb Atypical Angina' Typical Anginad
Age (v) Men Women Men Women Men Women
30 39
40-49
50-59
60 69
34
51
65
72
26
55
t3
B6
4
13
20
27
2
3
7
14
12
22
31
51
76
8l
93
94
'Each value represents the percentage with s gnrficant coronary anery disease on catheterization.
"Nonanglnal chest pain has one or none of the components of typical angrna.
'Atypical angl na has two of the three components of typica I angi na.
Reproduced with permission from F hn SD, Gardin JM, Abrams J, et al; Amer can College of Cardiology Foundat on.2A12 ACCF/ANNACP/AATS/PCNAJSCA /STS gu oelrne i.ri
College of Cardiology Foundation and the Amelcan Hean Association, lnc. Published by Elsevier lnc. Ail rights reserued.
13

26. Coronary Artery Disease
No additional testing
Yes
. No response to
therapy
o Lifestyle-limiting
symptoms
. Progression to
unstable angina
Stress lest
Exercise ECG
Exercise/pharmacologic MPI
Exercise/pharmacologic echocardiography
Coronary CT angiography
Criteria for Markedly Positive Test Result
Significant ST-segment J at low workload, ST-segment 1, hypotension
TID or lung intake of thallium, ischemia in >2 vascular distributions, EF <35%
EF <3596 at rest, ischemia in >2 vascular distributions, fall in EF with stress
Significant stenosis (>70% in a major epicardial coronary artery)
FIGURE 4, Diagnosisof coronaryarterdisease.CAD=coronararterydisease; EF=ejectionfraction; MPI =myocardial
perfusionimaging;TlD=transientischemicdilation.
'lntermediate
pretesl probabi ity {likelihood)
is variably delined as between 1 0% and 90% 0r belween 25"kail75"k.
General Approach to Treatment of
Stable Angina Pectoris
All patients with angina should receive guideline-directed
medical therapy comprising risk factor modification (regular
physical activity, weight loss, tobacco cessation, and dietary
changes), cardioprotective medications to prevent thrombosis and
limit atherosclerotic progression, and antianginal medications to
improve frurctional capacity through reduced cardiac workload
and/or increased myocardial oxygen delivery (Figure 5 and
Figure 6). Blood pressure control (with a goal of <130/80 mm Hg)
and diabetes management should be emphasized.
Cardioprotective Medications
Aspirin reduces the risk for Ml and cardiovascular death in
patients with stable angina. Guidelines recommend low dose
aspirin (75 762 mg/d) for secondary prevention because it is as
effective as high-dose aspirin (325 mgid) in preventing MI and
confers a lower bleeding risk. In aspirin intolerant patients,
clopidogrel, a platelet P2Yr2 receptor inhibitor, is an acceptable
alternative. Neither prasugrel nor ticagrelor has been studied
in the context of stable angina, and their role in managing this
condition remains to be established.
Lipid-lowering therapy, targeting LDL cholesterol in par-
ticular, reduces the risk for vascular events and progression of
underlying CAD. Statin therapy remains a cornerstone of sec
ondary prevention because it has been shown to reduce the
risk for MI, death, and stroke. High intensity statin therapy
(atorvastatin, 40-80 mg/d, or rosuvastatin, 20-40 mg/d)
decreases the LDL cholesterol level by 50'7, or more and is pre
f'erred to moderate intensity therapy fbr secondary preven
tion, as higher statin dosing and the resultant lower LDL
cholesterol have been associated with graded improvement in
outcomes. In patients with statin intolerance (e.g., those who
develop significant myalgia) or inadequate LDL cholesterol
reduction with statin therapy, it is reasonable to address LDL
cholesterol with nonstatin medications, especially ezetimibe
or proprotein convertase subtilisin/kexin type 9 (PCSKg)
inhibitors. The addition of icosapent ethyl to statin therapy
Cardiovascular symptoms
Calculate pretest probability (likelihood) of CAD
Low lntermediate" Highb
ECG normal and
able to exercise?
Medical therapy
for CAD
Exercise ECG
ECG abnormal and
able to exercise?
Exercise MPl, exercise
echocardiography, or
coronary CT angiography
Pharmacologic MPl,
pharmacologic
echocardiography, or
coronary CT angiography
No
Yes No
Markedly
positiye test
Markedly
positive test
Markedly
positive test
Coronary angiography
14

27. Coronary Artery Disease
HbAr. level >5.7%?
BMI :35 kg/m'??
eGFR>45 oGFR <45
mUminl 1.7 3 m2 mU minJ 1 -l 3 m2
F I G U n E 6. Management of stable angina pectoris.
pfl
=
parcutaneous coronary intervention.
Recommendations based on tihn SD, GardinJM,AbramsJ, etal; Ameri6n College of Gr
diology toundation. 201 2 ACCF/AHA/ACP/MTS/PCNA/sCAI/SIS guideline for the diagnosis
and management 0f patients with stable isdemi( heaft disease: a reportof theAmelicn
College olGrdiology toundation/Ameilcn HeartlssodationTask Force 0n PndiceGuide-
lines, and the American College of Physicians,American Association forTholacic Sutgery,
Preventive Grdiovas.ular Nu6esfusociation, Society for GrdiovascularAngiography and
lnteoentions, and Sodety ol lhoracic Surgeons. J Am Coll Grdiol. 201 2;60:e44-e1 64.
IPMID: 231821251 doi:10.1016/ija(c2012.07.013
level
27
risk and
risk?
Ml? >70
F.sing TG >150 mgy'dL
(1.69 mmol/L)?
Shared
YesNo
LDL-C rcmains
mmol/L)?
LDI--C rcmains
.70mg,/dL(1,81 mmoYLP
BPstill >130/80
mm Hg?
FIGURE 5. Guideline-directedmedical therapyforpatientswithstableischemicheartdisease(SIHD).ACC=AmericanCollegeof Cardiology; ACEI
=ACEinhibitor;
AHA=
American HeartAssociation; ARB
=angiotensin receptor blocker; BB =
p-blocker; BID=twice daily; BP
= blood pressurei CPAP= continuous positive airway pressure; eGFR =
estimated glomerular filtration rate; GLP-1 =
glucagon-like peptide 1; HbA,,
=
hemoglobin A1.; LDL-C
=
LDI- cholesterol; Ml = myocardial infarction; PCSK9 =
proprotein
convertase subtilisin/kexin type 9; SG[T2
= sodium-glucose cotransporter 2; TG = triglycerides.
Continued symptoms
Continued symptoms and impaired quality of life
Continued symptoms and impaired quality of life
o pBlocker (if not prescribed previously)
r Sublingual nitroglycorin as needed for angina
heartmedicalGuidelino-directed ther.py
disease
. Optimize long-acting
. Consider renolazine
blockerand
Refer ior coronary angiography
Coronary anatomy suitable for PCI
or surgical rcvascularization?
L
Yes
No
15

28. Coronary Artery Disease
provides further risk reduction in high risk patients with
ll,pertriglyceridemia. Management of statin and nonstatin
lipicl lowering therapies is discussed in MKSAP 19 General
lntern:rl Medicine 1.
ACE inhibitor therapy is indicated for stable angina if
there is concomitant diabetes. chronic kidney disease. left
ventricular (LV) dysfunction (ejection fraction <40')1,), heart
lirilure, or history of MI. In these populations. ACE inhibitors
have additional benefits unrelated to CAD, including presen'a
tion of kidney function and improvement in LV function.
Angiotensin receptor blockers (ARBs) may be used as an alter
nativc to ACE inhibitors, although combination therapy is not
indic:rted given the associated increase in ad'r,erse erents
without clinical benefit.
Antianginal Medications
p I3krckers relieve angina by reducing heart rate, myocardial
contractility, and blood pressure. resulting in reduced myocar
di:rl oxygen demand. They are recommended as first-line ther
ilpv in patients with stable angina. All B-blockers are equally
efTicacious in reducing angina. and the choice of p blocker
rr-ray depend on concomitant medical conditions (LV dysfunc
tion, kidnev dysfunction. lung disease, or significant hyper
tension). Dosage should be titrated to achieve a resting heart
rate between 55/min and 60/min. B-Blockers should be used
n,ith caution in patients taking nondihydropyridine calcium
channel blockers (verapamil, diltiazem) because of additive
neqrrtive inotropic and chronotropic effects. Caution also
shoulcl be exercised in the setting of significant conduction
disease or LV dysfunction. B, Selective p blockers. such as
n-retoprolol. are preferred in patients with significant lung
clisease to avoid worsening respiratory flunction. In patients
n'ith reduced LV function. metoprolol succinate, bisoprolol.
anit canreclilol are associated with reduced long term mortal
itll Side eflects include fatigue. lethargl sleep disturbances.
irnd impotence.
Calcium channel blockers improve myocardial oxygen
delivery through coronary vasodilation and reduction in
corr)nrrry vilscular resistance while also decreasing myocardial
oxlrgen consumption through antihypertensive and negative
inotropic etTects. These agents can be useful when symptoms
persist despite B blocker therapy or when p blockers are not
tolerated. Shoft-acting dihydropyridine formulations. such as
short acting nifedipine, should be avoided because they can
p:rradoxically worsen angina by acutely lowering blood pres-
sure. resulting in reflex tachycardia and increased myocardial
oxygen demand. Nondihydropyridine calcium channel block
er-s shonld not be used in patients with LV dysfunction because
ot'the increased adverse events associated with their negative
chronotropic and inotropic effects.
Nitrates improve myocardial oxygen delivery through cor
onary vasodilation and reduce oxygen demand by reducing
prelold, thereby reducing ventricular wall stress. The benefi
cja I etfbcts may be offset by reflex tachycardia unless combined
n,itlr F blockers or calcium channel blockers. Short acting
sublingual nitrates should be prescribed for acute reliel of
angina. Long acting nitrates, including isosorbide mononitrate
or dinitrate and nitroglycerin patch formulations. provide
constant vasodilation. A nitrate free intenal of B to 12 hours.
generalll' at night. is needed to avoid derelopment ol nitrate
tolerance and reduced efficacy. Side effects include headache.
flushing, and hypotension. Because ofthe risk for h1'potension.
concurrent use ofnitrates and phosphodiesterase 5 inhibitors
(e.g.. sildenafil) is contraindicated.
Ranolazine reduces wall tension and myocardial oxlgen
consumption through inhibition of the late sodium current
and subsequent prevention of calcium overload. resulting in
reduced angina and increased exercise time. Ranolazine has a
modest QT prolonging effect but no proarrhlthmic effects.
The QT interval should be monitored n'ith co administration
ofother QT-prolonging drugs, and dose reduction is indicated
in patients receiving moderate inhibitors olcltochrome P
'150
3A,1 (CYP3A4), such as verapamil and diltiazem. Ranolazine
should not be used in combination r,r'ith strong CYP3A4 inhib
itors (clarithromycin, itraconazole. ketoconazole, severaI HIV
medications) because of resultant increases in ranolazine
serum levels.
f,rY POTf,TS
o All patients with coronary artery disease should be
counseled on lifestyle modification. blood pressure
control, and management of diabetes mellitus.
o First-line therapy for stable angina includes aspirin.
statin therapy, and p-blocker therapy.
o ACE inhibitor therapy is indicated for stable angina with
concomitant left ventricular dysfunction, heart failure.
diabetes mellitus, chronic kidney disease. or history of
myocardial infarction.
Coronary Revascularization
Decision to Revascularize
Patients with angina refractory to medical therapy or mark
edly abnormal stress testing or coronary CT angiography
results should be considered for invasive coronary angiogra
phy (see Figure 4). The risks. benefits. and alternatives to
angiography should be discussed along u'ith potential findings
and therapeutic options. The primary goals of revasculariza
tion in stable syndromes are to lessen angina and improve
quality ollife. In contrast, in unstable or acute presentations as
lvell as in stable patients \,ith high-risk anatomic or clinical
f'eatures (left main CAD, large ischemic burden. and or heart
failure). revascularization is indicated for prer,ention of future
events and improved survival.
Revascularization targets are identified based on ana
tomic and functional physiotogic characteristics associated
with myocardial ischemia. Techniques such as fractional flolv
reserve and instantaneous wave free ratio provide information
on the f'unctional hemodynamic significance of indeterminate
lesions identified on angiographic imaging (see Diagnostic
16

29. t
\
I
Testing in Cardiologz), reducing both unnecessary stenting
and the need for urgent revascularization.
Percutaneous Coronary Intervention
Percutaneous coronary intervention (PCI) comprises several
different catheter based techniques to improve coronary
blood flow by relieving coronary obstruction. Following early
experience with balloon angioplasty and bare metal stenting,
most PCI procedures currently involve second generation
drug eluting stent placement, which reduces the risk fbr in
stent restenosis compared with bare metal stenting.
PCI is indicated to relieve symptoms in patients with
medically refractory angina, those unable to tolerate optimal
medical therapy, and those with high risk features on nonin
vasive testing. PCI has not been shown to be superior to
guideline directed medical therapy in reducing the risk for
death or MI in patients with stable angina with or without
diabetes.
Coronary Artery Bypass Graft Surgery
Coronary artery bypass grafting (CABG) with optimal medi
cal therapy is generally recommended for patients with
multivessel CAD because it results in decreased recurrence
of'angina, lower rates of MI, and fewer repeat revasculariza
tion procedures compared with PCI or medical therapy
alone, especially when arterial (internal mammary artery)
conduits are utilized. CABC is associated with improved
survival in patients lvith left main or three vessel CAD and
is indicated in those with multivessel disease and diabetes.
CABG also improves 10 year survival compared with medi
cal therapy alone in patients with severe LV dysfunction.
Although myocardial viability is associated with improved
survival and ventricular recovery following revasculariz:r
tion in patients with LV dysfunction, the role of viability
testing before revascularization has not been established as
a predictor of outcome.
After Revascularization
Aspirin is recommended indef initely after revascularization.
't'he
addition of a P2Y,, inhibitor to aspirin (dual antiplatelet
therapy [DAPT]) is indicated to reduce risk fbr stent thrombo
sis and remote ischemic events. DAPT duration depends on
clinical considerations, including patient presentation and
bleeding and ischemic risks.
ln patients treated with bare metal stent placement, a
minimum of 1 month of DAPT is recommended. Current
guidelines recommend treating patients with stable angina
with DAPT for at least 6 months without interruption after
drug eluting stent placement, with the option to continue
therapy for a longer duration in those with a high risk for
thrombosis related complications (e.g., depressed LV func
tion, saphenous vein graft stenting, and diabetes) and a favora
ble bleeding profile. In patients at high risk for bleeding,
current evidence supports 3 months of DAPT followed by
lif'elong antiplatelet monotherapy as a reasonable strategz.
Coronary Artery Disease
Although guidelines define minimum DAPT duration, the
optimal duration should be individualized according to the
patient's risks for thrombotic and bleeding complications.
In patients requiring oral anticoagulation for atrial fibril
lation, warfarin or a direct oral anticoagulant (preferred) plus
clopidogrel can be considered without aspirin, often after 2 to
4 weeks of triple therapy. In patients with a mechanical valve
prosthesis, warfarin plus clopidogrel therapy is reasonable;
direct oral anticoagulants are contraindicated in these patients.
In patients undergoing CABG for stable CAD, DAPT for
12 months may be reasonable to improve the patency ol vein
grafts.
t( EY P0lltT5
o The primary goals of revascularization in stable ischemic
syndromes are to lessen angina and improve quality of
life.
o Percutaneous coronary intervention may alleviate
angina symptoms but does not decrease mortality or
risk for myocardial infarction in patients with stable
angina.
. In patients with stable angina who require revasculari
zation, coronary artery bypass graft revascularization is
generally preferred to percutaneous coronary interven
tion in those with left main or three-vessel coronary
artery disease or multivessel coronary artery disease plus
diabetes mellitus.
. Ten-year survival is improved in patients with coronary
artery disease and severe left ventricular dysfunction
who undergo coronary artery bypass grafting compared
with those who receive medical therapy.
. In patients with stable angina who undergo percutane-
ous coronary intervention, dual antiplatelet therapy
should be continued for at least 1 month after bare
metal stent placement and at least 6 months after drug
eluting stent placement.
Acute Coronary Syndromes
General Considerations
An acute coronary syndrome (ACS) results from acute or sub
acute plaque rupture or erosion and coronary blood flow
impairment, manilesting as acute onset chest pain or an
angina equivalent, often without a clear precipitant. The spec-
trum of ACS is further characterized by the presence of serum
biomarkers of myocardial injury (elevated troponin T or I).
Mycicardial injury or MI may be related to an atherothrombotic
event (type 1) or demand/supply mismatch (type 2) (Figure 7).
ST elevation Ml (STEMI) is differentiated from non
ST elevation acute coronary syndrome (NSTE ACS) by findings
on ECG (Figure 8). The hallmark ECG features of STEMI are
ST segment elevation of at least 1 mm in two or more contigu
ous limb or chest leads, although ST segment elevation in
leads V, and V" must be at least 2 mm in men and at least
L
L
!
17

30. With acute
ischemiab
Without acute
ischemiab
Elevated Cardiac Troponin Value(s) >9fth percentile URL
Troponin rise and/or fall Troponin level stable'
Coronary Artery Disease
Type 2 Ml: examples
. Severe hypertension
o Sustained tachyarrhythmia
1.5 mm in women for diagnosis. Posterior MI typically mani
fests as ST segment depression greater than 2 mm in the ante
rior leads (V, through V1) with tall R waves, often with
ST segment elevation in the inferior or lateral leads and
ST-segment elevation in posterior leads V, through Vr. New
F I G U R E 8, Diagnosis of acute coronary syndromes. NSTE-ACS = non-Sl-elevation
acute coronary syndrome; NSIEMI = non-ST-elevation myocardial infarction;
SIEMI = SI'elevation myocardial infarction.
bundle branch block may be considered a STEMI equivalent
and potentially reflects an acute left anterior descending artery
occlusion or extensive injury.
NSTE-ACS is categorized according to the presence ofbio
markers of cardiac injury (troponin T or l) in the serum. Non-
ST elevation MI is defined as a biomarker positive presentation
that does not meet criteria for STEMI. Unstable angina is charac-
terized by new or worsening angina, with or without ECG
changes, and without detectable levels of cardiac injury markers.
Sl-Elevation Myocardial t nfarction
Recognition
STEMI typically involves coronary plaque mpture causing
platelet adhesion, activation, and aggregation and acute
thrombotic occlusion. The sudden transmural myocardial
ischemia manifests as ST-segment elevation and signifies the
need for rapid initiation ofreperfusion therapy (Figure 9).
Although the presentation of STEMI is olten dramatic and
clear, several diagnoses can mimic STEML Acute pericarditis
presents with acute chest pain and ST-segment elevation sugges-
tive of STEMI. Distinguishing features may include pleuritic or
positional pain and diffuse or localized concave ST-segment ele
vation with corresponding PR segment depression (Frgure lO).
Pericarditis and myopericarditis resulting from viral infections
or autoimmune conditions can cause cardiac enzyme release,
further confusing the clinical picture and necessitating a
Examples
o Acute heart failure
. Myocarditis
FIGURE 7. Amodel{orinterpreting myocardial injur. lschemicthresholdsvarsubstantially in relationtothe magnitudeofthestressorandtheextentofunderlying cardiac
disease. Ml= myocardial infarction; URL= upper reference limit.
'Stable
denotes <20% variation of trop0nin values in the appropriate clin cal rontext.
blschemia
denotes signs and/0r symptoms of clinical myocardial ischemia.
@201 I The European Society of Cardiology; American College ol Cardioloqy Foundati0n; American Heart I6s0ciation, lnc ; and lhe World Heart Federation.
Acute myocardial
infaraion
Oxygen supply
and demand
imbalance
Atherosclerosis
+ thrombosis
Type 1 Ml: triggers
o Plaque rupture
. Plaque erosion
Acute myocardial
injury
Chronic
myocardial injury
o Structural heart disease
o Chronic kidney disease
Examples
Acute coronary syndrome
(STEMI and NSTE-ACS)
ST-segment elevation Nonspecific ECG changes
ST-segment depression
T-wave inversion
STEMI NSTE-ACS
Elevated cardiac
troponin levels
Normal cardiac
troponin levels
Unstable anginaNSTEMI
18

31. Coronary Artery Disease
STEMI
Evaluate:
1. Time from onset of symptoms"
2. High-risk featuresb
3. Time to device therapy with PCI
4. Risks of thrombolytic therapy (contraindications)
lnitiate medical therapy: aspirin,
p-blocker, nitrates, anticoagulant
PCI-capable facility Non-PCl-capable facility
Administer P2Yl, inhibitord
=
glycoprotein llb/llla inhibitor
Thrombolytic therapy
(goal s30 min from arrival)'
Administer clopidogrel
(300-mg loading dose)
PCI (FMC-to-device goal: <90
min for primary PCI)
Thrombolytic failure Successful reperfusion
Long-term medical therapy:
aspirin, P-blocker, ACE inhibitor,
high-intensity statin,
P2Y,, inhibitor Emergent transfer
for rescue PCI
Transfer for primary PCI'
Urgent transfer for
elective coronary
angiography and
possible PCll
FIGURE 9. Managementof ST-elevationmyocardial infarction(SIEI\41).Fl\4C=firstmedicalcontact; PCI =percutaneousc0r0naryintervention.
'lf )4 hours have elapsed since symptom 0nset, PCI is prefered.
bHigh.risk
features, such as cardioqenic shock and heart failure, favor PCl.
'F[,4C.to.device
("doorto balloon") goal for patients beinq transle(ed for primary PCI is as soon as possible and <1 20 minutes.
dP2Yr2
inhibitore: dopidogrel, prasugrel, ticagrelor.
within 30 minltes 0l h0spital presentati0n ("door to needle time') as a systems goal unless thrombolytic therapy is contraindicated.
thrombolytic therapy administration but is ideally performed within 24 hours.
CIR 0h013e3182742r{6
I
L
i
i
L
I
I
I
L
t
t
I
t
I
t
i
thorough history physical examination, and careful study of
the ECG and biomarker release patterns.
Acute aortic syndromes can cause ST-segment elevation if
the dissection involves the left or right coronary artery and is
due to transmural myocardial ischemia. Early recognition is
essential for this surgical emergency. Diagnostic clues to aortic
dissection include differential blood pressures in the upper
extremities, tearing quality of pain with radiation to the back,
and mediastinal widening on chest radiograph.
Severe hypercalcemia may result in ST segment elevation
that mimics ACS; however, other findings include a short QT
interval and flattened T waves.
Patients with accelerated hyper-tension, significant LV
hypertrophy, and cardiomyopathies may present with chest
pain and elevated cardiac troponin levels caused by elevated
LV filling pressures or wall tension with associated subendo-
cardial ischemia. The ECG findings are often abnormal in these
patients. LV hypertrophy induced ECG changes may look
similar to ST segment elevation injury currents; however,
these changes are tlpically concave in appearance. Comparison
with previous ECG findings is helpful in identifying acute
changes.
Patients with supraventricular tachycardias, which may
dramatically increase the rate pressure product, often present
19

32. Coronary Artery Disease
with chest pain, ST-segment depression, and elevated cardiac
enzyme levels, even if no CAD is present.
Reperfusion
Prompt reperfusion with primary PCI (PPCD or thrombolytic
therapy is indicated in all patients with STEMI who do not
have limited life expectancy from other nonreversible disease
(see Figure 9). Short times to repedusion are correlated with
improved outcome regardless of reperfusion strates/.
Primarg Percutaneous Coronary Interuention
PPCI refers to the process by which an emergency medical
provider activates a team of clinicians to initiate emergent
coronary angiography and PCI in patients with STEMI. The
goal time from first medical contact until PPCI is 9O minutes
or less. Because rates of achieving vessel patenry are higher
and more reliable with PPCI than with thrombolysis, PPCI is
the preferred method of treating STEMI when the patient pre-
sents to a PCl-capable hospital or can be transferred from an
20

33. TABLE 7. P2Y.2 lnhibitors Used in the Treatment of Patients With CAD Undergoing PCI
Coronary Artery Disease
Loading Dose Maintenance Dose Adverse Effects Contraindications
300-600 mg 75 mg/d
Drug
Clopidogrel
Ticagrelor
lndications
Stable CAD treated with
PCI
ACS
ACS
I ncreased
bleeding risk
Known allergy to the
drug
180 mg
\
ir.rdex hospital to a PCI capable center quickly (time from first
medical contact to PPCI of <120 minutes). Although the initial
fbcus of PPCI is on quickly restoring flow to the acutely
occluded artery there is a demonstrable reduction in cardio
vasclllar death and MI end points associated with complete
revascularization compared with culprit only PCI in patients
with multivessel disease. The relative benefit associated with
timing of PCI of nonculprit vessels during the same procedure
or within a short interval after PCI of the infarct related vessel
is not established.
Patients undergoing PPCI should receive aspirin (t0Z :ZS mg).
intravenous unfractionated heparin (with or without glyco
protein IIb/llla blockade) or bivalirudin, and loading doses of
additional antiplatelet drugs (P2Yu inhibitors) prior to or upon
arrival in the catheterization laboratory (Table 7).
Thrombolytic Therapy
'lhrombolytic
therapy is recommended for patients with
STEMI when symptom onset is within 12 hours and PPCI is not
available within 120 minutes of first medical contact. If symp
tcrnrs began 12 to 24 hours befbre presentation and there is
evidence of hemodynamic instability or significant myocar
dium at risk (such as with anterior MI), thrombolytic therapy
should be considered if timely transfer for PPCI (the pref,erred
strategy) is not available. Thrombolytic therapy is most
eftective within the first 3 to 6 hours from symptom onset,
after which time fibrin cross linking renders the clot rela
tively resistant to lysis. When compared with streptokinase,
r.rewer fibrin specific thrombolytic agents (alteplase, reteplase,
tenecteplase) are associated with improved infarct artery
patency and fewer allergic reactions, although they are more
costly and have not lowered the risk tbr intracerebral hemor
rhage (0.5'){, 0.97,).
Although thrombolytic therapy is potentially life saving,
it carries significant risks, primarily related to bleeding.
90 mg twice daily" lncreased
bleeding risk,
dyspnea
lncreased
bleeding risk
Known allergy to the
drug
h-rtracerebral hemorrhage is catastrophic, occurring in approx
imately l'1, of patients. Relative and absolute contraindications
to thrombolytic therapy are listed in Table 8.
ln addition to thrombolytic therapy, all patients without
a specific contraindication should receive a loading dose of
aspirin (162 325 mg) as well as intravenous unfractionated
heparin, enoxaparin, or fbndaparinux. Clopidogrel loading
(300 mg orally) has been demonstrated to increase rates of
vessel patency and is also recommended in this setting.
Prompt transfer to a PCI capable center following throm
bolytic therapy (for possible rescue PCI) is reasonable when
this option is available. The ECG should be monitored at 60 to
90 minutes to confirm reperfusion, reflected by at least 50'7,
improvement in maximal ST segment elevation. One quarter
to one third of patients do not achieve reperfusion, particu
larly with delayed presentation. Rescue PCI is associated with
improved outcomes compared with conservative management
in cases of failed reperfusion. Comnary angiography is recom
mended in all patients before discharge, even after successful
thrombolysis.
XEY POIilIS
. When available in a timely manner, primary percutane-
ous coronary intervention is preferred to thromboly'tic
therapy for the treatment of ST elevation myocardial
inlarction.
r If primary percutaneous coronary intervention (PCI) is
not available within 120 minutes of first medical con
tact, patients with ST elevation myocardial infarction
should receive thrombolytic therapy and be transferred
urgently to a PCl-capable center.
Complications of STEMI
Arrhythmias are common in the peri infarct setting. Atrial
fibrillation, which aftects up to 20'X, of patients with STEMl,
Prasugrel ACS treated with PClb 60 mg 10 mg/d' Known allergy to
the drug, previous
transient ischemic
attack/stroked, age
>-75 y
ACS = acute coronary syndrome; CAD = coronary aa(ery disease; PCI = percutaneous coronary ntervent on.
Ticagrelor should be used with aspirin, 81 mg/d.
"Prasugrel should not be loaded "upstream" (before catheterization).
' Prasugrel, 5 mg/d, shou ld be consrdered for those weighi ng less than 60 k9 ( 1 32 lb).
aftack or stroke
21

34. Absolute Contraindications
TABLE 8. Contraindications and Cautions for Thrombolytic
Thera py i n ST-Elevation Myocardia I I nf a rction'
Coronary Artery Disease
Any previous intracranial hemorrhage
Known structural cerebrovascular lesion (e.9., arteriovenous
malformation)
Known malignant intracranial neoplasm (primary or metastatic)
lschemic stroke within 3 mo (except acute ischemic stroke
within 4.5 h)
Suspected aortic dissection
Active bleeding or bleeding diathesis (excluding menses)
Significant closed head or facial trauma within 3 mo
lntracranial or intraspinal surgery within 2 mo
Severe uncontrolled hypertension (unresponsive to emergency
therapy)
For streptokinase:treatment within the previous 6 mo
Relative Contraindications
History of chronic, severe, poorly controlled hypertension
Severe uncontrolled hypertension on presentation
(SBP >1 80 mm Hg or DBP >1 10 mm Hg)"
History of ischemic stroke (>3 mo previously)
Dementia
Known intracranial abnormality not covered in absolute
contra indications
Traumatic or prolonged (>10 min)CPR
Major surgery within 3 wk
Recent (within 2-4 wk) internal bleeding
Noncompressible vascular puncture site
Pregnancy
Active peptic ulcer disease
Oral anticoagulant therapy
CPR = cardiopulmonary resuscitation; DBP = diastolic blood pressure; SBP = systolic
blood pressure.
nViewed
as advisory for clinical decision making and may not be all-inclusive or
definitive.
Reproduced with permission lrom O'Gara PT, Kushner FG, Ascheim DD, et al. 201 3
ACCF/AHA guideline for the management of ST.elevation myocardial infarction: a
report of the American College of Card iology Fou ndation/American Heaft
Association Task Force on Practice Guadelines. J Am Coll Cardiol.2013;61:e78 e140.
IPM lD: 232 5691 4l doi:1 0.1 01 6/j.jacc.2O12.1 1 .01 9. O201 3 American College of
Cardiology Foundatron and the American Hean Association, lnc. Published by
Elsevier lnc. All rights reserued.
complicates management and may cause hemodynamic insta
bility. Ventricular tachycardia or fibrillation may occur during
MI or after reperfusion. Repetitive and sustained bouts of
postinfarction ventricular arrhythmias, especially beyond
48 hours, may warrant a longer period of inpatient telemetry
and predischarge implantable cardioverter defibrillator ther
apy. Routine suppression of ventricular ectopy with antiar
rhythmic agents is not recommended and is associated with
increased arrhythmias and adverse outcomes. In particular,
accelerated idioventricular rhythm, which commonly arises
after reperfusion, is generally benign and transient, requiring
no treatment. Atrioventricular block, including Wenckebach
and complete heart block, may occur after inferior infarction'
requiring temporary transvenous pacing: howevet perTnanent
pacing is rarely required. Benign forms of vagally mediated
heart block must be differentiated from Mobitz type 2 second
degree atrioventricular block, which is more frequently
observed with anterior infarction and damage to the conduc
tion system. Mobitz type 2 block may progress to complete
heart block and requires permanent pacing.
Cardiogenic shock, a common complication of STEMI.
typically results from a large anterior MI due to severely
reduced LV systolic function. Cardiogenic shock carries a
mortality rate of 5O'2, to 80'7, and must be recognized early.
Shock is suggested by hypotension, sinus tachycardia. oligu
ria, cool extremities, and altered mentation. Untreated car
diogenic shock can progress rapidly to end organ failure. such
as acute kidney or liver failure or mesenteric infarction.
Patients, particularly those younger than 75 years. ha\€ a
higher rate ofsurvival ilthey receive emergent revasculariza
tion. An intra aortic balloon pump (IABP). an LV assist device.
or extracorporeal membrane oxygenation may be used tem
porarily, although limited data support their benefit in cardio
genic shock. Once the patient is stabilized. weaning the
patient from mechanical and inotropic support and slow
uptitration of afterload-reducing agents. such as captopril.
can be attempted. p Blockers should be avoided initially and
can be introduced once the patient is hemodynamically sta-
ble. Diuretics should be used to treat pulmonary vascular
congestion.
Approximately 10'/, to 20'2, of cases of anterior STEMI are
complicated by LV apical thrombus. Anticoagulation is gen
erally recommended for at least 3 months to reduce the risk
for systemic embolization, although LV aneurysm without
associated thrombus generally is not treated with anticoagu
lation unless other indications. such as atrial fibrillation, are
present.
Right ventricular (RV) infarction, typically identified by
ST segment elevation on right sided ECG Ieads (V, and V1R),
can complicate right coronary artery occlusion. Patients pre
sent with hypotension. elevated central venous pressure, and
clear lungs. RV dysfunction causes inadequate filling ofthe LV.
resulting in shock. Transthoracic echocardiography reveals RV
dilatation and dysfunction and may be useful in establishing
the diagnosis. Volume resuscitation, inotropes (dobutamine or
dopamine), and RV mechanical support may be necessary to
bridge to RV recovery which generally takes 2 to 3 days.
Nitrates are contraindicated because they may worsen hypo-
tension by reducing preload.
LV free nall rupture produces sudden onset chest pain or
syncope with rapid progression to pulseless electrical activity.
LV free wall rupture is more common in older adults.
,omen.
patients with anterior MI, those receiving anti inflammatory
agents, and patients with delayed reperfusion. Surgical, or
sometimes percutaneous, repair is indicated, but mortality
rates are very high.
22

35. Acquired ventricular septal defect (VSD) from septal wall
rupture may complicate infbrior or anterior STEMI, usually in
patients with multivessel CAD. VSDs typically occur within
5 days of STEMI presentation. Patients present with worsening
heart failure and shock, and a harsh holosystolic murmur may
be heard at the left lower sternal border. The diagnosis is con
firmed with echocardiography. Although initial management
may include afterload reduction with medical therapy and
IABP support, the mortality rate in patients with medically
treated postinfarct VSDs approaches 100'/u. Surgical closure
should be considered; however, the mortality rate in surgical
series is still high (approximately 50'/.). Patch closure can be
very difficult because of the necrotic tissue and inability tr_r
find viable myocardium to suture and patch. Percutaneous
closure with a VSD occluder device is possible but of'ten
unsuccessful because of the nature of the defect. and residual
shunting around the device is common.
When the posteromedial papillary muscle bloocl supply
f mm the posterior descending artery is interrupted during Ml,
rupture may occur, resulting in severe acute mitral regurgita,
tion several days after STEMI. Afterload reduction and IABP
placement may be temporizing, although urgent surgical
intervention usually is indicated. Acute severe mitral regurgi
tation also may result lruim l.V dysfunction and is often related
to an inferior MI with restriction of the posterior mitral leaflet,
termed functional ischemic mitral regurgitation. Ischemic
rnitral regurgitation is treated with revascularization and med.
ical therapy.
Non-ST-Elevation Acute Coronary Syndromes
NSI'E ACS, like SI'EMI, is on the spectrum of acute ischemic
disorders attributable to plaque erosion or rupture and throm
botic occlusion, manifbsting as acute chest pain at rest or with
minimal exertion. Unlike the complete coronary obstruction
resulting in STEMI. NSTI-I ACS involves incomplete or tr:rnsient
obstruction without ST segment elevation, although ST segment
clepression is often present.
Risk Stratification
Initial assessment of suspected NSTE ACS involves a careful
history physical examination. ECG. and serial biomarker meas
Llrement to determine the likelihood of a cardiac process. In
patients with lou' likelihoocl of a coronary event, alternative
causes should be investig:rted. In patients with an intermediate
or a high likelihood ol ACS. prognostic assessment with risk
scores. such as TIMI and GRACE risk models. is indicated. The
simpler of the two models, the TIMI risk score, predicts 14 day
cleath, recurrent MI, and urgent revascularization rates (Table 9).
'l'he
GRACE risk score (www.gracescore.org) incclrporates
examination findings, clinical teatures, ECG findings, and bio
nlarker variables (creatinine levels, elevated cardiac enz.ymes)
kr predict in house and postclischarge death and Ml risk.
Prognostic assessment along with serial troponin me:lsure
ment and clinical status helps determine the most appnrpriate
therapeutic strate$/ (Figure l1).
Coronary Artery Disease
Age >65 y
>3 Traditional CAD risk factorsu
Documented CAD with >50% diameter stenosis
ST-segment deviation
>2 Anginal episodes in the past 24 h
Aspirin use in the past wk
Elevated cardiac biomarkers (creatine kinase MB ortroponin)
0-2
3-4
5-7
Low risk
lntermediate risk
High risk
CAD = coronary artery disease; TlMl = thrombolysis in myocardial infarction.
oHypertension,
hypercholesterolemia, diabetes meilitus, being a current smoker
family history of CAD.
lnformation from Antman Et\4, Cohen M, Bernink PJ, et al. The TlMl risk score {or
unstable angina/non ST elevation Ml: a method for prognostication and
therapeutic decision making. JAMA. 2000;284:835 42. IPMID:
1 093817 2l
doi:1 0. 1 001 /jama.284. 7.835
Invasive Versus Ischemia-Guided Treatment
Urgent invasive treatment (within 2 hours) is recommended
for patients with NSTE ACS who have hemodynamic insta
bility, refiactory chest pain, heart failure, or ventricular
arrhythmias.
In high risk and troponin positive patients with NSTE
ACS who have been initially stabilized, an early invasive strat
egy improves the composite clinical end point of death,
recurrent MI, and repeat hospitalization compared with an
ischemia guided approach. In patients with an elevated clini-
cal risk score, significant S'l segment deviation, or elevated
cardiac biomarkers. cardiac catheterization is usually per
formed within 24 hours of presentation. Other patients,
including those with diabetes, stage 2 to 3 chronic kidney
disease, LV dysfunction, and recent PCI without elevated risk
scores, may be saf'ely evaluated with coronary angiography
within 72 hours of presentation (delayed invasive strateSr).
The choice ol revascularization procedure (PCI or CABG) is
based on the results ofangiography.
With :rn ischemia guided strategy, patients undergo
noninvasive stress testing ',{,ith LV function assessment
before hospital discharge. Cardiac catheterization is reserved
for patients with active or intermittent ischemia, including
those with angina despite medical therapy or evidence of
ischemia on stress testing, and patients at very high clinical
risk based on risk score. The ischemia guided approach is
appropriate lbr low risk patients (TlMl score <2 or GRACE
score <109). particularly low risk women, who may have
worse outcomes with an early invasive approach.
'fhe
rec'
ommendations fbr non low risk women are identical to
those for men.
Prognostic Variables (1 Point Each)
TABLE 9. TlMl Risk Score for Non-ST-Elevation Acute
Coronary Syndromes
TlMl Risk Score (Sum of Prognostic Variables)
23

36. Coronary Artery Disease
tIGURE ll.lnitial managementof non-ST-elevationacutecoronarysyndromes.NSTEMl=non-ST-elevationmyocardial infarction; UA=unstableangina;
UFH = unfractionated heparin.
'Clopidogrel or licagrelor may be administered at the time oi hospital admission and diagnosis oi a(ute coronary syndrome
blf
coronary artery bypass g(afting is required, dopidogrel or li(agrelor sh0u d be stopped, and surgery should be delayed lor at least 5 days
tlf
the decisi0n ls made t0 withh0ld a P2Yri inhibitor until the time ol angiography and a P2Yr: inhibitor rs desired, cloprdogrel, ticagrelor, or prasugrel can be nitialed
f,tY ?orilIt
. Patients with a non ST elevation acute coronary syn
drome who have hemodynamic instability, refractory
chest pain, heart failure, or ventricular arrh,'thmias
require emergent coronary angiography and percutane
ous coronary inlervention.
r The decision to pursue an invasive versus ischemia
guided approach in patients with a non-ST-elevation
acute coronary s)mdrome is guided by the patient's risk
for clinical events, which in turn is determined by deci-
sion support tools, such as the TIMI or GRACE score.
Medical Therapy for Acute
Coronary Syndromes
Medical therapies are consistent across the spectrum of
atherothrombotic ACS (Table lO). However. importantly.
thrombolytic therapy has no benefit in patients with NSTE
ACS and is not recommended.
All patients presenting with ACS should receive a loading
dose of aspirin. supplemental oxygen for oxygen saturation
less than 90'X, to 92'7,. therapy to relieve symptonls (nitrates).
therapy to reduce infarct size (0 blockers. ACE inhibitors),
high intensity statin therapy, and antithrombotic therapy
(antiplatelet agents and anticoagulants).
Normal or low-risk stress
test results and no
recurrent chest pain
Discharge with
follow-up
Non-ST-elevation acute coronary
syndrome (UA/NSTEMI)
lnitiate aspirin, p-blocker, nitrates, statin
Risk strati{ication
High risk
(TlMl 5-7 or
GRACE 141-372)
lntermediate risk
(TlMl 3-4 or
GRACE 109-140)
Low risk
(TlMl 0-2 or
GRACE 1-108)
lschemia-guided
strat6gY
. Antiplatelet therapy
(aspirin plus either
clopidogrel or
ticagrelor,b)
. Anticoagulant therapy:
UFH or enoxaparin;
alternatively,
fondaparinux
Delayed invasive
strategy (25-72 houB)
. Antiplatelettherapy
(aspirin plus either
clopidogrel or
ticagrelol.
b)
. Anticoagulant therapy:
UFH or enoxaparin;
selectively, fondaparinux
or bivalirudin
o Consider diagnostic
coronary angiography'
Early invasive strategy
(within 24 hours)
. Antiplatelettherapy
(aspirin plus either
clopidogrel or
ticagrelor',b)
. Anticoagulant therapy:
UFH or enoxaparin;
selectively, fondaparinux
or bivalirudin
. UrgenVimmediate
diagnostic coronary
angiography
Stress testing
Abnormal stress test results
or recurrent symptoms with
suspected cardiac etiology
Coronary
angiography
24

37. Coronary Artery Disease
TABLE 10. MedicalTherapy for Acute Coronary Syndromes
Medication Drugs in Class
Antiplatelet Medications
Aspirin N/A
Clopidogrel N/A
Prasugrel N/A
Ticagrelor N/A
Cardioprotective Medications
p-Blockers
Dosage
81-162 mg/d
75 mg/d
10 mg/d
90 mg twice
daily
Variable
Variable
Variable
25-50 mg/d
Indications
All patients with ACS, unless
intolerant or allergic
Aspiri n desensitization may
be considered for patients
with allergy
P2Y12 inhibitor in combination
with aspirin is indicated in all
patients after ACS for at least
1y
P2Y,2 inhibitor in combination
with aspirin is indicated in all
patients after ACS for at least
1 y; prasugrel is indicated only
in patients with ACS in whom
PCI is performed
P2Y12 inhibitor in combination
with aspirin is indicated in all
patients after ACS for at least
1y
All patients with prior Ml or
LV systolic dysfunction (only
metoprolol succinate,
carvedilol, or bisoprolol)
All patients with LV systolic
dysfunction, hypertension,
diabetes mellitus, or kidney
disease
All patients with LV systolic
dysfu nction, hypertension,
diabetes, or kidney disease
who are intolerant of ACE
inhibitors
Patients with sTEMI who
have an LVEF <40% and
either clinical heart failure
or diabetes
All patients
of CAD and
with evidence
age <75 y
All patients with evidence
of CAD and age >75 y or
otherwise intolerant of
hi gh-i ntensity statin therapy
Comments
Non-enteric formulation
recommended for aspirin-
naive patients with ACS
Clopidogrel is
recommended as an
alternative to aspirin for
patients with stable CAD
and intolerance or allergy
to aspirin
Contraindicated with age
>75 y or history of stroke/
TIA
Dosage adjustmentto 5 mg/d
should be considered for
patients weighing <60 kg
(1321b)
More rapid onset of action;
does not require first-pass
hepatic metabolism;
no known genetic
polymorphisms
lncreased risk for bleeding
with aspirin doses >100 mg
Avoid in patients with
cardiogenic shock,
hypotension, or conduction
disturbances
Particularly beneficial in
patients with anterior Ml
Should not be used in
patients already taking an
ACE inhibitor
Use with caution in patients
with chronic kidney disease
or hyperkalemia
Simvastatin dosage should
be limited to 20 mg/d in
patients taking amlodipine
ACE inhibitors
Angiotensin
receptor blockers
Aldosterone
in hibitor
High-intensity statin
therapy
Moderate-intensity
statin therapy
Atenolol, metoprolol,
carvedilol, nebivolol,
bisoprolol
Benazepril, captopril,
enalapril, fosinopril,
perindopril, trandolapril,
lisinopril, ramipril, quinapril
Losartan, valsartan,
olmesartan, candesartan,
irbesartan. telmisartan
Eplerenone
Atorvastatin
Rosuvastatin
Atorvastatin
Rosuvastatin
Simvastatin
Pravastati n
Lovastatin
Fluvastatin
40-80 mg/d
20-40 mg/d
10-20 mg/d
5-10 mg/d
20-40 mg/d
40-80 mg/d
40 mg/d
40 mg twice daily
(Continued on the nert page)
t
25

38. Coronary Artery Disease
TABLE 10. MedicalTherapy for Acute Coronary Syndromes (Continued)
Medication Drugs in Class Dosage lndications Comments
Antianginal Medications
Nitroglycerin Nitrostat (SL)
Nitronal(lV)
Nondihydropyridine Diltiazem,verapamil
calcium channel
blockers
0.4 mg every 5 min
for a total o{ three
doses
lnitial lV infusion
rate of 5-10 pg/min
Variable
As part of multimodal
treatment of ongoing
pain
Persistent chest pain
following three SL doses
and as part of multimodality
treatment of heart failure,
hypertension
PAtiENtS With NSTE-ACS
who are intolerant of
p-blockers or with angina
refractory to nitrates and
p-blockers
Avoid nitrates with SBP
<90 mm Hg or >30 mm Hg
below baseline,
bradycardia, tachycardia,
RV infarction, PDE-5
inhibitor use within the last
24-48h, HCM, or severe AS
No benefit in patients with
STEMI
May worsen clinical status
with coincidental heart
fa i I u re or LV dysfu ncti on
Avoid with evidence of
heart failure, cardiogenic
shock, or conduction
abnormalities
ity
chest
ACS=acutecoronarysyndrome;A5=aorticstenosis;CAD=coronaryarterydisease;HCM=hypenrophrccardiomyopathy;lV=intravenous;LV=leftventricular;LVEF=left
Antiplatelet Medications
Aspirin (162 325 mg) should be administered at presentation
to all patients with ACS. followed by a daily dose of 81 to
162 mg. Early clopidogrel loading has been recommended in
patients with ACS regardless of reperfusion or revasculariza
tion strategr, although the optimal timing for loading of other
oral antiplatelet agents is less clear. Prasugrel loading before
coronary angiography is not beneficial. Clopidogrel or ticagre
Ior therapy is recommended for 1 year after ACS presentation.
Prasugrel is indicated only in patients treated with PCI
because it is associated with comparable efficacy but increased
bleeding when compared with clopidogrel in medically man
aged patients. Evidence supports continuing DAPT beyond
1 year in patients at high risk for recurrent vascular events,
such as those with depressed LV function, saphenous vein
graft stenting, or diabetes, in whom the benefit exceeds the
bleeding risk.
Intravenous glycoprotein Ilb/llla inhibitors are generally
reserved for use during PCI in patients with evident thrombus at
the time of coronary angiography and intervention. In patients
tolerant of DAPT. upfront administration of glycoprotein Ilbrllla
inhibition in the emergency department is associated with lack
of benefit and increased bleeding risk, and similarly. there is no
proven role for initiation lollowing successful PCI.
Anticoagulant Medications
The choice olanticoagulant during ACS depends on the reper
fusion strategy available to the patient. In patients receiving
thrombolytic therapy, anticoagulation with unfractionated
heparin, enoxaparin, or fondaparinux is associated with
reduced reocclusion ofthe infarct related artery and improved
outcomes.
In patients undergoing PCI for ACS. especially those with
kidney dysfunction, unf'ractionated heparin is favored over
enoxaparin because of the ability to monitor the degree of'
anticoagulation with activated clotting times. Fondaparinux
is not indicated during PCI given the risk lor procedural
thrombosis.
For patients proceeding to the catheterization laboratory,
anticoagulant therapy should be provided until revasculariza
tion with PCI or CABG. In medically treated patients. antico
agulation is recommended for at least 48 hours and is generally
continued until discharge.
Antianginal Medications
Orygen therapy. often considered an antianginal therapy. is
no longer routinely indicated in ACS. Supplemental oxygen in
the setting of normal oxygen saturation is associated with
increased mortality in patients with ACS. Guidelines suggest
initiating oxygen therapy in patients with oxygen saturation of
less than 90% to 92"/,, and maintaining saturation at 93'X, to
96'l,. The American Heart Association. however, recommends
oxygen therapy for an oxygen saturation less than 90'7, or in
the presence of heart failure or dyspnea without providing an
upper oxygen saturation limit.
p Blockers decrease myocardial oxygen demand, reduce
the incidence ol ventricular arrhythmias, and improve long
term survival in patients with ACS. These agents should be
administered orally (not intravenously) within 24 hours of
presentation. except in patients with evidence of hypotension,
cardiogenic shock, pulmonary congestion. or atrioventricular
block.
Nitrates are used primarily to manage angina symptoms in
ACS. Sublingual nitrates should be administered at presentation
25

39. to relieve chest pain and thereby reduce counterproductive
sympathetic drive. For patients with persistent chest pain
despite B-blockade, intravenous nitroglycerin can alleviate
symptoms, particularly in those with hypertension. Patients
receiving nitroglycerin infusions for a prolonged time often
require increased doses because of the development of nitrate
tolerance. Nitrates should be avoided in patients who have had
recent exposure (within 24 48 hours) to phosphodiesterase 5
inhibitors, such as sildenafil. and in those with shock or RV
infarction.
Li p id -Low e r i ng Me d ic at io ns
Statin therapy reduces mortality and adverse clinical event
rates after ACS. High-intensity statin therapy is recommended
because it improves outcomes compared with Iower intensity
treatment. Initiating statins in the inpatient setting is associ
ated with greater medication adherence. Furthermore, statin
preloading bef<-rre PCI has been associated with lower rates of
periprocedural MI.
Angiotensin Receptor and Aldosterone Agents
ACE inhibitors are indicated in patients with ACS, particularly
those with impaired LV function, heart failure, anterior wall
infarction, or diabetes. ARBs may be used in patients intoler
ant of ACE inhibitors. These agents have shown significant
early benefit fbllowing STEMI and should be administered
within the first 24 hours of presentation in the absence of
contraindications.
Eplerenone, an aldosterone antagonist, has proved bene-
ficial in patients with STEMI who have an ejection fiaction of
4O"/,, or less and either heart failure or diabetes, especially
when initiated within l week of presentation. Potassium levels
must be monitored carefully, especially in patients with pre-
existing kidney dysfunction and those receiving concomitant
ACE inhibitors or ARBs.
rEY POI lIIS
HVC r Thrombolytic therapy is not indicated in patients with
non-ST elevation acute coronary syndromes.
o All patients presenting with acute coronary syndrome
should receive a loading dose of aspirin, supplemental
oxygen for oxygen saturation less than 90%, nitrates, a
B blockel an ACE inhibitor, a high-intensity statin, a
P2Y,, inhibitor, and an anticoagulant.
. Dual antiplatelet therapy is indicated for at least 1 year
after acute coronary syndrome (ACS); clopidogrel and
ticagrelor may be used in all patients with ACS, whereas
prasugrel may be used only in patients treated with per-
cutaneous coronary intervention.
o p Blockers decrease myocardial oxygen demand, reduce
the incidence of ventricular arrhythmias, and improve
long-term survival in patients with acute coronary syn
drome; however, these agents are contraindicated with
cardiogenic shock or high-grade atrioventricular block.
Coronary Artery Disease
Acute Coronary Syndromes Not Associated
With Obstructive Coronary Artery Disease
Some patients with chest pain, elevated cardiac troponin levels.
and characteristic ST segment elevation on ECG have MI in the
absence of obstructive CAD (MINOCA). Other related syn
dromes may mimic MINOCA but do not involve frank infarc-
tion. Regardless of whether there is irreversible myocardial
injury. treatment of these conditions with thrombolytic agents
and revascularization in the absence ofocclusive coronary dis-
ease and plaque rupture is not beneficial or recommended.
Spontaneous coronary artery dissection (SCAD) is a com-
mon cause of chest pain among younger women who present
with ACS and in the peripartum period. Although the patho-
physiologr is not clearly established, SCAD involves develop-
ment of a nontraumatic and non iatrogenic intramural
hematoma with or without intimal dissection with luminal
communication. The enlarging hematoma in the false lumen
compresses the true lumen of the coronary artery and in poten-
tial combination with obstructing dissection leads to chest pain,
ischemia. and/or infarction. Diagnosis requires a high index of
suspicion and confirmation by invasive or noninvasive angiog
raphy, using care to minimize unnecessary radiation exposure.
When associated with STEMI, SCAD may be managed inva-
sively; CABG, however, is rarely indicated. PCI may be safely
deferred when coronary flow is preserved and symptoms can be
controlled and closely monitored, because acute vascular
manipulation often results in dissection extension, and early
vascular healing often occurs without further intervention.
Coronary vasospasm is sudden coronary artery constric-
tion occurring spontaneously or lollowing use of illicit sub
stances (methamphetamines, cocaine) or prescription drugs
(5 fluorouracil, bromocriptine). Spontaneous coronary vaso
spasm often occurs at night and may occur at rest or following
exercise. ECG abnormalities may be nonspecific or mimic
STEMI patterns. Coronary vasospasm is a diagnosis of exclu
sion and often involves coronary angiography to exclude fixed
disease. Invasive provocative testing can be performed but
usually is not indicated. Patients suspected of having vaso
spasm (or the related microvascular endothelial dysfunction)
often are treated empirically with nitrates and/or calcium
channel blockers.
Cardiac syndrome X is a poorly defined condition charac-
terized by anginal chest pain in the presence ofangiographi-
cally normal coronary arteries or insignificant CAD (<50'1,
stenosis). Several hypotheses have been proposed to explain
the pathogenesis of this syndrome, with one of the most
accepted centering on microvascular dysfunction as the cause.
Cardiac syndrome X is a frequent cause of chest pain syn-
dromes in women and may be associated with adverse out
comes despite the frequent lack of traditional risk factors for
CAD. Patients may be treated with p blockers. calcium chan-
nel blockers, nitrates, and ranolazine.
Takotsubo cardiomyopathy presents as acute chest
pain, ECG changes (often ST-segment elevation), and elevated
27

40. Coronary Artery Disease
cardiac enzyme levels in the absence of coronary occlusion.
Takotsubo cardiomyopathy more commonly occurs in women,
and there is often an antecedent psychological or physical
stressor. It is a diagnosis of exclusion based on lack of signifi-
cant coronary stenosis with significant wall motion abnormal
ity (often systolic apical ballooning and notable sparing ofthe
base ofthe heart) on echocardiography or ventriculography.
Care After an Acute Coronary Syndrome
Ali patients with ACS should continue aspirin, preferably
81 mg/d, indefinitely. DAPT is recommended for at least l year
(see Table 10). There is some evidence fbr extending DAPT
beyond 1 year; however, the decision to prolong therapy should
be individualized, with the risk for bleeding weighed against
the risk fbr thrombosis.
Statin therapy should continue indefinitely. Patients with
a history of multiple major atherosclerotic cardiovascular dis
ease (ASCVD) events or one major ASCVD event and multiple
high risk conditions are judged to be at very high risk for
recurrent ASCVD events. These patients may benefit from the
addition of nonstatin drug therapy to maximally tolerated
statin therapy (see N4KSAP 19 Ceneral Internal Medicine 1).
B Blockers should be continued indefinitely in all
patients. ACE inhibitors should be continued indeflnitely in
patients with LV dysfunction and may be beneficial in patients
with preserved LV function, especially in those with diabetes.
Guidelines recommend avoiding NSAIDs if possible, because
of the increased cardiovascular risk associated with these
drugs.
In patients at risk for sudden cardiac death after MI, guide
iines do not recommend placement of an implantable cardio
verter defibrillator for primary prevention within 40 days
of infarction because of lack of benefit. Despite a theoretical
primary prevention benefit in high-risk patients following MI.
routine use of a temporary wearable cardioverter defibrillator
has not shown benefit in reducing arrhyhmic death within
84 days of MI in the large, randomized VEST study. There are
no clear indications for a wearable cardioverler defibrillator:
however, it is an option for select patients with a high risk for
sudden death before implantation of a permanent deflbrillator
device.
Patients with ACS should be refbrred for cardiac rehabili
tation, a medically observed exercise program that reduces
mortality while improving functional capacity, medication
adherence, and risk factor profiies. Patients at low risk (aged
<75 years with symptoms less than New York Heart Association
functional class III to IV a normal ejection fraction, and no
arrhythmia) who prefer home based cardiac rehabilitation
with remote coaching and indirect exercise supervision ben
efit as well.
Sexual activity can generally resume after 2 weeks in
younger, previously fit patients with uncomplicated MI and
after 6 weeks in patients with more extensive infarcts,
assuming other aspects of cardiac rehabilitation are pro
ceeding uneventfully. Patients should be counseled that
erectile dysfunction and decreased satisfaction during sex
ual activity are not uncommon following MI. Drug therapy
for erectile dysfunction is effective and safe for patients who
are not taking nitrates (see I\4KSAP 19 General Internal
Medicine 2).
Physicians should counsel patients that treatment goals
after MI are focused on risk reduction and achieving baseline
functional status, although that expectation must be tem-
pered in those with post MI heart failure or significant
reduction in ejection fraction. Depression after MI is com
mon, and screening is recommended; cognitive behavioral
therapy and selective serotonin reuptake inhibitors are first
line therapies.
rtY PornI
. All patients with acute coronary syndrome should be
referred fbr cardiac rehabilitation. which reduces mor
tality while improving functional capacity, medication
adherence, and risk factor profiles.
Management of Coronary Artery
Disease in Specific Populations
Patients With Asymptomatic Vascular Disease
Silent myocardial ischemia is a long recognized syndrome
with widely varying prevalence and risk estimates, and its
recognition is increasing with the use of diagnostic chest
imaging and dedicated coronary artery calcium scoring stud
ies. Although it is clear that patients with silent myocardial
ischemia, especially those with known underlying heart dis
ease, have a worse prognosis, there are no guideline recom
mendations on which patients should be evaluated with stress
testing, ambulatory monitoring, or cardiac catheterization nor
on how establishing the diagnosis modifles secondary preven
tion opportunities.
Patients Older Than 75 Years
Patients older than 75 years are at heightened cardiovascular
risk because of a higher prevalence and greater severity and
extent of CAD. Comorbid conditions, loss of muscle mass, and
gait abnormalities often limit or prohibit the use of exercise
stress testing for prognostic and diagnostic purposes. When
cardiovascular disease is identified. older adults often are not
treated with recognized secondary prevention therapies (e.g.,
antiplatelet agents, B-blockers, ACE inhibitors, and statins).
This is at least partly due a perceived heightened risk for
adverse therapeutic response compounded by the lack of out
come data in these patients, who are often excluded from
prospective trials. Furthermore, invasive angiography and
revascularization are used less often in older adults, likely due
to higher procedural morbidi[z and mortality. Exercise, diet,
and healthy lifestyle choices remain essential elements of risk
reduction in all age groups, with antiplatelet therapy, Iipid
lowering therapy, blood pressure control, and glycemic control
28

41. of benefit in patients at suitable risk. regardless ol age.
Hyperlipidemia remains associated with adverse outcomes in
the elderly, with a recent meta analysis of primary and sec
ondary prevention trials demonstrating reduction in cardiac
events and disabiliry in this group.
Women
Clinical Presentation
Women usually develop ischemic heart disease at an older age
than men and more commonly present with stable CAD than
ACS. In women with typical angina symptoms, nonobstructive
coronary stenoses are present on coronary angiography in
more than 50'7, of cases, and microvascular dysfunction
(endothelium-dependent or endothelium independent) is
thought to be a predominant cause of symptoms in these
patients. In women with acute MI. the predominant symptom
is chest pain or pressurei however, women can have atypical
symptoms, such as fatigue, dyspnea. nausea, or abdominal
symptoms.
Several unique manifestations of cardiovascular disease,
including SCAD, takotsubo cardiomyopathy, and coronary
vasospasm. occur primarily in women.
Evaluation and Treatment
Diagnostic and therapeutic recommendations for CAD do not
differ for men and women. Noninvasive stress testing has a
lower sensitivity and specificity in women than in men, and
exertional ST segment deviation has a lower reported accuracy
in women.
Reports from observational studies and secondary analy
sis of randomized controlled trials suggest that women have
worse outcomes after STEMI presentation. possibly due to
delays in recognition and longer ischemic time, as well as a
recognized increase in complication rates after reperfusion
therapy for STEMI.
Patients With Diabetes Mellitus
Risk and Evaluation
Diabetes is associated with a two to threefold increase in age
adjusted risk for CAD events, including cardiovascular mortal
ity. This increased risk is multifactorial and is associated with
a higher incidence of cardiovascular risk factors as well as
underlying vascular and myocardial dysfunction accompany
ing the diabetic state.
Evaluation and diagnostic strategy for CAD are similar
in patients with or without diabetes. although cardiac
symptoms such as dyspnea and nausea are more common.
requiring a high index of suspicion in at risk patients. The
diagnostic accuracy of noninvasive stress testing in sympto
matic patients with diabetes is similar to that in patients
without diabetes. Although traditional CAD risk factors
should be aglressively managed in all patients with diabetes.
screening for CAD in asymptomatic persons is controversial
and without proven outcome beneflt: thus, routine stress
testing is not recommended.
Coronary Artery Disease
Medical Therapy and Secondary Prevention
Optimal medical therapy, including aggressive risk factor
reduction. glucose control, and antianginal therapy. is founda
tional for patients with diabetes and CAD. Although specific
blood pressure goals remain controversial, a blood pressure
goal ofl less than l4Ol90 mm Hg provides a balance of safety
and efficacy for most patients with diabetes. The American
Diabetes Association (ADA) recommends a blood pressure
target of less than 140/90 mm Hg in patients at lower risk for
ASCVD and suggests that a blood pressure target ofless than
130,80 mm Hg may be appropriate for patients with either
existing ASCVD or a l0 year ASCVD risk of lS'X, or greater. if
it can be achieved without undue treatment burden. The
American College of Cardiologr/American Heart Association
guidelines recommend antihypertensive treatment rith a tar
get blood pressure below 130/80 mm Hg in patients (ith dia-
betes. ACE inhibitors or ARBs are the preferred antihyperten
sive therapies given their renal protective effects. High intensity
statin therapy is indicated in patients with diabetes and CAD.
with the addition of ezetimibe or PCSK9 inhibitors as needed
for optimal lipid control.
Aspirin is recommended for secondary prevention in all
patients with diabetes and CAD. The role of P2Y,, inhibition
(clopidogrel or ticagrelor) with or without aspirin continues to
evolve, with evidence supporting a secondary prevention ben-
efit at the expense of excess bleeding.
Tight glycemic control reduces microvascular compli-
cations but does not reduce the risk lor Ml. particularly
when initiated late in the disease course. The use of sodium
glucose cotransporter 2 (SGLT2) inhibitors or glucagon like
peptide 1 (GLP 1) receptor agonists in patients with type 2
diabetes reduces rates ofacute MI, stroke, and cardiovascu
lar death. Based on strong evidence. the ADA recommends
introducing an SGLT2 inhibitor or a GLP I receptor agonist
with demonstrated cardiovascular disease benefit as part of
a glycemic control regimen in patients r.t,ith type 2 diabetes
and clinical ASCVD. If the patient is already taking met
formin combined with another therapeutic agent or agents
and not taking an SGLT2 inhibitor or GLP I receptor ago
nist. the ADA recommends considering switching to one of
these agents.
Invasive Treatment
Outcome following both surgical and percutaneous revas-
cularization is impaired in patients with diabetes. The
choice of revascularization strategv is based on the severity
and extent of CAD. comorbid conditions. and patient pref
erence. recognizing that CABG is associated with improved
outcomes, including lower rates of repeat revascularization
and the composite of death. MI. or stroke. Regardless of the
strateg), employed. optimal technique is essential. including
drug eluting stent placement in PCI and arterial conduit
use in CABG. Optimal medical therapy has been shown to
provide lasting benefit independent of revascularization
modality.
29

42. Heart Failure
I(EY POIilTS
HVC o Stress testing is not routinely recommended in asymp-
tomatic patients with diabetes mellitus to detect sub
clinical coronary artery disease.
. [n patients with type 2 diabetes mellitus and clinical
atherosclerotic cardiovascular disease, a sodium-glucose
cotransporter 2 inhibitor or glucagon-like peptide I
receptor agonist with demonstrated cardiovascular dis-
ease benefit should be initiated as part of a glycemic
control regimen.
o Coronary artery bypass grafting is associated with
improved outcomes, including lower rates of repeat
revascularization and the composite of death, myocar
dial infarction, or stroke, in patients with diabetes mel
I itus undergoing revascularization.
Heart Failure
Pathophysiology of Heart Failure
Heart failure is a clinical syndrome characterized by signs and
symptoms of fluid overload and decreased cardiac output. It can
result tiom any structural or lunctional impairment in ejection
of blood (systolic dysfunction) or ventricular filling and/or relax
ation (diastolic dysfunction). Systolic and diastolic dysfunction
both result in increased left ventricular (LV) filling pressures.
causing the classic signs and symptoms of heart failure, includ
ing dyspnea, paroxysmal nocturnal dyspnea, orthopnea, periph
eral edema, crackles, elevated central venous pressure. and an S,.
In systolic dysfunction, LV ejection fraction (LVEF) is reduced,
termed heart failure with reduced ejection fraction (HFTEF).
IIFTEF is fbrmally defined by an LVEF of 40"/,, or less. Common
causes of HFrEF include coronary artery disease (CAD), hyperten
si<-rn, obesity, diabetes mellitus, and vahular heart disease.
In response to a reduced ejection fraction, neurohormo
nal systems, including the renin angiotensin aldosterone sys
tem (RAAS) and sympathetic nervous system. are activated.
RAAS activation results in the production of angiotensin II and
aldosterone. Angiotensin ll causes vasoconstriction, thereby
increasing blood pressure, and also stimulates thirst; aldoster
one increases fluid retention by increasing sodium resorption.
Stimulation of the sympathetic nerv<.rus system results in the
release of'epinephrine and norepinephrine, which cause an
increase in heart rate, contractility, and vascular resistance.
Enhanced sympathetic activity and increased angiotensin II
levels stimulate vasopressin release, causing additional fluicl
retention. Initially, this response is adaptive and maintains an
adequate cardiac output and blood pressure; however, it
becomes chronic and maladaptive in the long term.
(lhronic
activation olthe neurohormonal systems in HFrEF
not only causes hemodynamic alterations by vasoconstriction
ancl tluid overload but also is responsible fbr structural and
firnctional changes in the individual myocytes, ultimately
leacling to worsening global tV function and dilation. This pro
cess is referred to as ventricular remodeling. These deleterious
changes result in a cycle of slowly worsening LV function n ith
decreasing lorward flow and increasing pulmonary and right
sided pressures.
Diastolic dysfunction usuirlly is characterized by a stiff
ened [V with abnormal relaxation cluring diastole. resulting in
an increase 1n 1y preload. t.VFlF classically remains normal in
this setting. knort,n as heart failure rvith presened ejection
fraction (HFpEF). HFpEF is ctimmonly defined by an LVEF of'
50'X, or greater. Hypertension is the most common causer how
ever, aging. obesity diabetes mellitus. atrial fibrillation. and
CAD are also contributors. Recently. amyloid deposits. due to
wild type or mutation in the transthyretin gene, have been
reported in more than 10'7, olpatients with HFpEF.
lleart failure with mid range ejection tiaction (HFmEF)
has been proposed as a category to clescribe patients rvith
LVEF between 40',1, and 50'){,. HFmEF includes up to 25'1, of all
patients with heart failure. There have been fen,clinical trials
evaluating therapy outcomes in these patients.
t(EY POIilIS
. Common causes of heart failure with reduced ejection
fraction include coronary artery disease, hypertension,
obesity, diabetes mellitus, and valvular heart disease.
. Hypertension is the most common cause of heart failure
with preserved ejection fraction.
Screening
A validated sex and race specific l0 \'ear Pooled Cohort
Equ:rtion to Prevent Heart F'ailure risk score can be used in
primary care settings to assist in iclentifying asymptomatic
patients at increased risk fbr heart failure who may merit
intensive screening and,ror targeted prevention strategies
(http:i,hf risk calculator.surge.shi).
In patients at risk fbr developing heart failure (such as
those with hypertension, diabetes. or vascular disease). natriu
retic peptide biomarker basecl screening can be useful to pre
vent the development of IV dysfunction. Small studies have
shown that in asymptomatic persons n,ith elevated B hpe
natriuretic peptide (BNP) or N terminal pro B lvpe natriuretic
peptide (NT proBNP) levels. team based care and aggressive
guideline based medical therapy involving a cardiovascular
specialist can help prevent future Ily' dysfunction or new onset
heart failure; however. there is n<t agreed on standard Ibr sr-rch
screening and no certainty as to its cost effectiveness.
Diagnosis and Evaluation
of Heart Failure
Clinical Evaluation
Clinical evaluation ofpatients suspected ofhaving heart failure
should include a comprehensive history and physical exami
nation, tbcusing on potential risk f:rctors and assessment of.
30

43. flr-rid irnd perfusion status. ileiirl frrilurc r-rrost contr-nonly ltre
scnts with signs and syntptolns of' rrolunte overload u,ith
norr-nal cardiac output. Less cor.r.rntorllyl volume or,erloird is
acconrpanied by low cardiac olltput, and rarely, patients l.rave
Itiw cardiac output without v<tlr"rr-nc overktad.
Volume overload is suggestecl ltv svntptoms such as r,reigl-rt
gitin. dyspnea, orthopnea, and pirroxl,sn-ral r-rocturnal dyspnea.
l3enclopnea (shortness of breath n,hen leaning over) is :rssoci
atecl with elevated filling pressr-rr-es. particularly in the setling
of reduced cardiac index. S1'nrptr)n1s typicall),progress fionr
exertional dyspnea to orthopnel lncl paroxl,smal nocturnal
dyspr.rea. Supportive exanrination f)ndings ir-rclude elevated
central venous pressure, cdcnla. lbdonrinal distention fronr
asciles, crackles or findings consistent with pleural eflusion on
lung examination, and an S.,.
l.o'nv cardiac output is signaled by hypotension, l61ar pulse
pressure, cool extremities, irncl reclucecl cogr-rition. Although
r,r,orsening kidney or liver fur-rction rlay be a sign of iolr,car
diac output. end organ clystirnction also can be causecl lry
virscular congestion.
Diagnosis
Initiirl diagnostic testillg for heart failure includes ECG, chest
radiography, and select laboratory str-rdies. An ECG can be
helptul in evaluating for possiblc myocardial infarction, tach
yarrhythmia. or LV hypertroplry. Ohest radiography may reveal
fir.rdir.rgs that support he:rrt failure. including cardiomegaly.
vascular congestion. engorged lynrphatics (Kerley B lines)
(Figure 12). and pleural efTusion. or nray exclude alternative
pulmonary causes of dyspnea.
In patients presenting ulith dyspnea of'unknou,n etiologi
BN['] or NT proBNP ]evel c:rn eflectively differentiate cardiac
fiom pulmonary causes. BNP levels are elevated in patier.rts
F I G U R E 1 2. Chest radiograph showing evidence o{ decompensated heart
failure with pulmonary edema characterized by difluse increased interstitial
opacities, including Kerley B lines; prominent and indistinct pulmonary vasculature;
and tiny pleural efiusions.
Heart Failure
with increased filling pressures and heart failure (typically
>,100 pglrrl-). rhereas BN [' levels are low to normal in paticlrts
with pulmonary disease (typically <100 pg/ml.). BNP levels
tencl to be lower in tlFpFiF than in tlFrEFl, Studies have shor.r,rr
that an elevated BNP level l.ras a sensitivity for heart f'ailure o{'
95'X, to 97"/,, and a negative predictive value of 9O'/,, lct
L)7'/,.
l.evels betr.teen 100 pgi n.rL and ,100 pg/ml- are not particulxrly
sensitive or specific in diagnosing or excluding heart f:rilure.
Other factors that increase BN[) levels include kidney failur-e.
older age. sepsis. r.r.redical therapy with an angiotensin
receptor neprilysin inhibikrr (ARNI), and female sex. BN['
levels are fypically reducecl in patients with an elevated BMl.
Laboratory assessnlent also should inciude a cor-r-rplete
blood count, serum electnrlytes and kidney function tests.
glucose and lipid levels, liver chenristry tests, and serunr
thyroid stimulating hormone ('l'Stl) level. TSH measurement
is indicated to evaluate fbr occult hypothyroidism or hyperthy
nridism as a reversible cause of heart failure. Evaluatior-r ftlr
other causes. such as hen.tochronratosis, HIV rheun.ratologic
disease. amyloidosis, and pheochromocytoma, should only bc
pcrfbrr-r-red u'her-r clinical suspicion warrants, not routitrely.
lJchocardiography is the prinrary diagnostic modality fbr
evaluatior-r of hearl failure. lt provides infbrmation on chanrber
size and thickness, systrilic and diastolic function, and valvular
pathotogr. Echocardiographic tindings additionally may pK)
vide clues to the underlying cause of heart failure. Regional
wall motion abnormalities increase suspicion fbr CAI).
whereas changes in the myoc:rrdium may suggest conditions
such as cardiac amyloidosis. l)rognostic information can bc
gained from echocardiography. particularly in the setting of
severely depressed tVEF.
Cardiac magnetic resonance (CMR) imaging is fieqr-rer.rtly
used to assess for myocarditis and intjltrative processes, st-tcl't
as her.nochromatosis, sarcoidosis, and amyloidosis. Currcttt
guidelines do not reconrnrend routine CMR imaging; it slrttulcl
be used only in the search tbr a specific diagnosis.
The HTFPEF risk score is a means of assessing the likeli
hrxrd of HFpEF and is used to discrin.rinate cardiac versus
noncardiac causes of dyspnea. Predictive variables include
obesity (2 points). atrial fibrillatior-r (3 points). age older than
60 years (l point). treatnrent rt,ith two or more antihyperten
sive drugs (1 point), echocardiographic Ere'ratio greater thrlr
9 (l point), and echocardiographic estimated pultt.tonary
artery systolic pressure higher than 35 mm Hg (1 point). l'hc
odds of HFpEF double for each l unit score increase, with thc
probrrbility of HFpEF <-r1 0.2 at a score ol1 increasing to 0.95 at
l score of 7.
Evaluation for lschemia
CAI) is the leading cause of heart firilure in the United States
(>.50',1, of patients) ar.rd should be considered in all patierlts
urith neu,ly diagnosed heart failure. The decision to evaluate
firr CAD with stress testiltg or coronary angiograllhy
depends on the patient's sylrptoms and risk factors (diabc
tes, hypertension, tobacco use, family history, male sex);
31

44. Heart Failure
findings on ECG and echocardiogrant also may suggest an
ischen.ric cause for LV dysfunction.
Patients with exertional chest pain, history of myocar
dial infarction, or other symptoms suggesting CAD should
undergo further evaluation with tunctional or anatomic
assessment for obstructive CAD as clinically appropriate (see
Diagnostic Testing in Cardiology). Identification of significant
CAD is important because LV dysf unction caused by ischemia
may lessen or resolve with appropriate medical therapl' or
revascu I a ri zat ion.
Classification
The severity of heart failure is categorized according to the
New York Heart Association (NYt{A) functional classifica
tion (Table 11) and the American College of Cardiology
(ACC)/American Heart Association (AHA) heart failure
stages (Table 12). Patients can move between NYHA classes
depending on fluid status and progression of heart failure:
however, they can only progress in the ACC,'AHA stages.
Both the patient's functional class and stage affect the choice
<-rf therapy.
Class Description
No limitations of physical activity
Slight limitation of physical activity
Marked limitation of physical activity
Symptoms with less than ordinary activity
Symptoms with minimal exertion
Unable to carry on any physical activity without
symptoms
Stage Description
At risk for heart failure but without structural heart
changes (e.9., patients with diabetes mellitus,
coronary artery disease, hypertension, or vascular
disease)
Structural heart disease (e.9., reduced ejection
fraction, left ventricu lar hypertrophy, cham ber
enlargement) but without heart failure symptoms
Structural heart disease with current or prior heart
failure symptoms
Refractory heart failure requiring advanced
intervention (e.9., biventricular pacemakel left
ventricular assist device, transplantation)
lnformation from Hunt SA, Abraham WT, Ch n MH, et al. 2009 Focused update
incorporated into the ACC/AHA 2005 gu delines for the dragnosis and management
of hean failure ln adults: a report o{ the American Col ege of Cardiology Foundation/
American Heart Assoc atlon Task Force on Practrce Guidelrnes: developed in
colLaboration with the lnternational Society for Heaft and Lung Transplanrarron.
I Crculation.2009;119:e391479.IPMID:19324966]doi:10.1161/CIRCULATIONAHA.
109.192065
TEY POIXIS
. B type natriuretic peptide measurement is a sensitive
and specific test for the diagnosis of heart failure in
patients with dyspnea of unknown etiolosr.
o Echocardiography is the primary diagnostic modality
fbr er,aluation of heart failurel it provides information
on chamber size and thickness. systolic and diastolic
function. valvular patholos/, and regional $all motion
abnormalities.
Management
Heart Failure With Reduced Ejection Fraction
'fhe
treatment of HFrEF includes long term therap]' to
decreirse morbidity and mortality and reliere s!'mptoms as
r,r,ell as nlanagement of acute exacerbations. Treatment of
stage B IIFTEF entails RAAS inhibition (ACE inhibitor. ARB. or
ARNI) and p blockade; therapies fbr stage C (s1'mptomatic)
[]FrEt are outlined in Figure 13.
Medical Therapy
ACE lnhibitors and Angiotensin Receptor Blockers
ACE inhibitors reduce morbidity and mofiality in patients
with tlFrEF and should be used in both symptomatic and
,synlptomatic patients. ACE inhibitors block the conversion of
angiotensin I to angiotensin II. inhibiting the upregulation ol
the RAAS pathway. The primary adverse effects are hypoten
sion. kidney dysfunction. ACE inhibitor induced cough. and
angioedema.
Although an ACE inhibitor should be considered in every'
patient \,ith HFTEF. worsening kidney function and h1'per
kalen.ria may prevent its use. Many physicians do not recom
n.rer.rd initiating or increasing the dosage if the creatinine level
rises to 2.5 mg/dl (221 pmol/L) or the estimated glomerular
filtration rate (eGFR) falls below 30 mlrminil.73 m2.
'l'he
eGFR should be monitored fbr decline during uptitration of'
therapy.
An ACE inhibitor induced cough. occurring in up to
20')1, of patients, is the primary reason to su,itch therapy from
an ACE inhibitor to an angiotensin receptor blocker (ARB).
Although fen,er data support the use of ARBs in asymptomatic
patients with reduced LVEFI there is consensus that all patients
with an ACE inhibitor induced cough should receive an ARB.
Patients who develop angioedema lvhile taking an ACE inhibi
tor are often switched to an ARB: however. there are rare
reports of ARB induced angioedema. and patients should be
infbrmed of this risk.
Angiotensin Receptor Neprilysin lnhibitor
'lhe
ARNI valsartan sacubitril belongs to a ne\' drug class
that combines an ARB with a nepril_rsin inhibitor. Nepril-rsin
is a neutral endopeptidase that degrades natriuretic peptides
and bradykinin. Inhibition of neprilysin increases levels ol
these substances, leading to enhanced diuresis, natriuresis,
iltA
iltB
Stage A
Stage B
Stage C
Stage D
Classification
TABLE 1 1 . New York Heart Association Functional
TABLE 12. American College of Cardiology/American
Heaft Association Stages of Heart Failure
32

45. L
t
L
t
L
t
t
t
t
i
I
I
Heart Failure
receptor-neprilysin inhibitor; eGFR = estimated glomerularfiltration rate; HR = heart rate; NYHA= NewYork HeartAssociation; SGLT2 = sodium,glucose cotransporter 2.
'An
ACEI/ARB should only be considered in patients with contraindications, intolerance, or inaccessibility to an ARNI.
bCaruedilol.netoprolol
succinate.o, b'soprolo..
'tnsure eGtR >30 mUmin/1 73 m2 for dapagliflozin and eGFR >20 mUmin/1 73 m'? for empaglillozin before initiation
College of Cardiology Foundation.
and myocardial relaxation. In the PARADIGM HF trial of
patients with symptomatic heart failure and LVEF below
40'X,, valsartan-sacubitril reduced mortality and heart failure
hospitalizationby 2O'1, compared with enalapril. In patients
taking valsartan sacubitril, the incidence of hypotension
was increased, while acute kidney injury hyperkalemia, and
cough were reduced.
Guidelines recommend replacing an ACE inhibitor or
ARB with valsartan sacubitril in patients with chronic symp
tomatic HFrEF (NYHA class II-IV heart failure symptoms and
LVEF <40'1,) or initiating valsartan-sacubitril instead of an ACE
inhibitor or ARB in patients with new-onset HFTEF. The FDA
has expanded the indications for valsartan sacubitril to
include patients with chronic heart failure and any LVEF,
although the benefits are most evident in patients with LVEF
below normal. Caution should be used in patients with hypo-
tension. Valsartan sacubitril should not be administered to
patients with a history of angioedema (even if tolerant of an
ARB) or within 36 hours of the last dose of an ACE inhibitor
because of the risk for angioedema with the combination of an
ACE inhibitor and ARNI.
B Blockers
p Blockers should be initiated in all patients with HFTEF.
p Blockers improve remodeling, increase LVEE, and reduce
hospitalization and mortality when added to ACE inhibitor
HFrEF Stage C Treatment
(ARNI prefened') AND
evidence-based p-blockeP
with diuretic agent
ARNI/ACEI/ARB
as needed
For patients
meeting eGFR
criteria", NYHA
class ll-lV
For patients with
persistent volume
overload, NYHA
class ll-lV
For patients with
eGFR >30/mVmin/1.73 m2 or
creatinine
s2.5 mg/dL(221 pmol/L)
in males or <2.0 mg/dL
(176.8 pmol/L) in females or
K. <5.0 mEq/L (5.0 mmol/L),
NYHA class ll-lV
For persistently
symptomatic Black
patients despite
ARNI/p-blocker/
aldosterone
antagonisVSGLT2
inhibitor, NYHA
class lll-lV
For patients with
resting HR >70/min,
on maximally
tolerated p-blocker
dose in
sinus rhythm,
NYHA class ll-lll
Add Titrate
Diuretic
agent
AddAdd
Aldosterone
antagonist
5GLT2
inhibitor
Add
Hydralazine
+ isosorbide
dinitrate
lvabradine
33

46. TABLE 1 3. Therapeutic Dosages of B-Blockers
for
Treatment of Heart Failure With Reduced Ejection Fraction
Heart Failure
Agent Target Dosage
Carvedilol 25 mg twice daily (50 mg twice daily if
weight >85 k9 [187 lb))
200 mg dailyMetoprolol
succrnate
Bisoprolol 10 mg daily
and diuretic therapy. In contrast to ACE inhibitors, the benefits
of p blocker therapy do not appear to be a class effect, and one
of three agents shown to have a mortality benefit (bisoprolol,
carvedilol, and metoprolol succinate) should be used.
p-Blockers are generally well tolerated but should not be
started when the patient is acutely decompensated. These
agents have negative inotropic properties and may exacerbate
heart failure in patients with acute volume overload. p blockers
should be initiated at low doses and slowly uptitrated over
weeks (not days) until the patient achieves the guideline
directed target dose or maximum tolerable dose (Table 13)
while monitoring for light headedness. hypotension. and
bradyarrhythmia. Contraindications to B-blocker therapy
include cardiogenic shock and second- or third degree atrio-
ventricular block. In patients with reactive airways disease or
COPD, B-blockers should not be initiated if the patient has
acute bronchospasm or evidence of pulmonary disease exac
erbation. Hospitalized patients generally should be started on
p blocker therapy before discharge.
Initiating and Managing ACE lnhibitor
andB BlockerTherapy
An ACE inhibitor (or ARB or ARNI) and a p blocker are indi
cated in all patients with HFTEF. Either drug may be initiated
first, although it is reasonable to select the first agent based on
patient factors. For example, a B blocker should be started first
in patients with CAD or atrial fibrillation who require heart
rate control. Conversely, an ACE inhibitor should be started
first in patients with diabetes for the additional renal benefits.
Studies have shown that patients receive additive benefit from
the second agent regardless ofthe order ofinitiationi however,
the second drug should be started befbre the dosage of the first
agent is maximized, especially if the patient has or is at risk for
hypotension. For both drugs, studies suggest that higher doses
are associated with reduced hospitalizations and improved
symptoms.
Aldosterone Antagonists
Aldosterone antagonists (spironolactone, eplerenone) reduce
mortality and heart failure hospitalizations in patients with
symptomatic HFrEF (NYHA functional class II IV) and in
patients with HFrEF after acute myocardial infarction. Despite
their proven efficacy, they are underused. Current guidelines
recommend initiating these agents in patients with sympto
matic HFrEF and an eGFR of at least 30 ml/min/1.73 m2.
Aldosterone antagonists can cause hyperkalemia and
should be used cautiously in patients with kidnel'dysfunc
tion. In clinical trials, potassium supplementation \^Ias rou
tinely discontinued at the beginning oftherapy. and electrolyte
measurement was repeated within I week of initiation.
Aldosterone antagonists should not be considered diuretic
therapy. and patients with volume overload should be treated
with a loop or thiazide diuretic. Of the two agents. eplerenone
is a more selective aldosterone antagonist and is associated
with lort'er incidence of grnecomastia and breast tenderness.
Isoso rbide D initrate -
H y d ralazine
In Black patients with NYHA functional class III to lV symp
toms. isosorbide dinitrate hydralazine used in combination
with an ACE inhibitor, p-blocker, and aldosterone antagonist
has been shown to reduce mortality compared with placebo.
Guidelines recommend the addition of isosorbide dinitrate
hydralazine in Black patients who remain s)'mptomatic on
maximal doses of a B-blocker:
ACE inhibitor. ARB. or ARNI:
and aldosterone antagonist. In patients who are intolerant of
ACE inhibitor or ARB therapy. especially those u'ith chronic
kidney disease, isosorbide dinitrate hydralazine may be
considered as a therapeutic option. Headache is a common
adverse effect.
Because patients may have difficulty adhering to a thrice
daily dosing regimen, physicians might consider switching to
once daily therapy with isosorbide mononitrate. However.
there is no experimental evidence that isosorbide mononitrate
plus hydralazine is as effective as isosorbide dinitrate plus
hydralazine or that adherence is improved.
Diuretics
Loop diuretics are the primary diuretic therapy for volume over
load in heart failure because of increased potency compared
with other diuretics. Of the four loop diuretics, furosemide is
most commonly used; however, some studies have shown tor
semide and bumetanide to be more effective because of their
increased bioavailability and longer half lives. Occasionally. loop
and thiazide diuretics are combined to potentiate diuresis. The
lowest dosage that achieves euvolemia should be used. Major
side effects include hypokalemia and hypomagnesemia.
Electrollte levels should be monitored. In patients lvho are eu
volemic following diuresis and able to adhere to a rigorous salt
restricted diet, diuretic dosage may be reduced or discontinued.
luabradine
Ivabradine is a sinoatrial node modulator that selectively
inhibits the 11 current in the sinoatrial node. causing a reduc
tion in heart rate. It has no negative inotropic effects. In
patients with HFrEF (LVEF <35')(,) and NYHA functional class
II to III symptoms who are in sinus rhyhm u,ith a heart rate
ol at least 7Oimin and taking maximally tolerated doses of a
p blocker, ivabradine reduces heart failure associated hospi
talizations and the combined end point of mortality and heart
tailure hospitalization.
34

47. Digoxin
Digoxin reduces the risk fbr hearl failure hospitalization but does
not reduce mortality. It is occasionally used in patients with
HFrEF and concomitant atrial fibrillation for rate control and in
patients with heart failure symptoms refractory to optimal ther
apy with an ACE inhibitor and B blocker. Because of its associ.
ated toxicity, digoxin should be managed carefully, and drug
levels should be monitored. Toxicity is more common in patients
with impaired kidney lunction, older adults, and women.
Calcium Channel Blockers
The nondihydropyridine calciunr channel blockers verapamil
and diltiazem are detrimental in patients with HFTEF, proba
bly related to negative inotropic eflfects, and should not be
used. Amlodipine and f'elodipine have shown neither benefit
nor harm and can be used in patients with persistent hyper
tension despite therapy with other agents at maximal dosage.
Stotins
Two large clinical trials have shown no benefit'a.hen rosuva-
statin is added to standard medical therapy in patients with
lIFrEF, and statins should not be used routinely in patients
with heart failure without another accepted indication.
Moderate intensity statin therapy can be considered in
patients with HFrEF attributable to ischemic heart disease if
life expectancy is reasonable (S 5 years).
Sodium-Glu cose Cotransporter 2 Inhibitors
Glucose lowering medications have been evaluated fbr their
effect on cardiovascular outcomes, including heafi failure
(Table 14). Multiple studies have documented significant reduc
tions in cardiovascular events with use of sodium glucose
Heart Failure
cotransporter 2 (SGLI2) inhibitors (empagliflozin, canagliflozin,
and dapagliflozin). SGLI2 inhibitors also appear to reduce the risk
for heart failure hospitalization by 35'1,. The mechanisms of this
reduction are unclear but are thought to be independent of
increased diuresis due to glucose excretion. The DAPA HF trial
showed that dapagliflozin reduced the risk for worsening heafi
failure or cardiovascular death in patients with HFTEE, independ
ent of underVing glucose control. SGLI2 inhibitors should be
initiated for patients with hearl lailure and NYHA functional class
II to IV symptoms with or without tlpe 2 diabetes, in addition to
guideline directed medical therapy, to reduce the risk for worsen
ing hearl failure and cardiovascular death. An eGFR of at least
30 ml/min/1.73 m2 for dapagliflozin and at least 20 ml/min/
1.73 m2 for empagliflozin is recommended before initiation.
lron Therapy
All patients should be evaluated at baseline for anemia, which
is independently associated with heart failure severity. Iron
deficiency has been linked with reduced lunctional capacity,
and multiple small studies in patients with heart failure and
concomitant iron deficiency have shown an improvement in
f'unctional capacity and quality of life with iron replacement.
Some experts contend that impaired iron absorption should
prompt intravenous rather than oral iron therapy.
t(EY POtltTS
o Guidelines recommend replacing an ACE inhibitor or
angiotensin receptor blocker (ARB) with valsartan
sacubitril in patients with chronic symptomatic heart
failure with reduced ejection fraction who tolerate ACE
inhibitor or ARB therapy.
. In patients with heart failure with reduced ejection
fraction, p blockers prolong survival and are generally
well tolerated, but they should not be initiated in patients
with acute decompensation.
o Aldosterone antagonists reduce mortality and heart fail
ure hospitalizations in patients with symptomatic heart
failure but are underused.
. In Black patients with New York Heart Association func
tional class III to IV symptoms, isosorbide dinitrate
hydralazine used in combination with an ACE inhibitor,
p blocker, and aldosterone antagonist has been shown
to reduce mortality.
o Ivabradine should be considered for patients with
symptomatic heart failure and an ejection fraction of
35'7, or less who have an elevated heart rate (>7Olmin)
in sinus rhythm and are taking maximally tolerated
B blockertherapy.
. The goal treatment regimen for mortality reduction in
patients with heart failure with reduced ejection frac
tion comprises an angiotensin receptor-neprilysin
inhibitor, a p-blocker, an aldosterone antagonist, and a
sodium glucose cotransporter 2 inhibitor.
Biguanides
(metformin)
Sulfonylureas
Th iazolid ined iones
GLP-1 receptor
antagonists
DPP-4 inhibitors
5GLT2 inhibitors
Survival benefit in patients with HF
Discontinue in patients with lactic acidosis
or cardiogenic shock
No randomized data in patients with HF
Not recommended because of increased
risk or worsening of HF
Observational studies suggest an
increase in HF with insulin therapy
Reduce major adverse cardiovascular
events but no impact or detriment in
patients with HF
lf recent HF hospitalization, use with
caution based on two smalltrials
No evidence of cardiovascular benefit
Reduce risk for worsening HF and
cardiovascular death in patients with HF
with reduced ejection fraction with or
without type 2 diabetes mellitus
lnulin
DPP-4 = dipeptidyl peptidase 4; GLP 1 = glucagon like peptide 1; HF = heart
failure; SGLT2 = sodium glucose cotransporter 2.
Drug Class Considerations
TABLE 14. Glucose-Lowering Medications in Patients
With Heart Failure
35

48. .Heart Failure
Device Therapy
lmplantable Cerdiouerter-Defibrillator Therapy
Arrhythmias are a common cause of death in patients with
heart failure, and an implantable cardioverter defibrillator
(lCD) improves survival when used fbr both primary and
secondary prevention ol sudden cardiac death. Current
guidelines recommend ICD placement in patients receiving
guideline directed medical therapy with an LVEF of 35'/. or
less and NYHA functional class II or III symptoms. Patients
with class IV symptoms should only undergo ICD placement
if they are candidates for heart transplant or LV assist device
(LVAD) placement. lmportantly, LVEF and symptoms should
be reassessed after guideline directed medical therapy
(+O days after myocardial infarction, 3 months in all others).
because many patients with new onset heart failure experi
ence substantial improvements in LVEF and may not require
or benefit from an ICD.
Results of the VEST trial demonstrated that among patients
with LVEF of 35% or less after acute myocardial infarction, a
wearable cardioverter defibrillator did not reduce the incidence
ofsudden cardiac death but did reduce the secondary outcome
of all cause mortality. There are no guideline recommendations
on the use of a wearable cardioverter defibrillator in patients
with heart failure; however, it is an option for patients at high
risk for arrhythmias as a bridge to ICD therapy.
Cqrdiac Re sy nchro n izotion T he rap y
Cardiac resynchronization therapy (CRT), or biventricular
pacing, involves traditional pacing of the right ventricular
apex and pacing of the LV lateral wall via a lead inserted
through the coronary sinus into a lateral cardiac vein. CRT
improves LVEF, reduces heart failure symptoms, and reduces
mortality in patients with dyssynchrony (demonstrated in
most trials by a prolonged
QRS interval or Ieft bundle branch
block [LBBB]). Patients with LBBB are most likely to benefit
from CRT. although patients without LBBB but with a QRS
complex of 150 ms or longer may derive a lesser benefit. CRT is
indicated in patients with an LVEF of 35'X, or less. NYHA func
tional class II to IV symptoms despite guideline-directed medi
cal therapy, sinus rhythm, and LBBB with a QRS complex of
150 ms or longer (class 1 recommendation). For patients with
LBBB and a QRS duration of 120 to 149 ms or those without
LBBB but with a QRS duration greater than 150 ms, CRT can be
useful and should be considered (class 2a recommendation).
Patients meeting the criteria for CRT typically have an indica
tion for a concomitant ICD. and in these cases. a CRT
defibrillator device is indicated.
f,TY PO I l{I5
. Implantable cardioverter-defibrillator placement is rec-
ommended in patients with heart failure who have an
ejection fraction of 35u1, or less and New York Heart
Association functional class II or III symptoms while
taking guideline directed medical therapy.'
(Continued)
IEY POltIt (uninued)
o Cardiac resynchronization therapy is indicated in
patients with an ejection fraction of 35'7, or less, New
York Heart Association functional class II to IV symp
toms despite guideline-directed medical therapy. sinus
rh1.thm, and left bundle branch block with a QRS com
plex of 150 ms or longer.
Disease Management
Patients u,ith chronic heart failure should be serially assessed
for progression of disease in the outpatient setting. Each
follow up visit should include evaluation of current symptoms
and functional capacity; assessment of volume status. electro
lytes. and kidney function; and review of the patient's medi
cation regimen lor adequacy (both appropriate doses and
appropriate medications as heart failure progresses). Ofequal
or greater importance is repeated patient education. nonjudg
mental assessment of adherence. and evaluation of obstacles
that may prevent patients from taking their medications as
prescribed or following recommendations for diet. activiry
and monitoring weight. Patients who appropriately take their
medications and avoid excess sodium and fluid intake can
greatly improve their functional status.
Sleep disordered breathing (obstructive sleep apnea, cen
tral sleep apnea) is underdiagnosed in patients with heart
failure. Recognizing and treating sleep disordered breathing is
important fbr improving quality of Iife in patients with heart
failure and for potentially improving heart failure related
outcomes. Current guidelines support obtaining a formal sleep
assessment in patients with symptomatic heart failure (NYHA
functional class II-IV) and excessive daytime sleepiness or
those suspected of having sleep-disordered breathing (see
MKSAP 19 Pulmonary and Critical Care Medicine). lnitial
therapy for patients with heart failure and sleep disordered
breathing is guideline-directed medical therapy for heart
failure because it improves both heart failure and sleep
disordered breathing clinical outcomes. Persistent sleep
disordered breathing despite guideline directed heart failure
therapy should be treated with continuous positive airway
pressure (CPAP). Treatment of obstructive sleep apnea with
CPAP improves sleep quality and reduces the apnea hypopnea
index. In contrast, treatment of central sleep apnea
"l'ith
adap
tive servoventilation in patients with an LVEF less than 45',4, is
associated with an increased risk for death.
Echocardiography in Chronic Heart Failure
Echocardiography is the most common method of assessing
LV function. ln patients with new onset heart failure, guide-
lines suggest repeating assessment of LV function after optimi-
zation of medical therapy. Patients with an LVEF of 35'X, or less
may be candidates for ICD placement or CRT. Current guide
lines recommend against routine surveillance echocardiogra-
phy in the absence of a change in clinical status or planned
intervention.
36

49. Serial B
'I'ype
Natriuretic Peptide Assessment
BNP measurement may support the diagnosis of volume over-
load in patients with acute or chronic heart failure and, in
patients with stable heart failure. can provide infbrmation
about prognosis and disease severity. Serial measurements and
BNP guided treatment, however, have not been shown to
reduce hospitalizations or mortality in patients with heart
failure.
Mult id isc i pl i no r A
Te am Ma nage me nt
Heart failure is a complex disease. and as many as 50'1, of
elderly patients with heart failure have four or more comorbid
conditions, such as hypertension, diabetes, chronic kidney
disease, COPD, dementia, malignancy. and depression. The
number of con.rorbidities correlates with an increased risk tbr
mortality. Optimal treatment ol heart failure and con.rorbid
conditions involves a collaborative. team based approach to
care. The multidisciplinary teanl should comprise l primary
care physician, cardiologist, and other specialists. Ideally, care
decisions should involve all interested groups rather than each
team member treating the patient individually.
Hospital discharge is an especially important time fbr
multidisciplinary team management. Discussions of therapy
selection, strategizing medication uptitration, identifying
early- and long term caregivers, and establishing early fbllow-
up can help prevent reudmission.
Primary Care Preuention Strclfegies
Hypertension and diabetes are the two greatest modiliable risk
factors for heart failure and should be the focus of prevention
elforts in the primary care setting. Evidence has shown that
heart failure incidence can be reduced by significantly lower
ing blood pressure to a goal of less than 130/80 mm Hg, and
ACCTAHA guidelines recommend treating to this target in
patients w,ith HFTEF. A meta analysis showed that each
10 mm Hg reduction in systolic blood pressure was associated
with a 2B'2, reduction in heart failure incidence. Control of
diabetes with metformin and/or SGLI2 inhibitors also may
reduce heart failure. Additional prevention strategies include
weight loss and smoking cessation. which decrease the inci
dence of CAD. the most common cause of heart failure.
Routine prin.rary care interventions. including vaccination lbr
pneumonia and influenza, should be performed.
Lifestyle Modification
Lifestyle modification, including weight loss and smoking
cessation, should be encouraged. Atcohol may be consumed
in moderation. Patients should be instructed to weigh them
selves daily because rapid changes in weight may be a pre
dictor of heart failure decompensation. Although sodium
restriction (<1.5 g/day) and t'luid restriction (t.s z Llday)
are routinely advised, few data are available on these
interventions.
Cardiac rehabilitation fbr heart failure patients has been
associated lr,'ith improvements in functional capacity, exercise
Heart Failure
duration, and quality of lif'e, and exercise training is recom
mended fbr all patients. Most trials of exercise training have
been small. although the large HF ACTION trial of 2331
patients showed a trend toward benefit in survival or hospitali
zation. ln patients with risk lactors fbr worse prognosis
(including atrial fibrillation or flufter, poor exercise tolerance,
depression, and Iower LVEt"), exercise improved survival.
Assessing Prognosis
Many prognostic models have been developed to assist in pre
dicting morbidity and mortality in patients with heart failure.
These models are usually derived from retrospective analyses of
clinical trials or large admission databases. To some extent, the
models reflect the unique patient populations enrolled in clini
cal trials. which tend to have f'ewer comorbid conditions. It has
been suggested that these tools be used in addition to, not in
place of, clinical judgment for heart failure management.
Clinical indicators associated with worse outcomes in the
1 b 2 years after diagnosis include heart failure hospitaliza
tion, poor exercise tolerance, ICD firings, hyponatremia, wors
ening kidney lunction, cardiac cachexia, required loop diuretic
doses of more than I mgikg, and symptomatic hypotension
necessitating reduced dosage of heart lailure medications.
Heart failure hospitalization is associated with a mortality rate
ol l0',1, to 20'2, over the next 6 months. Patients with poor prog
nosis should be engaged in a trank discussion of advanced
therapies, such as LVAD placement or heart transplantation.
End ofllife goals should be discussed with patients who are
ineligible lor or uninterested in such therapies, and palliative
care or hospice should be considered.
t(tY P0lilrs
o Current guidelines recommend against routine surveil HVC
lance echocardiography in patients with chronic heart
failure in the absence ofa change in clinical status or
planned intervention.
r Serial B type natriuretic peptide measurements should HVC
not be used to guide care of patients with chronic heart
failure.
o Heart failure incidence can be reduced by treating to a
target blood pressure of less than 130/80 mm Hg.
Heart Failure With Preserved Ejection Fraction
The incidence of HFpEF increases with age, although younger
patients may be affected. Patients hospitalized with HFpEF are
more likely to be older women with obesity and hypertension
and less likely to have overt CAD than those hospitalized
with HFTEF.
The primary therapies tbr HFpEF are diuretics to control
symptoms of volume overload and antihypertensive agents to
target a systolic blood pressure of less than 130 mm Hg. [n
patients with worsened symptoms of heart failure and con
comitant atrial fibrillation, restoration of sinus rhythm or rate
control may reduce symptoms.
L
t
37

50. Heart Failure
Despite many studies of therapeutic agents, no drug has
been shown to reduce morbidity or mortality in patients with
HFpEF a finding that may reflect the heterogeneity of HFpEF
etiologz. The TOPCAT trial showed no difference in the pri
mary combined end point of death, aborted cardiac arrest. or
heart failure hospitalization with spironolactone versus pla
cebo. In retrospective analysis, there was an unusual amount
of regional variation in efficacy. Specially, a mortality advan
tage was noted in the United States but not confirmed in
European patients. According to ACC/AHA guidelines. in
select patients with HFpEF with an elevated BNP level or heart
failure hospitalization within 1 year, aldosterone antagonists
might be considered to decrease hospitalizations. The con
traindications for aldosterone antagonists in patients with
HFpEF are the same as those for patients with HFTEF.
ln 2021. based on the results of the PARAGON HF trial.
which demonstrated a nonsignificant reduction in the primary
end point of heart failure hospitalization or cardiovascular
death, the FDA expanded the indication for valsartan sacubitril
to include all patients with heart failure, regardless of ejection
fraction. This decision was primarily based on the finding that
patients with an LVEF between 45'7, and 55'2, appeared to ben
efit from the drug.
XEY POIlII
. The primary therapies for heart failure with preserved
ejection fraction are diuretics to control symptoms of
volume overload and antihypertensive agents to target a
systolic blood pressure of less than 130 mm Hg.
Acute Decompensated Heart Failure
Initial management of acute decompensated heart failure
focuses on identifying the cause of the heart failure exacerba
tion, determining the patient's current physiologic state,
removing fluid to improve congestion, and optimizing medical
therapy before discharge.
Common causes of acute decompensation include fluid
overload in the setting of nonadherence to dietary fluid or salt
intake recommendations and recurrent ischemia in patients
with ischemic cardiomyopathy. Fluid overload may be related
to an inability to tolerate previous levels offluid and salt intake
due to progression of LV dysfunction or may occur after an
unintentional increase in salt intake. Other causes of decon.r
pensation include hypertension, concomitant illness, and
nonadherence to therapy with diuretics and other medica
tions. Identifying the cause of decompensation may mitigate
risk and prevent recurrence.
Volume status should be assessed in all patients hospital
ized for heart failure. Symptoms of volume overload include
orthopnea, paroxysmal nocturnal dyspnea, peripheral edema,
weight gain, and progressive exertional dyspnea. On physical
examination. jugular venous distention is usually present.
Patients may have crackles (which are much more likely in
acute than chronic heart failure), ascites, or peripheral edema.
Perfusion also should be assessed, and patients may be
classified as "warm" (adequate perfusion) or "cold" (inade
quate perfusion). Signs of inadequate perfusion include cool
extremities. narrow pulse pressure. poor mentation. and
worsening kidney function. Intravenous inotropes or other
advanced therapies should be considered in patients with poor
perfusion to improve cardiac function.
In patients with decompensated heart failure with fluid
overload. effectire diuresis is essential (Figure la). Loop diuret
ics are the principal therapy. ln a study evaluating different
strategies fbr diuresis, including varied diuretic dosages and
bolus versus continuous therapy, high dose diuretics (2.5
times the outpatient oral daily dosage) were associated r,r,ith
increased diuresis but also transient worsening of kidney
function. No differences were observed between bolus and
continuous intravenous infusion groups, and length ofstay did
not differ regardless of the strateg, used. ln patients '*'ho do
not achieve adequate diuresis with a loop diuretic. increasing
the dosage or adding a thiazide diuretic may be considered.
Notably, administering low dose dopamine to improve diure
sis and preserve kidney function offers no benefit.
Many patients with acute decompensated heart failure
either present with or develop concomitant acute kidney d1's-
function, termed cardiorenal s1'ndrome. Cardiorenal s1'n
drome is related to neurohormonal activation. abdominal
vascular congestion leading to decreased renal perfusion, and
worsening cardiac function (see MKSAP 19 Nephrologr). Often,
continued diuresis with higher diuretic doses is required
despite worsening kidney function. Withholding ACE inhibi
tors and aldosterone antagonists may be reasonable until kid
ney function improves. If kidney function '"torsens as the
patient approaches euvolemia, withholding diuretics for 1 day
to allow extravascular fluid to redistribute into the vascular
space should be considered.
Standard heart failure therapy including ACE inhibitor
(or ARB) therapy, p blockers. and aldosterone antagonists.
should be maintained throughout hospitalization or restarted
before discharge. If p-blockers are discontinued at admission
because ofsigns oflow cardiac output, therapy should not be
reinitiated until the patient improves. If the patient is admitted
with volume overload without signs of low cardiac output.
B blocker therapy can usually be maintained at the same or a
lower dosage during hospitalization.
In patients with respiratory distress not requiring
mechanical ventilation, noninvasive positive pressure ventila
tion is associated with reduced need for intubation and.
according to some data. a reduction in mortality. It is unclear
ilnoninvasive positive pressure ventilation results in a shorter
length of hospital stay.
BNP level should be measured on admission and before
discharge for prognostic purposes. High BNP levels on admis
sion are linked with increased mortality and rehospitalization.
Likewise, BNP levels that fail to decrease during hospitaliza
tion are associated with a higher mortality rate. Serum tro
ponin measurement on admission also can be used for
prognostication; patients with elevated troponin levels have
38

51. Heart Failure
tIGURE 14. Diuretictherapyindifferentclinicaltrajectories.BP=bloodpressure; ED-emergencydepartment; lV=intravenous.
worse clinical outcomes and a higher risk for death. Currently,
there are no absolute cutoff values for these biomarkers, and
measurement data should be used in combination with clini
cal judgment to guide management.
Cardiogenic Shock
Cardiogenic shock is characterized by signs and symptoms of'
low cardiac output with hypotension (systolic blood pressure
<90 mm Hg or support to maintain blood pressure) and evi
dence of end organ hypoperfusion. Laboratory evaluation
includes assessment of BNP. lactate, and kidney and liver
function. Treatment initially fbcuses on reversing the cause
ofshock, such as reperfusion in the setting ofacute coronary
syndrome. Intravenous inotropic agents are often required
to improve hemodynamic status, including cardiac output
and urine output (Table 15) (see MKSAP 19 Pulmonary and
Critical Care Medicine). Routine invasive pulmonary artery
catheterization to monitor hemodynamics does not improve
survival or reduce future hospitalization in patients with
decompensated heart lailure. Pulmonary artery catheteriza
tion should be used only when hemodynamic and volume
status is unclear or when hemodynamic data may lead to
Trajectory: lmproving
towards target
Continue diuretics
. Target relief of congestion
o Plan for transition to oral
therapy
IV
Diuretics
Trajectory: lnitial
improvement, then
stalled
Monitor symptoms, signs,
urine output, BP, electrolytes,
and assess trajectory
Escalate diuretics
. Usually increase loop
diuretic dose by 50o/o-1OO%
o Consider metolazone
2.5-5 mg 1-2x daily
r Consider other thiazides
lnitiate lV loop diuretics early (ED
or immediately after admission)
lnitial dose usually 1-2.5 times
total daily oral loop diuretic in
furosemide equivalents
Prescribe lV diuretics (every 8-1 2
h or continuous), depending on
patient characteristics, diuretic
response, kidney function
Trajectory: Not
improved/worsening
Change course
. Escalate diuretics
o Consider other
decongestion strategies
o Consider hemodynamic
monitoring
. Consider inotropes
. Consider advanced
therapies
TABLE 15. lntravenousVasoactive Medications Used forTreatment of Cardiogenic Shock
Medication
Milrinone
Dobutamine
Sodium nitroprusside
Nitroglycerin
Vasopressin
Dopamine
Norepinephrine
Mechanism
Phosphodiesterase inhibition
B1,
p2 Receptor agonism
Nitric oxide production 0
Nitric oxide production 0
Arginine vasopressin receptor (V receptor) agonism
Dopaminergic receptor (D receptor) agonism +
p1 Receptor agonism at intermediate dose
cr1 Receptor agonism at high dose
cr1, cr2 Receptor agonism greaterthan p1 receptor +
agonism
lnotropy Vasodilation
+
(+)(at low dose)
-
(vasoconstriction at high dose)
+
+r (mainly venous)
-
(vasoconstriction)
-
(vasoconstriction at high dose)
+-t-
#
Strength of effect: + indicates very strong; + indicates strong; (+) indicates weak; 0 ind cates neutral; indicates opposite effect.
-
(vasoconstriction)
39

52. Heart Failure
advanced mechanical circulatory support or consideration of
heart transplantation.
Percutaneous mechanical support during acute exacerba
tions has greatly increased in recent years. Intra aortic balloon
pumps, percutaneous ventricular assist devices' and extracor
poreal membrane oxygenators can be quickly placed to sup
port the critically ill patient. Treatment by a team comprising
a heart failure physician, critical care physician. and cardiac
surgeon is suggested to rapidly deploy therapy and provide
postprocedure care. Consideration of'longer term options for
advanced heart failure (heart transplantation or LVAD place
ment) is an important aspect o1'management for patients
receiving mechanical support in the acute setting. For patients
who do not show clinical improvement, there should be daily
discussions about treatment options and goals of care, includ-
ing transplantation, permanent device placement, and pallia-
tive or hospice care.
Strategies to Prevent Readmission
The first step in preventing heart lailure readmission is to treat
reversible causes of the exacerbation. Discharge should not
occur until the patient has approached euvolemia with diure-
sis. Medication reconciliation befbre discharge should ensure
that the patient is taking the appropriate medications, particu
larly those that reduce mortality and morbidity in heart failure.
Education on heart failure physiologr. the importance of medi
cation and dietary adherence, signs and symptoms of worsen
ing hearl failure, and when to contact a physician should be
provided. A home nurse, in follow up after discharge, can
ct-rntinue patient education, provide confirmation that dis
charge medications coincide with home medications. and
assess the patient's condition. If home nurse follow up is not
instituted, a phone call within 48 hours of discharge may be use
ful to confirm a safe transition ofcare. Finally, an early follow
up appointment (within 7 days) should be scheduled to review
the medication list, assess volume status and adherence to diet
and medications, and reinforce patient education points.
XEY POITIIS
. Management of patients with acute decompensated
heart failure focuses on identifying the cause ofthe
heart failure exacerbation, determining the patient's
physiologic state, treating fluid overload, and optimizing
medical therapy before discharge.
HVC o Routine invasive pulmonary artery catheterization for
hemodynamic monitoring is not recommended in
patients with decompensated heart failure.
. In patients hospitalized with acute heart failure, early
follow-up with home nurse visits, phone contact within
48 hours ofdischarge, and a prompt physician appoint
ment (within 7 days) to review the medication list,
assess volume status and adherence to diet and medica-
tions, and reinforce patient education reduces the risk
for heart failure readmission.
Advanced Refractory Heart Failure
Patients with persistent severe l.reart failure symptoms despite
maximal medical therapy (ACC/AHA stage D) are candidates
for advanced treatment. Cardiac transplantation remains the
gold standard therapy tbr patients with end stage heart failure:
however. because of a lack of appropriate donors. only 3500
heart transplantations are performed in the United States each
,ear. accounting for approximately 50'),, of eligible patients
awaiting transplantation. Acceptable candidates for transplan
tation generally are younger than 65 to 70,€ars $'ith no medi
cal contraindications
(e.g.. diabetes with end organ complica
tions. malignancies within 5 years. kidney dysfunction. or
other chronic illnesses that will decrease survival) and good
social support and adherence. Many patients awaiting trans
plant also require an LVAD for support until an organ becomes
available. Hospice may be considered as an option in shared
decision making discussions.
Mechanical Circulatory SupPort
Clinical outcomes in patients rn,ith advanced heart failure hare
markedly improved u'ith the use of LVADs. With neu€r
continuous flow devices, patients have 1 year sun'ival approx
imating that of cardiac transplant recipients and substantial
improvements in functional capacity and quality of life. The
use ofLVADs as destination therapy rather than as a bridge to
transplant is increasing. LVAD placement is currently indi
cated in patients with LVEF less than 25'1, and poor exercise
tolerance (NYHA functional class lll,'lV) despite maximal
tolerated therapy. r,r,ith either a high predicted 1 to 2 year
mortality or inotrope dependency. rtho continue to desire
aggressive restorative care.
Because these devices provide continuous flow. most
patients no longer have a palpable pulse. and blood pressure
must be measured by Doppler. Therapy includes anticoagula
tion to prevent pump thrombus formation; continued heart
failure therapy with an ACE inhibitor. ARB. or ARNI and
p blocker; and management of fluid overload with diuretics.
Clinicians caring for hospitalized patients with an LVAD
require specialized training and continuous emergency access
to an LVAD specialist.
LVADs have several potential major complications related to
either the pump itself or the driveline. r.thich passes through the
skin and connects the internal pump to a po er source. Major
complications at 2 years reported with a current generation
device include hemorrhagic and thrombotic strokes (9.9'7,)r
inf'ections (skin or at the pump) (58.3'7,); pump thrombosis
(1.4'){,); arrh}'thmias (gs.g'it,): and gastrointestinal bleeding
(24.5'X,)
. which is usually associated with small bouel arterio
venous malformations. The incidence of these complications
is decreasing as pump technologr'improres.
Management of Posttransplant Patients
Most patients who undergo heart transplantation quickly
recover physical activity and have normal quality of life, with
a mean survival of more than 1l years. The most frequent
40

53. complication within the first year after transplant is infection.
Cytomegalovirus (CMV) infection is common, and patients at
moderate risk (CMV positive donor/CMV-positive recipient)
and high risk (CMV-positive donor/CMV,negative recipienr)
should receive antiviral prophylaxis (valganciclovir or ganci
clovir) for 6 months.
Incidence of rejection is highest in the first 6 months after
transplantation. Because most patients with early rejection are
asymptomatic, regularly scheduled endomyocardial biopsies
or gene expression profiling tests are performed to detect
rejection for the first few years after transplant. Severe rejec
tion is characterized by acute heart failure and atrial arrhyth
mias or conduction abnormalities.
Early complications related to immunosuppressive ther
apy include hypertension (more than 90"/,, of patients) and
diabetes (15'X, 2O"1, of patients). Long-term complications after
transplantation include CAD and an increased incidence of
malignancies, including skin cancer (common) and B-cell lym
phoma related to immunosuppressive therapy (less common).
Careful attention must be paid to the medication regimen
of cardiac transplant recipients to avoid drug drug interac
tions. Cyclosporine and tacrolimus, two agents commonly
used for immunosuppression, are metabolized by the CYP3A4
system ancl are subject to substantial drug interactions with
both inhibitors and inducers of this isoenzyme. An extensive
list of drugs that can interact through the CYP3A4 isoenzyme
can be found at http:/lmedicine.iupui.edu/clinpharm/ddis.
KEY PO I l{TS
. Patients with severe heart failure symptoms despite
maximal medical therapy are candidates for advanced
treatment, including placement of a left ventricular assist
device and heart transplantation.
. Patients who undergo heart transplantation have a
median survival of 11 years and, typically, a normal
quality of life.
r In cardiac transplant recipients, cltomegalovirus infec-
tion (within the first year) and rejection (within the first
6 months) are important complications.
o Endomyocardial biopsy or gene expression profiling
should be routinely performed after heart transplanta-
tion to diagnose early asymptomatic rejection.
Specific Populations
Patients With Diabetes Mellitus
Patients with diabetes have a more than twofold increased risk
for heart failure and experience worse outcomes, more hospi
talizations, and a worse prognosis than those without diabe
tes. Even when blood glucose levels are controlled, patients
with diabetes have a higher incidence of heart failure.
Aggressive blood glucose control has not been associated with
better outcomes. Implementing therapy with agents shown to
improve cardiovascular and heart failure outcomes is prudent
(see Table 1.1).
Heart Failure
Black Patients
Black patients have been underrepresented in many hearl tailure
clinical trials, including those involving valsartan-sacubitril, SGLI2
inhibitors, ivabradir.re, and long acting metoprolol. Based on the
benefits observed ir.r the general population, recent guidelines rec
ommend that tslack patients also receive these medications. Expert
consensus suggests first initiating an ARNI, ACE inhibitor, or ARB
(ARNI preferred); a p blockeri alt aldosterone antagonist; and an
SGLI2 inhibitor. Once target or maxin-rally tolerated doses of an
RAAS inhibitor, p blocker, and aldosterone antagonist are achieved,
adding isosorbide dinitrate hydralazine is recommended for Black
patients with HFrEF who remain qrn.rptomatic. Black patients
have a higher incidence of angioedenra while taking RAAS inhibi
tors (ACE inhibitors, ARBs. ARNI); hol,rever, these agents should be
initiated despite the increased risk.
Specific Ca rd iomyopath ies
Fbr a discussion of'hypertrophic cardiomyopathy and restric
tive cardiomyopathy, refer to Myocardial Disease. Peripartum
cardiomyopathy is discussed in Pregnancy and Cardiovascular
Disease.
Ta kotsu bo Ca rd iomyopathy
'lakotsubo
cardiomyopathy. also knou,n as stress induced car
diomyopathy or apical ballooning syndrome, is a clinical syn
drome associrrted with reduced LVEF. elevated cardiac enzyme
levels, and sigr.rs of ischen.ria on E(l(1. It typically occurs in
older women and is usually precipitated by a stressful physical
or emotional event, such as the death ofa loved one or a sud
den surprise. The patl-rogenesis of takotsubo cardiomyopatlry
is unknown. but the condition is postulated to result from
reversible myocarclial toxicity induced by very high catechola
mine levels. Cardiac irnaging shows wall motion abnormalities
that do not follow ir coronary artery territory (typically, apical
dyskinesis or ballooning) with preservation of bas;rl wall
motion (Figure 15). Because takotsubo cardiomyopathy resem
bles an acute coronary syndrome, enrergent coronary angiog
raphy is often perfbrmed. Treatment is largely supportive and
is similar to that fbr heafi failure of other causes. Most patients
recover cardiac function over the course of a few weeks to
months. As with other forms of new onset heart failure, repeat
echocardiography should be perfbrmed in 3 to 6 months to
evaluate recovery. It is unclear for how long medical therapy
should continue in patients with recovery of cardiac function,
but most clinicians continue therapy fbr at least 1 year.
Acute Myocarditis
Myocarditis is a clinical syndrome ol acute onset heart failure.
Causes include viral. bacterial. or other inf'ections: toxins; and
immunologic syndromes. The classic fbrm is viral in origir.r and
is preceded by a typical upper respiratory tract infection caused
by adenovirus, echovirus, or coxsackievirus. Patients may have
no antecedent symptoms or have a viral prodrome with fever,
myalgia, and respiratory symptoms. Although the pathogenesis
I
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I
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:
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41

54. Heart Failure
FIGURE 15. Leftventriculogramshowingtakotsubo(stress) cardiomyopathyduringsystole(/ef)anddiastole(lght). lnsystole,thebasal segmentsof theheartcontract
well, while the apical portion of the heart is dyskinetic (bulges outward as opposed to inward) (arrows).
is not completely understood, it is thought that acute viral
infection causes early destruction of myocytes followed by an
immune response that causes further destruction.
Echocardiography helps rule out other causes of heart
failure. Definitive diagnosis may require CMR imaging or endo-
myocardial biopsy. Biopsy is recommended for patients with
recent-onset heart failure (<2 weeks) accompanied by hemo
dynamic compromise and patients with unexplained new-
onset heart failure of 2 weeks' to 3 months' duration who
develop new arrhl.thmias or do not respond to evidence based
therapy within 2 weeks. Standard therapy for heart failure is
recommended; anti inflammatory agents do not offer benefit.
Giant Cell Myocarditis
Giant cell myocarditis is an acute and often fatal form of myo-
carditis that typically occurs in younger persons. It is often
rapidly progressive and can cause both left and right ventricu-
lar dysfunction. Giant cell myocarditis is also associated with
an increased incidence of high-grade atrioventricular block
and ventricular arrhythmias. Patients with acute heart failure
unresponsive to usual care or with accompanying arrhy.thmias
should undergo endomyocardial biopsy for diagnosis. Initial
biopsy findings may be negative because of the patchy nature
of the inflammation. Unlike in acute myocarditis, aggressive
immunosuppressive therapy has some benefit and should be
initiated. Patients often require percutaneous or surgical ven
tricular support until they recover or need heart transplanta
tion or LVAD placement. If giant cell myocarditis is suspected,
prompt transfer to a hospital equipped with mechanical sup
port should be considered because patients can progress from
feeling well to moribund within hours.
Sarcoidosis
Cardiac sarcoidosis is found at autopsy in up to 25% ofpatients
with systemic sarcoidosis. Cardiac manifestations include ven-
tricular tachycardia, second- or third degree atrioventricular
block, and HFTEF. Diagnosis is made by the presence of non
caseating granuloma with no alternative cause identified on
extracardiac or endomyocardial biopsy and clinical cardiac
manifestations. Cardiac imaging studies for diagnosis include
PET and CMR imaging. Therapy for patients with sarcoidosis
and heart failure includes standard heart failure therapy and
immunosuppressive medications. For acute flares of either
heart failure (decline in LVEF) or arrhythmias, high dose
prednisone is typically initiated, followed by tapering over 3 to
6 months.
Tachycardia-Mediated Cardiomyopathy
Tachycardia-mediated cardiomyopathy has been associated
with supraventricular and ventricular arrhl.thmias. Reversible
causes of tachycardia (e.g., hyperthyroidism) should be
excluded. Importantly, rate control (B blockers) or rhythm
control (catheter ablation) improves LV function in these
patients. In patients with atrial fibrillation associated with
rapid ventricular response, no evidence shows that convert
ing to sinus rhythm is more efficacious than controlling
heart rate. In patients with ventricular arrhl.thmias or fre-
quent premature ventricular contractions. cardiomyopathy
is generally thought to develop when the burden of prema
ture ventricular contractions is more than 10,000 per day
or more than 10% of all beats; ablation, especially if the
premature ventricular contractions are unifocal, should be
considered.
42

55. Arrhythmias
lntroduction
Disruptions in cardiac rhythm or rate occur in seven basic pat
terns: early beats, bigeminal beats, grouped beats, pauses,
bradycardia, tachycardia, and chaotic rhythms. This section
provides an approach to arrhythmias and discusses the diag
nosis and management of specific rhythm disorders.
Approach to the Patient
With Bradycardia
Clinical Presentation and Evaluation
Bradycardia (heart rate <50/min) may be asymptomatic or asso
ciatecl with light headedness, syncope, exertional inttilerance,
dyspnea, or fatigue. It can be a normal finding or result lrom
disease in the sinus node, atrioventricular (AV) node. or His
Purkinje system or from dysfunction of'the autonomic system.
Diagnostic evaluaticin consists of a thorough history,
physicat examination, focused laboratory testing (electrolyte
levels, thyroid function testing). and resting 12 lead ECC.
Severe or unstable conduction abnormalities that require
urgent intervention must be identified.'[he evaluation should
also include investigation for extrinsic and reversible causes of
bradycardia, including ischemia, myocarditis, endocarditis,
hypothyroidism, infecticlus diseases, neurologic events, elec
trolyte disturbances, and medication use (especially AV nodal
blockers and parasympathomimetics). Echocardiography is
fiequently performed, and exercise stress testing to assess
chronotropic competence as well as ambulatory ECG monitor
ing may be helpful. Clues fiom the history and physical exami
nation (e.g., rash suggestive of' Lyme disease) may dictate
further testing. In patients with nocturnal bradycardia or con
duction disturbances, sleep apnea should be considered and,
ifappropriate, evaluated and treated. Isolated nocturnal brad
yarrhythmias are unlikely to require a permanent pacemaker.
2nd A I
Arrhythmias
Sinus Bradycardia
Sinus bradycardia is defined as the presence ofsinus rhythm
with a heart rate below 50/min. Sinus bradycardia may be
appropriate in trained athletes and during sleep. Inappropriate
or patholoqic sinus bradycardia is most commonly caused by
sinus node dysfunction due to age related myocardial fibrosis.
Less commonly, sinus node dysfunction may result from right
coronary ischemia, hypothyroidism, intracranial hyperten
sion. postoperative scarring or fibrosis from cardiothoracic
surgery or infiltrative or inflammatory disorders (e.g., sar
coidosis). The most common extrinsic cause is medication use
(B blockers, donepezil, neostigmine, pyridostigmine).
Atrioventricular Block
AV block may be classified as first degree, second degree, or
third degree. First degree AV block is defined by a delay in AV
conduction (PR interval >200 ms). In large cohort studies,
first degree AV block has been associated with an increased
risk for atrial flbrillation (AF) and all-cause mortality.
In second degree AV block, only some P waves conduct to
the ventricles. Mobitz type I second degree (Wenckebach) AV
block is characterized electrocardiographically by a PR interval
that progressively prolongs until a QRS complex is dropped,
resulting in grouped beating (Figure 16). Mobitz type 2 second-
degree AV block is typitied by intermittent nonconducted P
waves with unchanging PR intervals (Figure 17). When 2:1
block is present, the Mobitz type cannot be determined defini
tively. However, the distinction between types is important
Mobitz fype 2 AV block usually occurs below the AV node and
has a higher risk for progression to complete heart block.
tligh degree AV block ref'ers to the presence of more than one
successive nonconducted P wave, resulting in several consecu
tive P waves without QRS complexes.
In third degree AV block, also termed complete heart
block. no P waves conduct to the ventricles. AV dissociation is
observed on the ECG (Figure 18).
Bate 58 BPM 10 mm'mv 02:10:10 5,24'2017
i
.
tIGURE 16. ECGshowingMobitztypelsecond'degreeatrioventricularblock(Wenckebachblock),whichmanifestsasaprogressiveprolongationof thePRinterval until
there is a dropped ventricular beat (arrow).
43

56. Arrhythmias
Il.'
FIGUnE lT.ECGshowingMobitztype2second-degreeatrioventricularblock.Pwavesareblockedintermittently(arows),andthePRinterval isfixed.Although2:1 block
can be a manifestation of Mobitz type 1 second-degree atrioventricular block, note the wide QRS complexes, which are more consistent with block below the compact
atrioventricular node.
FIGURE 18. lnthisECG,thePwaves(shortanowslandtheORScomplexes(longarrows)arenotassociatedwitheachother,indicatingthepresenceof
completeheart
block.
44

57. I
I
I
Treatment
In patients with symptomatic ltradl,cardia and hen.rodynamic
distress. atropine should be aciurinistered. Ifatropine is ineflbc
tive. chronotnrpic drug intusions (e.g.. dopamine or epineph
rine) can be giver-r until transcutaneous pacing or a temporary
pacing wire (pref'erred) can be implemented. 1'emporary pac
ing is indicated fbr transient conditions causing l.remodynami
cally unstable bradycardia or lsystole.
In henrodynamically stirble patients. reversible and
extrinsic causes of bradycardi:r should always be addressed
before more invasive measures. such as permanent pacing,
are considered. Common indications for permanent pacing
include:
. Symptom:rtic bradycarclia r,r'ithout reversible ciruse
. Pernranent AF and symptomatic bradycardia
o Alternating bundle branch block (signifies higl.r risk con
duction disease)
o Complete heart block. high degree AV block. or Mobitz
type 2 sccond degree AV block. regardless of symptoms
Patients with stable letl bundle branch block or right
bundle branch block with or without a prolonged PR interval
do not require permanent pacing because intraventricular
conductior.r clelays have a low risk for progressing to complete
heart block (1'){, 3'X, per year).
Arrhythmias
Types of implar.rted cardiac electronic devices, their func
tions, and their general indications are reviewed in Table 16.
Figure 19 shows a leadless pacemaker in the region of the right
ventricle.
rEY POI]ITS
. Permanent pacing is indicated fbr symptomatic brady
cardia without reversible cause.
. Patients who have atrioventricular (AV) and infranodal
conduction disturbances with a high risk for progress
ing to complete heart block or asystole, such as alter-
nating bundle branch block, high degree AV block, or
Mobitz type 2 second-degree AV block, should receive a
permanent pacemaker.
Approach to the Patient
With Tachycardia
Clinical Presentation and Evaluation
Patients with tachycardia (heart rate >100,'min) may be
asynlptomatic or experience tachypalpitations, a sens:rtion of
skipped beats, light l.readedness, clizziness. chest discomfort,
dyspnea. exertional intolerance, tatigue, progressive heart fail
ure, near syncope, or syncope. In :rsymptomatic patients,
tachycardia nray be discovered incidentally.
\
I
Functions
TABLE 16. Cardiac lmplantable Electronic Devices forTreatment of Cardiac Rhythm Disorders
\
Device Components lndications Pacemaker
Function
Antitachycardia Defibrillation
Pacing
No
No
Transvenous
pacemaker
Lea d I ess
pacemaker
(see Figure 19)
lmplantable
cardioverter
defibrillator
Su bcutaneous
implantable
cardioverler-
defibrillator
Cardiac
resynch ro n ization
therapy-paci ng
(cRr-P)
Cardiac
resynch ronization
thera py-defi bri llator
(CRT D)
Pulse generator and
intravascular leads (single
or dual chamber)
Pulse generator with tines
implanted directly into
the cardiac chamber; no
leads
Defibrillator and
intravascular leads (single
or dual chamber)
Defibrillator and a single
lead that are entirely under
the skin (extravascular); no
transvenous leads
Pulse generator and
intravascular leads,
including a pacing lead in
the coronary sinus to
pace the left ventricle
Defibrillator and
intravascular leads,
including a pacing lead in
the coronary sinus to
pace the left ventricle
Sinus node dysfunction,
AV block, nonreversible
symptomatic bradycardia
Atrial fibrrillation with
bradyca rdia, paroxysma I
bradycardia (e.9., brief
sinus node dysfunction or
AV block)
Monitoring and treatment
of ventricu lar arrhythmias
Monitoring and treatment
of ventricular arrhythmias
Restoring electrical
synchrony in select
patients with
symptomatic heart failure
Restoring electrical
synchrony between the
ventricles in patients with
heart failure; monitoring
and treating ventricular
a rrhyth mias
Yes
Yes (atrial
sensing and
ve ntri cu la r
paci ng )
Yes
Yes
No
No
Yes
Yes
No
Yes
No
No
Yes
No
AV = atrioventricular
Yes Yes
45

58. Arrhythmias
F I G U R E 1 9. Chest radiograph (posteroanterior view) showing a leadless
pacemaker in the region of the right ventricle, which could be verified to be
retrosternal on a lateral film. Prior sternotomy and signilicant ascending aorlic
calcification are also noted.
Documentation of tachycardia on ECG and correlation
with symptoms is the key component of the diagnostic evalu
ation, and a 12 lead ECG should be obtained in all patients
with stable tachycardia. A 12 lead ECG recorded during symp-
toms, although often not possible to obtain, is far superior to
most forms of ambulatory monitoring in terms of diagnostic
value (see Diagnostic Testing in Cardiologr for strategies in
selecting an appropriate monitoring device). Based on ECG
findings. tachyarrhythmias are traditionalll' categorized as
supraventricular or ventricular. Supraventricular arrhythmias
involve conduction through the AV node and are characterized
by normal appearing QRS complexes unless complicated by
an aberrant ventricular condition (e.g.. bundle branch block).
Ventricular arrhythmias originate belor,r'the AV node and are
characterized by abnormal appearing and widened QRS
compleres.
In addition to a thorough history'"r'ith medication review
and physical examination, thyroid function testing and
echocardiography may be considered in select patients $'ith
tachycardia.
Antiarrhythmic Drugs
Antiarrhythmic agents have trerditionally been organized
according to primary mechanism of action using the Vaughan
Williams classification system (Table 17). although most antiar-
rhyhmic drugs exert their effects through several mechanisms.
Class I and class III agents are the most effectile antiarrhl'thmic
drugs: however, due to their membrane active effects. they
carry some paradoxical risk of inducing arrhythmia.
Flecainide and propafenone are the most commonly used
class I agents (lC)r they are primarily used to treat atrial
arrhythmias and usually in conjunction with AV nodal block-
ers to prevent 1:1 atrial flutter. Toxicity can manif'est as
QRS widening (Figure 2o and Figure 2l). Class tC agents are
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tIGURE 20. ECGinapatientwithsignificantflecainidetoxicity,manilestingwithseverelyprolonged0RSduration,withbroadandveryunusual morphologyinthesetting
of underlying atrial fibrillation orflutter.
46

59. Classification Mechanismof
Action
Primary
Potential
ECG Effects
Side Effects ContraindicationsUseExamples
AV = atrioventricular; PAT =
paroxysmal atrial tachycardia; NYHA = New York Heart Association; SVT = supraventricular tachycardia.
TABLE 17. Commonly Used Antiarrhythmic Medications
Arrhythmias
Class lB
Class lC
Class ll
Class lll
Class lV
Multichannel
blockers
Adenosine
receptor
agonists
Cardiac
glycoside
Sodium channel
blockade
Sodium channel
blockade
p-Adrenergic
blockade
Potassium
channel
blockade
Calcium
channel
blockade
(nondihy-
dropyridines)
Several
mechanisms,
including
potassium,
sodium, and
calcium channel
blockade
A1-receptor
agonist
lncreases vagal
activity
Lidocaine,
mexiletine
Flecainide,
propafenone
Metoprolol,
propranolol,
carvedilol,
atenolol,
bisoprolol,
nadolol
Sotalol,
dofetilide
Verapamil,
diltiazem
Amiodarone,
dronedarone
ORS wideningVentricular
arrhythmias
Headache, dizziness,
or other neurologic
symptoms (both
drugs)
Seizures (lidocaine
toxicity)
Headache,
dizziness, or other
neurologic
symptoms
Fatigue, drowsiness,
dizziness, hair loss,
cold hands and feet,
depression, erectile
dysfunaion,
bronchospasm
Headache, dizziness,
bradycardia, fatigue,
dyspnea; rarely,
torsades de pointes
(sotalol)
Headache,
dizziness, diarrhea;
rarely, torsades de
pointes (dofetilide)
Dizziness,
constipation,
dependent
edema, nausea
Advanced liver
disease
lschemic or
structural heart
disease, sinus node
dysfunction,
second- or third-
degree AV block or
bundle branch
disease without a
pacemaker
Severe asthma,
cardiogenic shock,
second- or third-
degree AV block,
preexcitation
Renal insufficiency,
OT prolongation,
bradycardia, orAV
block without a
pacemaker
Significant sinus
node dysfunction,
second- orthird-
degreeAVblock
withouta pacemaker,
preexcitation
Advanced liver, lung,
orthyroid disease
(amiodarone)
Advanced liver
disease, permanent
atrial fibrillation,
recent
decompensated or
advanced heart
failure (NYHA
functional class lll-
lV) (dronedarone)
Severe asthma (can
worsen)
Advanced kidney
impairment
(requires dose
adjustment)
ORS wideningAtrial
fibrillation, SW
L
Decreased
heart rate,
prolonged
interval
PR
OT
prolongation
Decreased
heart rate,
prolonged
interval
PR
Many effects:
decreased
heart rate;
prolonged PR,
ORS, or OT
interval
Rate control of
atrial
arrhythmias,
SW
Atrial
fibrillation,
atrialflutter,
ventricu la r
arrhythmias
SW, rate
control of atrial
arrhythmias
t
i
:
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t
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t
)
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i
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t
I
t
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I
t
L
t
r
Adenosine Brief AV block
Digoxin Slows AV
node
conduction
Atrial
arrhyth mias
(both drugs),
ventricular
arrhythmias
(amiodarone)
Fatigue, dizziness,
nausea, vomiting,
constipation or
diarrhea, tremor,
liver and lung
toxicities (both
d rugs)
Thyroid and eye
toxicities
(amiodarone)
Flushing, dyspnea,
chest pain,
hypotension,
dizziness, nausea
Nausea, vomiting,
dizziness, blurry
vision and
yellow halos,
th rom bocytopen ia,
PAT with AV block
(toxicity)
Termination of
SW(intravenous
only)
Rate control of
atrialfibrillation
47

60. Arrhythmias
.tt
\,5
tIGURE 21. ECGinapatienttakingunusuallyhighpropafenonedoses,manifestingwithORSprolongationandbroad,relativelyslowwavesof atrial flutter(bestseenin
leads ll and lll).
va
\,!tI
roI
vt
t
contraindicated in patients with ischemic or structural heart
disease because of the risk for promoting ventricular arrhyth
mias and death.
Class ll agents (p-blockers) and class IV agents (nondihy
dropyridine calcium channel blockers) are commonly used to
inhibit arrhythmia induction and AV conduction (to decrease
rate) in patients with supraventricular or atrial arrhythmias.
Class III agents sotalol and dofetilide are used to treat atrial
and ventricular arrhythmias. Class III antiarrhy,thmic therapy
typically is initiated in an inpatient setting, with regular assess-
ment of the corrected QT interval (QTc) and caution exercised
in patients with kidney disease. Dofetilide is particularly noto
rious for common and dangerous drug drug interactions.
Amiodarone, a class lll multichannel blocker, is frequently
used to treat patients with recurrent ventricular tachycardia
(W) or AE Amiodarone has a low risk of pro arrhythmia; how-
ever, it is associated with thyroid, liver, lung, and eye toxicities
as well as neurologic side effects. Thyroid and liver function
should be monitored every 6 months, and pulmonary function
testing and ophthalmologic examination should be performed
annually. Amiodarone interacts with many drugs, including
warfarin, statins, and digoxin. Dronedarone, another class III
multichannel blocker, can be used in patients with paroxysmal
AF and no overt heart failure.
Digoxin and adenosine are excluded from the Vaughan
Williams classification. Digoxin is a positive inotropic agent
that also increases vagal activity, Ieading to a lower resting
heart rate. It can be used for rate control in patients with AF.
Adenosine is used in the acute treatment of arrhy'thmias to
interrupt AV conduction and terminate supraventricular tach-
ycardia (SVT). Administering adenosine can also help in deter-
mining the type of arrhythmia.
Sinus Tachycardia
Sinus tachycardia (sinus rhythm with a heart rate >100/min) is
the most common tachycardia and is typically the result of
physiologic demand or distress, including exercise, pain, acute
illness, hypovolemia, or anxiety. Diagnostic evaluation and
treatment are guided by the underlying cause.
lnappropriate sinus tachycardia (lST) is a disorder charac
terized by an elevated resting heart rate, with exaggerated
increases in heart rate with light actMgr. The sinus rate typically
decreases during sleep. IST frequently presents in women in
their second to fourth decade. Symptoms vary and can include
palpitations, light headedness, syncope (or near-syncope),
dyspnea, and fatigue. Most importantly, the diagnosis of IST is
based on the exclusion ofsecondary causes oftachycardia, such
as hyperthyroidism, anemia, pheochromocltoma, and struc-
tural heart disease. First line therapy is removal ofaggravating
factors and exercise therapy. In patients with bothersome and
persistent symptoms, pharmacologic therapy can be consid
ered, but the condition frequently improves over time.
Postural orthostatic tachycardia syndrome (POTS) is
another condition that often presents with tachycardia. POTS is
a form ofdysautonomia characterized by orthostatic intolerance
48

61. I
I
i
i
I
t
:
t
I
I
i
i
I
i
t
t
and excessive tachycardia, pafiicularly with standing. Diagnostic
criteria for POTS include an increase in heart rate of 30/min or
more or an increase to greater than 120/min within 10 minutes
of standing. The diagnosis is often confirmed with tilt table test-
ing. Behavioral modification, compression stockings, exercise
training, and increased fluid intake are important components
of therapy. Medical therapy lor POTS is highly variable and may
include p blockers, ivabradine (ofT'label use), tludrocorlisone.
selective serotonin reuptake inhibitors (off' label use), midodrine,
and pyridostigmine (oflf label use).
r(rY Potl{I
o Sinus tachycardia is the most common tachycardia and is
typically the result o[physiologic demand or distress,
including exercise, pain, fever, hypovolemia, and anxiety.
Su praventricu la r Tachycard ias
Clinical Presentation
SVTs are rapid heart rhythms that arise fiom the atrium or
require conduction through the AV node. AI.'and atrial flutter
are technically SVTs, although the term is generally reserved for
a narrow group of arrhythmias described herein. SVTs can
affect all age groups but are frequently seen in younger patients.
Prevalence is higher in women than in men. SVTs usually occur
in the absence olstructural heart disease, although echocardi
ography should be perfbrmed to exclude underlying cardiac
dysfunction or structural defects. Patients often have repeated
episodes of tachycardia and may report palpitations, a sensation
of pounding in the neck, fatigue, light headedness, chest dis
comfort, dyspnea, presyncope, and, less commonly, syncope.
The ECG typically demonstrates a narrow complex
tachycardia; however, wide QRS complexes (>t20 ms) may be
Arrhythmias
present in cases of'bunclle branch block, aberrancy, pacing,
or anterograde accessory pathway conduction (antidromic
tachycardia).
Vagal maneuvers, including the Valsalva maneuver or
carotid sinus massage, are first line therapy to restore sinus
rhythm acutely in patients with SVT. Adenosine can be used to
terminate SVT and simultaneously help diagnose its mecha-
nism. Tachycardias that terminate with adenosine are typically
AV node dependent (atrioventricular nodal reentrant tachy
cardia [AVNRT] and atrioventricular reciprocating tachycardia
tAVRll), whereas contir.rued atrial activity (P waves) during AV
block is consistent with atrial flutter or atrial tachvcardia.
Atrioventricular Nodal Reentrant Tachycardia
AVNRT accounts fbr two thirds of ail cases of SVT. not includ
ing cases of AF ancl atrial flutter. It is caused by a reentrant
circuit 'o,ithin the AV node that uses both the fast and slow
pathways. AVNRT is characterized by a short RP interval with
a retrograde P wave inscribed very close to the QRS complex.
AVNRT may be terminated with vagal manellvers or aden
osine. AV nodal blockers (0 btockers or calcium channel
blockers) are usecl to prevent recurrent AVNRT. In patients
with recurrent AVNRl'and those who do not tolerate or prefer
to avoid long term medical therapy, catheter ablation should
be considered. Catheter ablation ofAVNRT has a high success
rate, although it is associated with a 1',,{, risk lor injury to the AV
node necessitating pacemaker in.rplantation.
Atrioventricular Reciprocating Tachycardia
AVRT is an accessory pathway mediated tachyc:rrdia that is
often observed as preexcitation (delta wave) or.r ECG (Figure 22).
Early ventricular activation over the accessory pathway causes
l,lr
^J^
rrlt
,t r, .i .:
--
!J..=,-. !\?.+i-
\---'_-{ i
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.' *.-.,, -,,-
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r ,-,'--v,',-'..-",'..2'.--, >'--':1,-l--.\"r-1-.^ y''*---, r-t'..
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FIGURE 22.ECGdemonstratingsinusrhythmwithpreexcitationasindicatedbythepresenceof adeltawave(arror,,u).Theslurringofthe0RSupstrokerepresentspremature
depolarization of the ventricular tissue adjacent to the accessory pathway.
49

62. Arrhythmias
shortening of the PR interval, and the initial part of the QRS
complex is slurred because of premature ventricular depolari
zation in the myocardial tissue adjacent to the accessory path
way. In AVRT, conduction is anterograde over the AV node
(orthodromic, narrow-complex AVRT) in 90'7, to 95'1, of cases:
conduction is anterograde over the accessory pathway (anti
dromic, wide-complex AVRT) in the remaining cases.
Wolff-Parkinson-White (WPW) syndrome is defined by
symptomatic AVRT with evidence of preexcitation on resting
ECG. AF occurs in up to one third of patients with WPW syn-
drome. Rapid conduction over an accessory pathway in AF can
result in ventricular fibrillation (VF) and sudden cardiac death
(SCD), although this occurs in less than 1'1, of cases of WPW
syndrome.
Risk stratification for SCD can be performed with exercise
testing, although more frequently, patients are referred for
electrophysiolog, testing for both risk stratification and cura
tive ablation. Catheter ablation is first line therapy for patients
with WPW syndrome. The success rate for ablation is high
but is dictated by the location of the accessory pathway.
Antiarrhythmic therapy is second line therapy.
In asymptomatic patients with preexcitation on ECG,
management is controversial. Invasive testing is generally not
required unless the patient has a high risk occupation, such as
a commercial airline pilot.
PACs are O,pically treated with B blockers or calcium channel
blockers.
Atrial tachycardia can arise in the presence or absence of
structural heart disease. p Blocker or calcium channel blocker
therapy is first line treatment for symptomatic atrial tachy
cardia. Second line treatment is catheter ablation or antiar
rhythmic drug therapy. Ablation success rates are generalll'
lower in patients with atrial tachycardia than in patients $'ith
other SVTs.
Multifocal atrial tachycardia is typified by three or more
P-wave morphologies and a heart rate greater than 100,min
(Figure 23). It is usually seen in patients with severe pulmo'
nary disease.
f,EY POIlITS
. Vagal maneuvers may restore sinus rhythm in patients
with supraventricular tachycardia.
. Patients with recurrent supraventricular tachycardia are
treated with atrioventricular nodal blockers (p blockers
or calcium channel blockers) or catheter ablation.
r First-line therapy for patients with Wolff Parkinson
White syndrome is catheter ablation; antiarrhythmic
drugs are second-line therapy.
Atrial Fibrillation
AF is characterized by disorganized atrial activity with an
irregularly irregular ventricular response on ECG (Figure 24).
It is the most common sustained arrhythmia. t.ifetime risk fbr
AF is 25'l. in patients older than 40 years. lncidence is strongly
associated r,r'ith and increases with age. with 10'7, of persons
older than B0 years affected. AF is associated with an increased
risk for adverse cardiac events. including a fivefold increased
Premature Atrial Contractions and
Atrial Tachycardia
Premature atrial contractions (PACs) are early isolated beats
that arise from the atria. They are exceedingly common, and
their frequency increases with age. During ambulatory ECG
monitoring, only 1'7, of persons have no PACs. High PAC bur-
den is associated with increased risk for AF. Symptomatic
rVB
a!!
I
tIGURE 23.ECGshowingmultifocalatrialtachycardiatypiliedbythreeormoreP-wavemorphologies(arows).
v2
o\,,Ftc
50

63. Arrhythmias
I
aVl-
evF v6
L
tIGURE 24. ECGdemonstratingatrialfibrillation.NoclearPwavesareseen,andlhevenlricularresponseisirregular
risk for stroke. as well as increased risk for heart failure and
dementia. Among patients aged 55 years and older with an
ischemic neurologic event (i.e., stroke or transient ischemic
attack) of undetermined source. occult intermittent AF is
thought to be present in up to 25'1, of cases, and 30 day ambu
latory ECG monitoring is indicated for detection. If 30 day
ambulatory monitoring is inconclusive, implantation of a car
diac monitor (loop recorder) is reasonable to optimize detec-
tion of silent AF.
AF is usually the result of long standing risk factors, such
as diabetes mellitus, smoking, moderate to heaqr alcohol
consumption, obesity, hypertension, coronary artery disease
(CAD), heart failure, and obstructive sleep apnea. Behavioral
risk factor modification, including increased activity, weight
loss, smoking cessation, and abstinence lrom alcohol, is rec-
ommended. Bariatric surgery should be considered for appro-
priate candidates. Blood pressure and blood glucose control
and obstructive sleep apnea management also reduce risk.
AF is less commonly caused by reversible or acute physi-
ologic insults, including cardiac surgery pulmonary embo
lism, critical illness, or hyperthyroidism. When there are no
identified risk factors, a predisposing genetic background is
often present.
Clinical Presentation
Patients with AF may be asymptomatic or experience palpi-
tations, light headedness, dizziness, dyspnea, exercise intol
erance, chestpain, near syncope, or, rarely, syncope. In some
cases, AF can lead to hemodynamic compromise, especially
in patients with advanced diastolic dysfunction or restrictive
cardiomyopathy. Patients with AF less commonly present
with tachycardia induced cardiomyopathy, characterized by
asymptomatic left ventricular (LV) dysfunction or overt heart
failure.
AF is categorized according to its duration. Paroxysmal AF
stops spontaneously within 7 days of onset, whereas persistent
AF lasts lor 7 days or more. Long standing persistent AF is
continuous, with a duration ol more than 1 year. Permanent
AF refers to cases in which the physician and patient have
abandoned attempts to maintain sinus rhythm.
Acute Management
Urgent cardioversion (electrical or pharmacologic) is indicated
in patients with AF and hypotension, acute myocardial
ischemia, or decompensated heart failure. In patients with AF
or atrial flutter of more than 48 hours' duration or unknown
duration that requires immediate cardioversion, anticoagula-
tion should be initiated as soon as possible and continued for
at least 4 weeks unless contraindicated. The decision about
long term anticoagulation should be based on the thrombo
embolic risk profile and bleeding risk profile (see Long Term
Management).
In stable patients, the primary goals ol therapy are to (t)
prevent stroke, (2) control heart rate, (3) minimize or eliminate
symptoms, and (4) modify underlying risk factors. Upon diag-
nosis, reversible causes must be ruled out. All patients should
undergo thyroid function testing to evaluate for hyperthyroid-
ism. Patients with risk factors for or symptoms suggestive of
sleep apnea should undergo testing. Echocardiography is indi
cated to evaluate for potential valvular or other structural
I
51

64. Arrhythmias
heart disease. Echocardiography can also be used to assess left
atrial size, which helps determine the severity of the underly-
ing atrial myocardial dysfunction. Elective cardioversion can
be pursued in patients with significant symptoms who are
hemodynamically stable.
Acute Anticoagulation
In patients who are not undergoing cardioversion, acute intra-
venous anticoagulation is usually unnecessary; however, oral
anticoagulation should be initiated if the patient has sufficient
risk factors for stroke.
If cardioversion is planned, the duration of AF guides
therapy. AF with a known duration of less than 48 hours
confers a low risk for thrombus formation and subsequent
stroke, and preprocedural anticoagulation can be considered
in patients who are otherwise at low risk for stroke.
Preprocedural anticoagulation is reasonable as soon as pos-
sible before cardioversion for men with a CHATDST-VASc
score of 2 or greater and for women with a score of 3 or
greater followed by long-term anticoagulant therapy. In
patients in whom the duration of AF is unclear or in whom
AF has lasted longer than 48 hours, anticoagulation therapy
for 3 weeks is recommended before cardioversion. In patients
who would benefit from cardioversion sooner, transesopha
geal echocardiography can be performed to exclude the pres-
ence of left atrial appendage thrombus and facilitate urgent
cardioversion. Regardless ofthe duration or nature ofAE, all
patients who undergo cardioversion must receive anticoagu-
lants for at least 4 weeks following the procedure because of
an increased risk lor thromboembolic events after sinus
rhythm is restored.
Rate Control
Heart rate control is necessary in patients with rapid ventricu-
lar rates to improve cardiac function and alleviate symptoms.
Acutely, the goal heart rate should be between 60/min and
110/min. Commonly used medications include AV nodal block-
ers, such as metoprolol or diltiazem. Intravenous or oral admin-
istration may be appropriate depending on symptoms. In
patients with LV dysfunction, calcium channel blockers should
be avoided. Digoxin can be used as adjunctive therapy to
improve rate control, especially in patients with heart failure.
Long-Term Management
Anticoagulation
Arterial thromboembolic events are the most serious compli-
cation of AF and can occur with any form (paroxysmal, persis-
tent, or permanent). In patients with nonvalvular AF (AF in
the absence of a mechanical prosthetic valve or moderate/
severe mitral stenosis), the absolute risk for stroke is approxi
mately 4"1, per year; however, the presence of comorbidities
(e.g., heart failure, hypertension, diabetes, or vascular disease)
can increase the risk 15- to 20 flold. Hypertension is associated
with an increased risk for both AF and stroke; therefore, blood
pressure control is critical in the management of AF.
N/A = not applicable; OAC = oral anticoagulation.
"CHA2DS2-VASc scoring (maximum 9 points): One point each is given for heart
failure, hypertension, diabetes mellrtus, vascular disease (prior myocardial
infarction, peripheral artery disease, aortic plaque), Iemale sex, and age 65 to
74 years. Two points each are given for previous stroke/transient ischemic attack/
thromboembolic disease and age >75 years.
bData
from Friberg L, Rosenqvist M, Lip GY. Evaluation of risk strati{icatron schemes
for ischaemic stroke and bleeding in 182 618 patients with atrial fibilllation: the
Swedish Atrial Fibrillation cohort study. Eur Heart J. 20 1 2;33:1 500- l 0.
I PMID:
222464431 doi:1 0.1 093/eurheartj/ehr488
'Recommendations from January CT, Wann LS, Calkins H, et al. 201 9 AHA/ACC/
HRS focused update of the 20 1 4 AHA,IACCIHRS guideline for the management of
patients with atrial fibrillation: a repon of the American College of Cardiology/
American Hean Association Task Force on Clinical Practice Guidelines and the
Heart Rhythm Society. J Am Coll Cardiol. 20 1 9;74:1 04'1 32. I PMI D: 3070343 1
]
doi:1 0.1 01 6/j.jacc.20 1 9.01 .0 1 1
cHA2D52-
VASc Score'
lncidence of
lschemic Stroke/
100 Patient-Yearsb
Stroke Prevention
Therapy''
0
1
0.2
0.6
2.2
Men
None
Consider
OAC
OAC
OAC
oAc
OAC
oAc
Women
N/A
None
Consider
oAc
OAC
oAc
OAC
OAC
2
3
4
5
6+
3.2
4.8
7.2
10.3
Risk Strotilicotion
Stroke prevention with oral anticoagulation depends on the
patient's thromboembolic risk in the absence of obvious con
traindications. Although several risk stratification scores are
available, current guidelines recommend use of the CHA2DS,
VASc score in patients with nonvalvular AF. Adjusted stroke
rates and recommendations for antithrombotic therapy based
on the CHA2DST-VASc score are shown in Table 18. The 2019
American College of Cardiologr/American Heart Association/
Heart Rhythm Society focused update on AF recommends
anticoagulation to prevent stroke in patients u,ith nonvalvular
AF who have a CHATDS, VASc score of 2 or greater in men or 3
or greater in women. The American College of Chest Physicians
guideline on antithrombotic therapy for AF recommends anti-
coagulation for patients with one or more non sex CHA2DS2
VASc stroke risk factors (score of >1 in men or >2 in women).
Antiplatelet therapy alone is no longer routinely used for
stroke prevention in AE
Antico agulant S ele ctio n
Oral anticoagulation in patients with AF can be accomplished
with a vitamin K antagonist (warfarin) or direct oral antico-
agulant (DOAC) (Table f9). Anticoagulation in patients with
valvular AF (moderate/severe mitral stenosis or mechanical
valve prosthesis) should be obtained with warfarin; DOACs
TABLE 1 L CHA2DS2-VASc Score, Adjusted Stroke Rates,
and Stroke Prevention Therapy Recommendations for
Nonvalvular Atrial Fibrillation and Flutter
52

65. Arrhythmias
Medication Frequency Type ofAF Cautions and Dosing
Avoid in pregnancy
Caution with idiopathic thrombocytopenic purpura, heparin-
induced thrombocytopenia, liver disease, protein C or S
deficiency
Many drug and food interactions
Caution with use of P-glycoprotein inhibitors
Reduce dose with CrCl 15-30 mUmin/1.73 m2
Avoid with CrCl <30 mUmin/1 .73 m2, moderate liver impairment,
use of strong P-glycoprotein inhibitors, or use of strong
cytochrome P-450 inducers and inhibitors
Reduce dose with CrCl 30-50 mUmin/1 .73 m2
Avoid with use of strong P-glycoprotein inhibitors or strong
cytochrome P-450 inducers and inhibitors
Reduce dose with two of the following criteria: creatinine level
>1.5 mg/dl (133 pmol/L), age >80 y, or weight <60 kg (132 lb)
Avoid with use of strong cytochrome P-450 inducers and inhibitors
Avoid with CrCl >95 mUmin/1 .73 m2 (reduced stroke prevention
effectiveness). Reduce dose with CrCl 30-50 mlJmin/1 .73 m2,
weight <60 kg (1 32 lb), or concomitant use of verapamil or
quinidine (potent P-glycoprotein inhibitors)
Dose adjusted to INR
Twice daily Nonvalvula r
Once daily Nonvalvular
Twice daily Nonva lvular
Once daily Nonvalvular
Warfarin (vitamin K
antagonist)
Valvular" or
nonvalvular
Apixaban (factor Xa
in h ibitor)
Edoxaban (factor Xa
inhibitor)
Dabigatran (direct
thrombin inhibitor)
Rivaroxaban (factor Xa
inhibitor)
"Valvular atrial fib,riliation refers to atrial fibrillation in the presence of moderate to severe mitral stenosis or mechanical valve prosthesis.
AF = atrial fibrillation; CrCl = creatlnine ciearance.
TABTE 19. Anticoagulants Approved for Stroke Prevention in Atrial Fibrillation
\
are not approved for use in valvular AF or those with any
mechanical heart valve. DOACs are recommended in prefer-
ence to warfarin in DOAC eligible patients, including patients
with AF and no valvular disease as well as patients with AF
and aortic valve disease, mitral regurgitation, tricuspid regur
gitation, or mild mitral stenosis in the absence of a mechani
cal prosthesis.
Warfarin
Dose-adjusted warfarin is an effective, low-cost therapy; how-
ever, its limitations include the need for frequent monitoring
and adjustment and numerous food and drug interactions.
The safeg and efficacy of warfarin therapy depend on the
time that the patient is in the therapeutic range (lNR, 2 3).
Management of warfarin overdose and warfarin-related bleed-
ing is discussed in MKSAP 19 Hematologr.
Direct Acting Oral Anticoagulants
Four DOACs are approved for the prevention of stroke in AF.
Dabigatran, an oral direct thrombin inhibitor, is superior to
warfarin for the prevention of ischemic stroke and results in
less intracranial bleeding. Patients taking dabigatran have a
higher risk for gastrointestinal bleeding relative to warfarin
and may experience dyspepsia. Rivaroxaban, a direct factor Xa
inhibitor, is noninferior to warfarin in the prevention of stroke
or systemic embolism and is associated with less intracranial
and fatal bleeding. As with dabigatran, patients taking
rivaroxaban have a higher risk for gastrointestinal bleeding
compared with those taking warfarin. Apixaban, another oral
factor Xa inhibitor, is superior to warfarin for stroke preven-
tion and confers less risk for major bleeding, including intra
cranial and gastrointestinal bleeding. Edoxaban is noninferior
to warfarin for stroke prevention and is associated with less
major bleeding.
DOACs have shorter half lives than warfarin; however,
there are no quick, readily available serum assays to accurately
determine anticoagulant activity. Andexanet alfa or 4-factor
prothrombin complex concentrates are recommended for life-
threatening bleeding due to rivaroxaban, apixaban, or edoxa
ban. Idarucizumab is a dabigatran reversal agent available for
emergency invasive or surgical procedures or in cases of
uncontrolled or life threatening bleeding.
Kidney and liver function should be evaluated before
initiation of a DOAC and reevaluated at least annually. See
MKSAP 19 Hematology for recommendations regarding
DOAC dosing in patients with chronic kidney or Iiver
disease.
Specific Populations
In patients with AF who have undergone percutaneous
coronary intervention for acute coronary syndrome, both
anticoagulant and antiplatelet therapies are necessary.
Among these patients with a CHA2DS2 VASc score of 2 or
greater, "double therapy" with clopidogrel or ticagrelor plus
a DOAC is recommended over "triple therapy" with an oral
anticoagulant, aspirin, and P2Y12 inhibitor to reduce the
risk for bleeding. In patients taking two or more antithrom
botic agents, it is recommended that a proton pump
53

66. Arrhythmias
inhibitor be initiated and NSAIDs be avoided to reduce the
risk of gastrointestinal bleeding.
In patients with AF and stable CAD, treatment with riva-
roxaban alone is noninferior to rivaroxaban plus aspirin in
the prevention of the composite end point of stroke, systemic
embolization, myocardial infarction (MI), need for revascu
Iarization, or death from any cause. Rivaroxaban monotherapy
also is associated with significantly less bleeding.
Rate-Only Versus Rate Plus Rhythm Control Treatment
The first priority among patients with AF is to control the ven-
tricular rate. Rate control with a resting heart rate goal ofless
than 80/min is reasonable for symptomatic management of
AF. A lenient rate-control strates/ (resting heart rate <110/min)
may be reasonable for asymptomatic patients without LV dys-
function. p Blockers, calcium channel blockers, and, less
often, digoxin are used to control the ventricular rate, although
combination therapy may be necessary. Aside from resting
heart rate assessment. evaluation of the heart rate with activ
ity, such as with a 6 minute walk test, stress test, or 24 hour
ambulatory ECG monitoring, may be performed.
Although historical trials have not proved a mortality
benefit to maintaining sinus rhythm, numerous studies
have demonstrated superior symptom treatment with rate
plus rhythm control compared with rate control alone.
Furthermore, because the long term effects of rate only con-
trol are unknown, rhythm control is often pursued in younger
patients (aged <50 years) and may improve long term clinical
outcomes. A strategz of early rhythm control (within 1 year of
diagnosis) among patients at high risk for adverse cardiovas
cular events was tested in the randomized EAST AFNET 4 trial.
High risk was defined as age older than 75 years or a previous
transient ischemic aftack or stroke, or meeting two of the
following criteria: age older than 65 years, female sex, heart
failure, hypertension, diabetes, severe CAD, chronic kidney
disease, and LV hypertrophy. Early rhythm control (drugs or
ablation) reduced the primary composite end point of cardio-
vascular death, stroke, or hospitalization for heart failure or
acute coronary syndrome, compared with usual care. Rhythm
control may require cardioversion in addition to antiarrhyth
mic therapy, which is guided by the patient's comorbid condi-
tions and safet5z considerations. However, catheter ablation
has been shown to be more effective than antiarrhy,thmic
drugs, with an acceptable safety profile.
Nonpharmacologic Approaches to Atrial Fibrillation
Catheter ablation with pulmonary vein isolation is an effective
rhy.thm control therapy in patients with recurrent sympto
matic AF despite antiarrhy.thmic drug therapy. Catheter abla
tion is most effective in patients without significant left
atrial enlargement and multiple comorbid conditions. One
year after the procedure
,7O"/,, to 90'7, of patients with paroxys
mal AF are symptom-free; however, success rates vary.
Complications include thromboembolism (0.5'2, 1'l,, risk), tam-
ponade, and vascular complications (e.g., insertion hematoma,
pseudoaneurysm, arteriovenous fi stula, retroperitoneal bleed
ing). Longer term complications, such as pulmonary vein ste
nosis, are uncommon.
AV node ablation is an option for patients with AF who
have continued symptomatic tachycardia despite rate and
rhyhm control therapy. Therapeutic ablation of the AV node
requires implantation of a permanent pacemaker. These
patients remain in AF and still require anticoagulation.
Approximately lOn/,' to 25"1' of patients with AF have con-
traindications to oral anticoagulation or discontinue therapy
for various reasons, including bleeding events. In patients who
are at moderate to high risk for stroke (CHA,,DS, VASc score
>3), left atrial appendage occlusion to prevent stroke and sys
temic thromboembolism can be considered. Occlusion of the
left atrial appendage can be achieved percutaneously rt'ith an
implanted self expanding device or r.tith surgical closure.
However, appropriate patient selection remains a challenge,
and most patients undergoing percutaneous left atrial append-
age occlusion require at least short term oral anticoagulation
while the device endothelializes. These patients also remain on
long term antiplatelet therapy.
Management in Patients With Heart Failure
AF complicates nearly 40"/,, of cases of heart failure, and the
combination of AF and heart failure dramatically worsens
outcomes. Several clinical trials have demonstrated that
aggressive efforts to achieve rhythm control in patients with
AF and concomitant heart failure decrease morbidity and
mortality. In patients with heart failure with reduced ejection
fraction, recent clinical trials have shown that catheter abla-
tion of AF is associated with a favorable effect on morbidity
and mortality compared with medical therapy. Patient selec
tion for catheter ablation remains important. and many of the
trials enrolled selected patients.
Subclinical Atrial Fibrillation
The entity of subclinical AF is being increasingly recognized
because of the growing prevalence of implantable cardiac
devices and widespread availability of ambulatory ECG moni
tors (e.g., mobile continuous telemetry) and consumer devices
(e.g., personal ECG monitors, smartwatches). Subclinical AF
most commonly refers to AF that is asymptomatic and brief
(definitions range from 20 seconds to <24 hours). Although
definitive studies to guide management are lacking, there are
several important considerations. First, episodes are frequently
asymptomatic and short lived; thus, symptom management
and rate control are rarely required. Second, subclinical AF is
associated with progression to AF; therefore, it is reasonable to
consider traditional AF risk factor modification. Lastly. stroke
risk in these patients is unclear. Studies have varied in their
definition ofsubclinical AF and its associated stroke risk. and
there is no consensus on the net clinical benefit of anticoagu
Iation for subclinical AF. One notable exception is the detec-
tion of AF lasting more than 30 seconds among patients with
embolic stroke of undetermined source (cryptogenic stroke);
54

67. Arrhythmias
anticoagulation is routinely indicated in these patients.
Implantable loop recorders to detect subclinical AF may be
indicated in selected patients with embolic stroke of undeter
mined source.
. Urgent cardioversion to sinus rhythm is indicated in
patients with atrial fibrillation who have hypotension,
acute myocardial ischemia, or decompensated heart
failure, regardless of atrial fibrillation duration.
. Current guidelines recommend calculation of the
CHA2DS2-VASc score for stroke risk stratification in
patients with nonvalvular atrial fibrillation; patients
with a CHATDST-VASc score of 2 or greater in men or 3
or greater in women should be treated with oral antico-
agulation to prevent stroke.
o Direct-acting oral anticoagulants are recommended in
preference to warfarin in patients with atrial fibrillation,
excluding those with moderate to severe mitral stenosis
or a mechanical valve prosthesis.
o In patients with atrial fibrillation, rate plus rhythm con-
trol offers superior symptom management compared
with rate control alone.
Atrial Flufter
Atrial flutter is an organized macro-reentrant tachycardia with
discrete regular atrial activity on ECG, usually with an atrial rate
of 250/min to 300/min. Tlpical atrial flutter is characterized
electrocardiographically by a sawtooth pattern with inverted
flutter waves in leads II, II], and aVF and positive flutter waves in
lead V, (Figure 25). Typical atrial flutter, the dominant form in
patients without prior cardiac disease, is the result of counter-
clockwise reentry around the tricuspid annulus. Atypical flutter
is primarily seen among patients with prior ablation for AF or
prior cardiac surgery In atypical flutter, the circuit is usually in
other locations in the right and left atria.
Management of anticoagulation in the setting of chronic
atrial flutter is similar to that for AF; however, a rhythm con-
trol stratery is favored in atrial flutter because rate control may
be difficult and often requires high doses of more than one AV
nodal blocker. Catheter ablation is the definitive treatment for
typical atrial flutter because of a very high success rate (>95%)
and low complication rate. Oral anticoagulation in patients
with atrial flutter without ablation is approached in the same
manner as in patients with AE
r Catheter ablation is the definitive treatment for typical
atrial flutter, with a high success rate (>957.) and low
risk for complications.
Ventricular Arrhythmias
Premature Ventricular Contractions
Premature ventricular contractions (PVCs) occur in up to 75%
of healthy persons. Symptoms include palpitations or the per-
cepflon ofskipped beats. Forceful beats are caused by increased
cardiac filling during the pause following the PVC. PVCs are
t I G U R E 2 5 . ln this ECG demonstrating typical atrial flutter, negatively directed sawtooth waves are seen in the inferior leads (ll, lll, and aVF), and positive waves a re seen
in lead V1. ln the bottom rhythm strip,2:1 and 4:1 conduction patterns aIe most easily seen.
55

68. Arrhythmias
more common in patients with hypertension, LV hypertrophy,
previous MI, and other forms of structural heart disease, such
as nonischemic cardiomyopathy.
In the absence of high risk features (syncope, family his
tory ofpremature SCD, structural heart disease), reassurance
is often appropriate, and medical therapy is unnecessary.
However, PVCs require treatment when symptoms are bother-
some or frequent (>to% ofall beats or 10,000 PVCs per day).
PVC-induced cardiomyopathy may result from frequent PVCs
(generally >10'2,-15% of beats), although it occurs only in a
minority of patients (see Heart Failure).
First line treatment for PVC suppression is p blocker or
calcium channel blocker therapy. p-Blockers are preferred in
patients with ventricular dysfunction. Alternative antiarrhyth
mic therapy may be used if PVCs persist despite p-blockade or
calcium channel blockade. The selection of an antiarrhlthmic
medication for PVC suppression depends on many factors,
including age, kidney function, cardiac structure, and comor
bid conditions. Catheter ablation should be considered in
patients with continued frequent PVCs despite medical ther
apy, patients who cannot tolerate medical therapy, and patients
who develop PVC induced cardiomyopathy.
Ventricular Tachycardia
Clinical Presentation
VT is awide-complex tachycardia (QRS complex >120 ms). The
differential diagnoses for wide-complex tachycardia include
SW with aberrancy, preexcited tachycardia (antidromic tachy
cardia), ventricular paced rhythms, and most commonly, VL
In adult patients with known structural heart disease, 95'7, of
wide-complex tachycardias are VT, and additional ECG or
clinical criteria are often unnecessary. However, in patients
without known structural heart disease, several important
clinical and ECG features can distinguish VT from other condi
tions. Key features of VT on ECG include AV dissociation,
fusion beats. and capture beats (Figure 26). When the origin
of a wide complex tachycardia is in question, VT should be
assumed.
Ventricular arrhythmias most commonly occur in
patients with structural heart disease, including both ischemic
and nonischemic cardiomyopathy, in whom the presence of
abnormal conduction and/or myocardial scar tissue facilitates
the development of VT. In these patients, sustained VT
(>30 seconds) can lead to hypotension, syncope, VF, and car
diac arrest; however, on occasion, VT can be well tolerated.
Thus, the absence of hemodynamic compromise does not
exclude VT as a diagnosis.
VT in the absence of structural heart disease (idiopathic
VT) typically arises from the ventricular outflow tracts, fasci
cles, and papillary muscles. Patients with idiopathic VT usu-
ally present with palpitations in the third to fifth decades of
life. Episodes of syncope are uncommon. Arrhythmic events
are often triggered by stress, emotion, or sleeplessness.
Evaluation and Management
Patients with VT and hemodlmamic instability should undergo
immediate direct current cardioversion (see Sudden Cardiac
Arrest for a discussion of advanced cardiac life support).
I
I
I
va
y6
..1- _.... r
v2
li
.vL
.vF u8'
\ \\
:
1
)
t
I
FIGURE 26. ECGdemonstratingaregularmonomorphicwide.complextachycardiainaleftbundlebranchblockpattern.Thepresenceof
atrioventriculardissociation
confirms the diagnosis of ventricular tachycardia. The arrows identify nonconducting
p
waves.
56

69. Arrhythmias
Intravenous amiodarone should be administered if the VT per-
sists or recurs after cardioversion. Patients with VT or VF with
ST elevation MI should undergo emergency revascularization.
In patients with hemodynamically stable VT not in the setting
of an acute MI, intravenous procainamide can be useful for VT
termination, or intravenous amiodarone or sotalol may be
considered.
Evaluation with resting ECG, exercise treadmill testing (to
provoke arrhl,thmias), and cardiac imaging (to identiS/ struc
tural heart disease) is indicated in patients with VI, Cardiac
magnetic resonance (CMR) imaging generally allows for tissue
characterization, making it an important modality in the evalu-
ation of myocardial diseases that may manifest as VI, including
arrhlthmogenic right ventricular cardiomyopathy, myocardial
fibrosis/scarring, cardiac sarcoidosis, and other infiltrative car
diomyopathies (e.g., amyloidosis). CMR imaging can also clariSr
the extent and pattern of myocardial scarring.
Patients with ischemic cardiomyopathy who present with
VT should be considered for angiography and revasculariza-
tion, if appropriate, primarily to reduce ischemic burden
rather than to treat the arrhythmia. Patients with cardiomy-
opathy and heart failure should receive guideline directed
medical therapy to minimize risk for ventricular arrhythmia.
In patients with recurrent VT despite B-blocker therapy, anti-
arrhythmic drug therapy or catheter ablation may be consid
ered. Contemporary evidence suggests ablation to be more
effective than medical therapy. Implantable cardioverter-
defibrillator (lCD) placement is indicated for secondary pre-
vention of SCD in patients with structural heart disease or
cardiomyopathy who have sustained VT/VF, provided that
clearly reversible causes have been excluded (e.g., acute coro-
nary ischemia, cocaine ingestion).
In patients with idiopathic VT. calcium channel blockers,
especially verapamil, and p blockers are flrst-line therapy.
Catheter ablation can be considered if symptoms continue
despite these therapies. ICD placement is generally unneces
sary in idiopathic VT because ofthe benign prognosis and high
efficacy of other therapies.
l(EY POTnTS
HVC r Premature ventricular contractions (PVCs) without
high-risk features (syncope, family history of premature
sudden cardiac death, structural heart disease) are
managed with reassurance; treatment is reserved for
bothersome symptoms or frequent PVCs.
. Idiopathic ventricular tachycardia occurs in patients
without structural heart disease and is unlikely to cause
slmcope or sudden cardiac death; p-blockers and calcium
channel blockers are first-line therapy, with catheter abla-
tion reserved for symptoms refractory to drug therapy.
o Ventricular tachycardia in the setting of structural heart
disease often requires eggressive rhythm control along
with implantable cardioverter defibrillator implantation
for secondary prevention.
I n herited Synd romes Cha racterized
by Sudden Cardiac Death
Patients younger than 40 years without ischemic or structural
heart disease who have unexplained cardiac arrest, unex
plained near drowning, or recurrent high risk syncope should
be evaluated for inherited arrhythmia and/or cardiomyopathy
syndromes. Unexplained premature death (age <35 years) or
sudden death (age <40 years) in a first-degree family member
also should raise suspicion for an inherited arrhythmia syn-
drome and prompt referral to a cardiovascular specialist, with
genetic counseling and testing as indicated by clinical find
ings. The diagnosis of inherited arrhythmia syndromes can be
complex because of variable penetrance and expressivity of
these disorders. Characteristic findings and treatments for
some common syndromes are reviewed in Table 20.
Genetic long QT syndrome is among the most common
inherited arrhythmias, affecting between 1 in 1000 and 1 in
5000 persons (Figure 27). However, the presence of a pro
longed QTc
(>440 ms in men, >460 ms in women) alone is
insufficient to diagnose long QT syndrome. QT
prolongation
can have many causes, most of which are acquired, such as
medication use, structural heart disease, and electrolyte
abnormalities. Drugs that have been implicated in QT prolon-
gation include antiarrhythmic agents, antibiotics (including
some macrolides and fluoroquinolones), antipsychotic drugs,
and antidepressants. Given great variability in QTc across pop
ulations as well as variable penetrance ofand subtypes oflong
QT slmdrome, diagnosis is complex and should be referred to a
specialist. A list of drugs categorizcd by their potential to cause QT
prolongation is available at https: //crediblemeds.org. Treatment
includes
B blockers (first line); avoidance of QT-prolonging
drugs; and, for selected patients, ICD implantation, surgical sym-
pathectomy, and exercise restriction.
Brugada syndrome is distinguished by right precordial
ECG abnormalities, including ST segment coving (concave or
linear downsloping ST segment) in leads V, through V., with
or without right bundle branch block, VF, and cardiac arrest
(Figure 28). Brugada syndrome has an increased prevalence in
men and persons of Asian descent. Arrhythmic events (includ
ing SCD) in patients with Brugada syndrome are more com
mon at night during sleep. Abnormalities on ECG can be
intermittent and may be elicited by fever or pharmacologic
challenge with sodium channel blockade, such as procaina
mide infusion.
Hypertrophic cardiomyopathy and arrhythmogenic right
ventricular cardiomyopathy/dysplasia (ARVC/D) can often
present as SCD in young persons. Hypertrophic cardiomy
opathy and arrhythmic risk stratification are discussed in
Myocardial Disease. ARVC/D usually appears between puberty
and young adulthood; however, it can also be identified in
older age. Patients with ARVC/D typically present with fre
quent ventricular ectopy and/or monomorphic Vl although
in severe cases, patients can present with heart failure. The
diagnosis is established by ECG abnormalities, family history
57

70. Arrhythmias
T&*t[ :*. lnherited Syndromes Characterized by Sudden Cardiac Death
Disorder
Long QTsyndrome
Brugada syndrome
Catecholaminergic polymorphic VT
ARVC/D
Hypertrophic cardiomyopathy
Presenting Symptoms and
Characteristi< Findings
Syncope during sleep, auditorytriggers,
and/or during exercise (depending on
subtype); QTc usually >460 ms; torsades
de pointes
Syncope during sleep, VF, coved
ST-segment elevation in early precordial
leads (V1 through V3)
Syncope, polymorphic or bidireaional W
during exercise or emotional distress
Syncope, palpitations, T-wave inversions in
leads V1 through at least V3, monomorphic
VT, frequent PVCs, and abnormal right
ventricular size and function on
echocardiography or CMR imaging
Syncope, VF during exercise, increased
QRS voltage with or without repolarization
abnormalities on ECG
Potential Treatments'
p-Blockers, avoidance of OT-prolonging drugs;
selected patients: lCD, sympathectomy,
exercise restriction
lCD, avoidance or man
(drugs, feved, catheter
agement of triggers
ablation
p-Blockers, verapamil, flecainide, lCD, exercise
abstinence (uniform)
lCD,
B-blockers, antiarrhythmic medications,
catheter ablation, exercise abstinence
(uniform)
lCD, p-blockers, disopyramide, catheter
ablation, surgical myectomy
ARVC/D = arh)'thmogenic right ventricular cardiomyopathy/dysplasia; CMR = cardiac magnetic resonance; ICD = implantable cardioverter defibrillator; PVC = premature
ventricular contraction; OTc = corrected OT interual; VF = ventricular fibrillation; VT = ventricular tachycardaa.
antiarrhythmic drugs are often required for recurrent ventricular arrhythmias.
t/!t
FIGURE 2l .ECGdemonstratingsignificantprolongationofthe0Iinterval aswell asabnormal morphology,whichisbestseenintheearlyprecordial leads.0f note,
when the heart rate is less than 60/min, the absolute 0T interval is routinely used instead of the conected 0T interval (0Tc) for heart rate. A 0T or OTc greater than 500 ms is a
risk factor for adverse events.
va
vt
\6
vl
vil
\a
alrR
at
,tG
t
58

71. arrhythmias, and structural abnormalities of the right ventri
cle. CMR imaging can demonstrate enlargement (segments of
poorly contracting heart muscle), focal aneurysms, and wall
motion abnormalities in the right ventricle (hypokinesis).
ARVC/D is usually progressive, and patients should abstain
from vigorous exercise. Patients with ARVC/D and cardiac
arrest or risk factors (nonsustained VT, inducible VT) should
be offered an ICD. B
Blockers are first line therapy for ven
tricular arrhythmias, although antiarrhythmic therapy with
sotalol or amiodarone or catheter ablation is often required for
recurrent VT.
IEY POTXI
. Unexplained premature death or sudden death in a
first degree family member should raise suspicion for
an inherited arrhythmia syndrome and prompt referral
to a cardiovascular specialist.
Sudden Cardiac Arrest
Epidemiology and Risk Factors
SCD is defined as a fatal event or collapse within t hour of
symptom onset in a person without recent acute illness. In
patients in whom death was unwitnessed, SCD is considered
to have occurred if the patient was known to be alive and well
within the last 24 hours. VT and VF are the most common
causes of SCD.
Arrhythmias
v3
In the United States, more than 350,000 episodes of SCD
occur each year. The annual risk for SCD is 1:1000 in the general
population. The highest incidence occurs in patients with pre-
existing structural heart disease, although LV function is normal
in most patients who experience SCD. fusk factors include heart
failure, diminished LV function, previous MI, unexplained syn-
cope, LV hypertrophy, nonsustained ventricular arrhythmia,
chronic kidney disease, and sleep apnea. It is important to dis
tinguish between MI precipitating death and nonischemic SCD
when a family history of cardiac disease is obtained.
Acute Management
Cardiac arrest necessitates immediate cardiopulmonary resus
citation (CPR) and advanced cardiac life support (Figure 29).
Interruptions in chest compressions should be minimized, and
defibrillation should occur as soon as possible in patients with
a shockable rhy'thm because time to defibrillation is an impor-
tant determinant of likelihood of survival to hospital discharge.
The presence or absence ol a shockable rhythm guides
management after CPR initiation. In patients with out-of-
hospital arrest, early cardiac catheterization has value in (1)
providing early diagnosis of the etiologr of arrest, (2) facilitat
ing early intervention when applicable, and (3) providing
opportunities for advanced hemodynamic support (e.g., extra-
corporeal membrane oxygenation). Any reversible causes,
such as tamponade, should be identified and treated.
I aVL
aVFlI
t I G U R E 2 8 . ECG demonstrating a type 1 Brugada pattern, >2 m m J-point elevation, ST,segment coving (concave or linea r downsloping 5T seg ment) (arorvs), a nd T,wave
inversions in leads V, through V3.
59

72. Arrhyrthmias
Yc! ilo
@
Shock EpinephrineASAP
Shock
Yer
Shock
No
Pnco, = partial pressure end{idal carbon dioxide; pW = pulseless ventricular tachycardia; VF
=
ventricular fibrillation.
ahajournah.org/doi/1 0.1 1 61/C1R.000000000000091 6).
nar resuscitation and emergency Grdiovascular care. Circulation. 2020;142:5374
[pMlD: 33081 529] doi:1
()_1
1 61 /C|R.00000b000000091 6. @2020 AmeriGn Head A$ociation.
No
Yer
No
Yer
Asystole/PEA
Goto5orT
o lf no signs of return of
spontaneous circulation
(ROSC), go to 10 or 1 1
r lf ROSC, go to Post-Cardiac
Arrest Care'
. Consider appropriateness oI
continued resuscitation
. Push hard (at least 2 inches
[5 cml) and fast (100-120/min)
and allow complete chest recoil.
o Minimize interruptions in
compressions.
o Avoid excessive ventilation.
. Change compressor every 2
minutes, or sooner if fatigued.
. lf no advanced airway,30:2
com pression-ventilation ratio.
. Ouantitative waveform
capnography
- lf Prrco, is low or decreasing,
reassess CPR quality.
. Biphasic: Manufacturer
recommendation (e.9., inhial
of 12O-2OO Jl; if unknown, use
maximum available.
Second and subsequent doses
should be equivalent, and higher
doses may be considered.
o Monophasic 360 J
. Epinephrine lV/lO dose:
1 mg every 3-5 minutes
. Amiodarone lVllO dose:
First dose: 300-mg bolus.
Second dose: 1 50 mg.
or
Lidocaine lVllO dose:
First dose: 1-1 .5 mg/kg.
Second dose: 0.5-0.75 mg/kg.
o Endotracheal intubation or
supraglottic advanced airway
o Waveform capnography or
capnometry to confirm and
monitor ETtube placement
. Once advanced airway in place,
give 1 breath every 6 seconds
(10 breaths/min) with continuous
chest compressions
o Pr:lse and blood pressure
. Abrupt sustained increase in
Percq (typically >40 mm Hg)
. Spontaneous arterial pressure
waves with intra-arterial
monitoring
'Hypovolemia
. Hypoxia
o Hydrogen ion (acidosis)
'
Hypo-/hyperkalemia
'Hypothermia
o Tension pneumothorax
o Tamponade, cardiac
o Toxins
o Thrombosis, pulmonary
o Thrombosis, coronary
60

73. Valvular Heart Disease
Device Therapy for Prevention of Sudden Death
ICDs have demonstrated efficacy in the primary and second
ary prevention of SCD through their treatment, not preven
tion. of VT/VF with defibrillation. Patients with sustained
ventricular arrhythmias (>30 seconds) or cardiac arrest
without a reversible cause have a class 1 recommendation for
secondary prevention ICD placement. ICD placement is rec
ommended for the primary prevention of SCD in patients with
ischemic or nonischemic cardiomyopathy, ejection lraction
less than 35'1,. and New York Heart Association functional
class II or III heart failure. Patients with heart failure and inter
ventricular conduction defects (predominantly left bundle
branch block) often benefit from cardiac resynchronization
therapy or cardiac resynchronization therapy in combination
with a defibrillator (see Heart Failure).
In the past, ICDs were implanted almost exclusively using
a transvenous approach. New techniques allow for implanta
tion of defibrillators in the lateral chest at the midaxillary line
adjacent to the heart with tunneling of the lead under the skin
next to the sternum. Subcutaneous defibrillators have several
advantages, including reduced risk for device infection.
Infection is a major and chronic risk of implanted cardiac
devices. Pacemaker and defibrillator infections. even of the
pocket alone, must be managed aggressively to reduce mor
bidity and mortality. However, device infection presentation
may be insidious and underwhelming, potentially limited to
only pain or erythema over the pocket. Nevertheless, any
patient suspected ofhaving a cardiac device infection should
be referred urgently for specialist evaluation. Empiric antibiot
ics alone (without blood cultures) may cloud or delay diagno-
sis, and diagnostic aspiration of the device pocket is never
indicated because of the risk for introducing infection in an
uninfected pocket. Effective treatment of cardiac device infbc
tion usually includes complete extraction ol all hardware,
debridement of the pocket, sustained antibiotic therapy, and
re implantation at a new location after infection has been
eradicated.
I(EY POITIS
. Implantable cardioverter-defibrillators are effective for
primary and secondary prevention ofsudden cardiac
death.
. Infection of cardiac implanted devices may present
insidiously but requires urgent and specialized evalua
tion, often necessitating complete hardware removal for
a durable cure.
Valvular Heaft Disease
General Principles
Valvular hearl disease (Vl{D) involves cardiac dysfunction due
to structural or functional valve abnormalities resulting ftom
failure of the valves to either competently close (regurgitation)
or effectively open (stenosis). VHD affects approximately 20
million persons in the United States. Although there are con
genital forms, VHD is largely age dependent, with a prevalence
of 3'l, to 6'7, in persons aged 65 years or older.
Many heart valve lesions progress slowly, causing patients
to limit their activity unconsciousiy in response; therefore, a
careful history and detailed physical examination are essen
tial. Exertional dyspnea is the most common symptom.
Depending on the lesion and severity, other symptoms include
angina, syncope, palpitations, Iower extremity edema, and
increasing girth (ascites). Typical physical examination find
ings for valvular and other cardiac lesions are described in
Table 21. Twelve lead ECG, chest radiography, and transtho
racic echocardiography (TTE) are the essential tests used to
evaluate VHD.
To facilitate the timing of monitoring and interr,zention,
VHD is classified into four stages (A through D), which con
sider risk factors, presence of symptoms, Iesion severity, ven
tricular response to the volume or pressure overload caused by
the lesion, effect on the pulmonary or systemic circulation,
and heart rhl,thm changes (Table 22). Sur-veillance intervals for
echocardiographic evaluation based on disease severity are
listed in Table 23 on page 64.
Medical therapy, although often effective for syn.rptom
palliation, has not been shown to prevent VHD progression
or improve long term survival in patients with VHD. Surgery,
however, can be a life saving intervention in select patients,
and surgicai risk calculation is a key component of the
patient evaluation. Risk calculation involves assessment of
the patient's age, morbidities, frailty, and impediments spe
cific to the procedure under consideration (e.g.. previous
chest irradiation for a sternotomy approach). Risk calculators
derived fiom national databases can assist in estimating risk
for morbidity and mortality for surgical valve procedures.
One such calculator, the Society o1'Thoracic Surgeons Adult
Cardiac Surgery Risk Calculator, is available at http://
riskcalc.sts.org/stswebriskcalc. Although risk calculators
contain many data inputs, frailty and some other important
patient and procedural characteristics are not factored into
the calculations. Therefore, a comprehensive approach is
required for determining patient surgical risk and candidacy.
Frailty, which is variably defined as a geriatric syndrome of
decline in several physiologic systems and processes, por
tends an increased risk for mortality in patients undergoing
surgery and can be measured preoperatively (see MKSAP 19
General Internal Medicine 1).
For all patients in whom surgical or interuentional ther
apy is being considered. a multidisciplinary approach with a
heaft team consisting of a cardiologist, a surgeon, and an
interventional cardiologist is recommended. Evaluations in
centers with specialized expertise in VHD (e.g.. a Heart Valve
Center of Excellence) is also advised for patients in whom
intervention is being considered when there are no symptoms,
multipie or complex morbidities are present, or surgical valve
repair is lavored over valve replacement.
51

74. Valvular Heart Disease
Aortic stenosis
Aortic
regurgitation
Mitral stenosis
Mitral
regurgitation
Tricuspid
regurgitation
Tricuspid
stenosis
Pulmonary valve
stenosrs
Pulmonary valve
regurgitation
Midsystolic;
crescendo-
decrescendo
Diastolic;
decrescendo
Diastolic; low-
pitched,
decrescendo
Diastolic; low-
pitched,
decrescendo;
increased
intensity during
inspiration
Systolic;
crescendo-
decrescendo
Diastolic;
decrescendo
RUSB
LLSB (valvular)
or RLSB
(dilated aorta)
(best heard
sitting and
leaning
forward)
Apex (best
heard in left
lateral
decubitus
position)
Enlarged, nondisplaced
apical impulse; Sa; bicuspid
valve without calcification
will have systolic ejection
click followed by murmur
Enlarged, displaced apical
impulse; 53 or Sa; increased
pulse pressure; bounding
carotid and peripheral
pu lses
Loud 51; tapping apex beat;
opening snap after 52 if
leaflets mobile; irregular
pulse i{ atrial fibrillation
present
Systolic click in mitral valve
prolapse; 53; apical impulse
hyperdynamic and may be
displaced if dilated left
ventricle; in mitral valve
prolapse, Valsalva maneuver
moves onset of clicks and
murmur closer to S,;
handgrip maneuver
increases murmur intensity
Merged and prominent c
and v waves in jugular
venous pulse; murmur
increases during inspiration
Elevated central venous
pressure with prominent a
wave, signs of venous
congestion (hepatomegaly,
ascites, edema)
Pulmonic ejection click after
S1 (diminishes with
inspiration)
Loud P2 if pulmonary
hypertension present
Systolic; holo-, Apex
mid-, or late
systolic
Holosystolic LLSB
Right clavicle,
carotid; apex
None
None
Axilla or back;
occasionally
anteriorly to
precordium
LUSB
None
Left clavicle
Severe aortic stenosis
findings may include
decreased 42; high-pitched,
late-peaking murmur;
diminished and delayed
carotid upstroke; radiation
o{ murmur to both clavicles
and carotids
Radiation of murmur down
the descending thoracic
aorta may mimic mitral
regurgitation
Acute severe regurgitation
murmur may be masked by
tachycardia and short
duration of murmur
Severity in chronic
regurgitation is difficult to
assess by auscultation
lnterval between 52 and
opening snap is short in
severe m;tral stenosis
lntensity of murmur
correlates with transvalvular
gradient
P2 may be loud if pulmonary
hypertension present
Acute severe regurgitation
may have soft or no
holosystolic murmur, mitral
inflow rumble, or 53
Right ventricular impulse
below sternum
Pulsatile, enlarged liver with
possible ascites
Murmur may be high-
pitched if associated with
severe pulmonary
hypertension
Low-pitched frequency may
be difficult to auscultate,
especially at higher heart
rate
lncreased intensity of
murmur with late peaking
Murmur may be minimalor
absent if severe due to
minimaldifference in
pulmonary artery and right
ventricular diastolic pressures
,
LLSB
LUSB
LLSB
!
Cardiac
Condition
Characteristic Location
Murmur
Radiation Associated Findings Severity and Pitfalls
(Continued on the next page)
TABLE 21. Valvular and Other Cardiac Lesions and Their Associated Examination Findings
62
None

75. I
t
I
t
i
t
I
i
1
I
t
t
i
{
I
t
L
t
!
!
I
t
t
I
I
t
!
i
L
I
I
i
t
I
Valvular Heart Disease
Radiation Associated Findings Severity and PitfallsCardiac
Condition
Benign
(innocent)flow
murmur
Hypertroph ic
obstructive
rdiomyopathy
Characteristic Location
Murmur
Midsystolic;
grade 1/6 or
2/6 in intensity
Systolic;
crescendo
decrescendo
RUSB
LLSB
RUSB
None
None
None
None
Normal intensity of 42;
normal splitting of 52; no
radiation
Enlarged, hyperdynamic apical
impulse; bifid carotid impulse
with delay; increased intensity
during Valsalva maneuver or
with squatting to standing
Fixed split 52; right
ventricular heave; rarely,
tricuspid inflow murmur
Palpable thrill; murmur
increases with handgrip
maneuver
May be present in conditions
with increased flow (e.9.,
pregnancy, fever, anemia,
hyperthyroidism)
Murmur may not be present
in nonobstructive
hypertrophic
cardiomyopathy
May be associated with
pulmonary hypertension with
increased intensity of P2,
pulmonary valve regurgitation
Murmur intensity and
duration decrease as
pulmonary hypertension
develops (Eisenmenger
syndrome)
Cyanosis if Eisenmenger
syndrome develops
CA
Atrial septal
defect
Ventricular
septal defect
Systolic;
crescendo-
decrescendo
Holosystolic LLSB
TABLE 21. Valvular and Other Cardiac Lesions and TheirAssociated Examination Findings (Continued)
A? = aortic component
upper sternal border.
of52;LLSB=leftlowersternalborder;LUSB=leftuppersternalborder;P,=pulmoniccomponentofSz;RLSB=rightlowersternalborder;RUSB. right
\
!
:
t
I
I
L
a
I
A At risk
B Progressive
Patients with risk factors for
development of VHD
Patients with progressive VHD
(mild to moderate severity and
asymptomatic)
Asymptomatic patients who have
the criteria for severe VHD:
C1 : Asymptomatic patients
with severe VHD in whom the
left or right ventricle remains
compensated
C2: Asymptomatic patients
with severe VHD, with
decompensation of the left or
right ventricle
Patients who have developed
symptoms as a result of VHD
C
D
VHD = valvular heart disease.
Reproduced with permission from Oto CM, Nishimura RA, Bonow RO, et al; Writing
Committee Memb,ers. 2020 ACC/AHA guideline for the management oI patients
with valvular heart disease: a report of the American College of Cardiology/
American Heart Association Joint Committee on Clinical Practice Guidelines. J Am
Coll Cardiol. 202 1 ;77:e25 e1 97. IPMID: 33342586] doi:1 0.1 01 6ij.jacc.2020.1 1 .0 1 8.
02021 American College of Cardiology Foundation and the American Heart
Association, lnc.
f,EY POIXIS
o Many heart valve lesions progress slowly, causing patients
to limit their activity unconsciously in response; therefore,
a careful history and detailed physical examination are
essential'
(continued)
fEY POltIl (ooilnued)
r Medical therapy, although often effective for symptom
palliation, has not been shown to prevent disease pro
gression or improve long term survival in patients with
valvular heart disease.
o For all patients with valvular heart disease in whom
surgical or interventional therapy is being considered, a
multidisciplinary approach with a heart team consist-
ing of a cardiologist, a surgeon, and an interventional
cardiologist is recommended.
Aoftic Stenosis
Clinical Presentation and Evaluation
Aortic stenosis may be congenital, as in persons with a bicus
pid aortic valve, or acquired. The most common cause is
degeneration of'the valve that occurs with aging (Figure 3O);
severe lesions occur in approximiltely 3')(, of persons aged
65 years or older. Other acquired cluses include rheunlatic
disease and chest irradiation. Although rheumatic discase of'
the mitral valve frequently occurs in isolation, rheumatic aor
tic valve disease almost never occurs without mitral valve
involvement. Chest irradiation (e.g., mantle therapy Ibr non
Hodgkin lymphonra) commonly results in a combination of
stenosis and regurgitation.
Aortic stenosis causes chronic pressure overload of the
left ventricle (lV), leading to concentric LV hypertrophy and
myocardial interstitial fibrosis. Diastolic dysfunction fbtlows,
with eventual systolic heart failure ancl pulmonary congestion.
'fhe
disease typically progresses with a decrease in the aortic
Asymptomatic
severe
Symptomatic
severe
63

76. Aortic Stenosis
TABLE 23. Serial Evaluation of Asymptomatic Patients with Left-Sided Valvular Conditions
Valvular Heart Disease
Factors Considered
Stenosis severity; rate of progression; LV
systolic function; ascending aorta dilation
if associated with bicuspid aortic valve
Aortic Regurgitation
Regurgitation severity; rate of progression;
LV ejection fraction; LV chamber size;
ascending aorta dilation if bicuspid aortic
valve
Mitral Stenosis
Stenosis severity
Mitral Regurgitation
Regurgitation severity; rate of progression;
EF; LV chamber size
Lesion Severity
At risk (V.", <2 m/s)
Mild (V.", 2.0-2.9 mls or mean gradient
<20 mm Hg)
Moderate (V-.,3.0-3.9 m/s or mean
gradient 20-39 mm Hg)
Severe (V-u, >4 m/s or mean gradient
>40 mm Hg, AVA typically <1 .0 cm2)
Very severe (V-"* )5 m/s or mean gradient
>60 mm Hg)
Frequency of Evaluation
Clinical evaluation yearly; echo every 3-5 y
Clinical evaluation yearly; echo every 1-2 y
Clinical evaluation yearly; echo every
6-12 mo
Clinical evaluation yearly; echo every
6-12 mo
Mild (VC <0.3 cm, ERO <0.10 cm2, RV
<30 mUbeat, RF <30%); normal EF
Moderate (VC 0.3-0.6 cm, ERO 0.1 0-0.29 cm2,
RV 30-59 mUbeat, RF 30%-49"/")
Severe (VC >0.6 cm, ERO >0.3 cm2, RV
>60 mUbeat, RF >50%)
EF >55%; LVESD <50 mm
EF <55%; LVESD >50 mm
Mild and moderate (MVA>1.5 cm2,
diastolic pressure half-time <150 ms)
Severe (MVA l1 .5 cm2, diastolic pressure
half-time>1 50 msor>220 mswithvery
severe stenosis, PASP >50 mm Hg)
At risk (VC <0.3 cm)
Clinical evaluation yearly; echo every 3-5 y
Clinical evaluation yearly; echo every 1-2 y
Clinical evaluation every 6-12 mo; echo
every 6-12 mo, more frequently for dilating
LV
Clinical evaluation every 6-12 mo; echo
every 6-12 mo, more frequently for dilating
LV
Clinical evaluation yearly; echo every 3-5 y
Clinical evaluation yearly; echo every 1-2 y
for MVA 1 .0-1 .5 cm2, every year for MVA
<1 .0 cm2
Clinicalevaluation yearly; echo only if
symptomatic
Clinicalevaluation yearly; echo every 3-5 y
for mild severity, every 1-2y for moderate
severity
Clinical evaluation every 6-12 mo; echo
every 6-12 mo, more frequently for dilating
LV
t
I
Mild and moderate (VC <0.7 cm, ERO
<0.40 cm2, RV <60 mUbeat, RF <507o)
Severe (VC >0.7 cm, ERO >0.4 cm2,
RV>60 mUbeat, RF >50%)
Recommendations based on Otto CM, Nishimura RA, Bonow RO, et al; Writing Committee Members.2020 ACC/AHA guideline for the management of patients wrth valvular hean
disease: a report of the American College o{ Cardiology/American Heart Association Joint Committee on Cl inical Practice Guidelines. J Am Coll Cardtol.2O21:77:e25 e197 .
I PMI D: 33342586] d oi:1 O.1 01 64.jacc.2A20. 1 1 .0 1 8
valve area of approximately 0.12 cm2 per year, but the rate
depends on patient age, underlying severity of the stenosis,
and comorbid conditions, such as kidney failure and hyper
tension. Exertional dyspnea, syncope, and angina are the most
common symptoms; however, symptoms may not appear until
stenosis is severe. Among asymptomatic patients with severe
aortic stenosis, 75'7, will die or develop symptoms within
5 years. Once symptoms occur, Iife expectancy is generally
only 1 to 2 years. Thus, serial evaluation every 6 to 12 months
is recommended for patients with severe disease (see Table 23).
The characteristic clinical findings of severe aortic steno
sis include a late peaking systolic murmur, a diminished or
absent aortic component of the 52. and a delay in the carotid
upstroke (pulsus tardus) that may be accompanied by a
decreased pulse amplitude due to low cardiac output (pulsus
parvus). Clinical findings suggestive of severe aortic stenosis
should be promptly evaluated (see Table 21).
The primary imaging modality for evaluation of aorlic
stenosis is TTE (Figure 31). Echocardiography can determine
the cause of stenosis, stenosis severity (with gradient and valve
64

77. N
area assessment), LV function and wall thickness, right ven-
tricular (RV) function, pulmonary artery pressure, and the
presence or absence of other valve pathologr. In some patients,
echocardiography may underestimate the severity ol aortic
stenosis. Further evaluation with cardiac catheterization. dur
ing which cardiac output and the aortic pressure gradient can
be measured, is required when there are discrepancies between
the clinical and echocardiographic findings in symptomatic
patients being considered for intervention. Exercise stress test
ing is useful in asymptomatic patients with severe aortic steno
sis to confirm asymptomatic status, but it should be performed
under cardiologist supervision. Exercise stress testing is con
traindicated in symptomatic severe aortic stenosis.
Valvular Heart Disease
Severe aortic stenosis is typically defined by a small valve
area (<1.0 cm2), high peak velocity (>4 m/s), and/or high mean
gradient (>40 mm Hg). There are tvvo patient subsets in which
severe aortic stenosis may be present with a snrall valve area
and either low velocity or low gradient (1) patients with
severe LV dysfunction and low cardiac output (low flow low
gradient aortic stenosis) and (2) patients with preserved LV
lunction and paradoxical low floW low gradient aortic steno
sis. In the former group, dobutamine echocardiography or
dobutamine cardiac catheterization is needed to distinguish
true aortic stenosis fiom pseudostenosis. In pseudostenosis,
dobutamine increases cardiac output and the opening lorces
on the aortic valve, causing the valve area to increase out of the
'i'
!
tIGURE 30.Aorticstenosis.Grossspecimensshowingpathologyof degenerativeao(irstenosis(topleltpanel),bicuspidaorti(stenosis(toprightpanel),andrheumatic
overloadfromsevereaorticstenosislarrowheadinbottomrightpanel).Ao=ascendingaorta; lX=leftatrium; N=nonroronarycusp.
lmages c0ua(esy of Dr William Edwards, l\layo Cl nic
65

78. Valvular Heart Disease
t lG U R E 3 1 . Echocardiographic findings in aortic sten0sis. Calcific aortic
stenosis (arowhead) is present in parasternal long-axis (top panel) and short,axis
(middle panellviews. Leftventricular(LV) hypertrophy is also present. Doppler
echocardiogram shows a mean aortic gradient of 56 mm Hg, consistent with severe
aortic stenosis (bottom panel\. Ao = ascending aorta; l-A = left atrium.
severe range. With low-flow, low gradient aortic stenosis, the
calculated valve area remains in the severe range with dobu
tamine administration, and the aortic valve gradient and
velocity increase with increased stroke volume. Patients with
paradoxical low flow low-gradient aortic stenosis have low
stroke volume (<35 ml/m,) resulting from a combination of
small LV size and high aortic impedance to flow (e.g., hyper
tension) or other causes of low cardiac output (e.g., atrial
fibrillation, pulmonary hl,pertension). Determination of lesion
severity in paradoxical aortic stenosis requires consideration of
the hemodynamics, valve morpholory (e.g., degree of calcifica
tion), presence of LV hypertrophy, and clinical presentation of
the patient. In patients with suspected low flow low gradient
severe aortic stenosis with normal or reduced left ventricular
ejection fraction, measurement of aortic valve calcium score
by CT is reasonable to further define severity. In patients with
either low-flow low gradient severe aortic stenosis with
reduced LV function or paradoxical low-floW low-gradient
severe aortic stenosis, observational studies have shown
improved survival with aortic valve replacement compared
with medical therapy.
Management
Aortic valve replacement is a life-prolonging procedure in
patients with severe aortic stenosis. The indications for aortic
valve replacement in severe aortic stenosis are the presence of
symptoms (e.g., dyspnea, angina, presyncope, syncope, or
heart failure), LV systolic dysfunction (ejection fraction <50'/.)
in an asymptomatic patient, or a concomitant cardiac surgical
procedure for other indications (e.g., coronary artery bypass
grafting or ascending aorta surgery). Aortic valve replacement
is reasonable in asymptomatic patients with very severe aortic
stenosis and low surgical risk and asymptomatic patients with
abnormal results on supervised exercise testing, such as poor
exercise tolerance, abnormal ECG changes, or hypotension
during testing.
Aortic valve replacement can be performed with open car-
diac surgery (surgical aortic valve replacement [SAVR]) or via
transcatheter approach (transcatheter aortic valve implantation
[TAVI])
(Figure 32). SAVR and TAVI have similar procedural and
long term survival rates, with expected operative mortality
rates of1% to 3%. The choice between surgical and transcatheter
interventions is based on the presence of symptoms and the
patient's surgical risk, as determined through comprehensive
multidisciplinary assessment. TAVI is recommended orer SAVR
for symptomatic patients with severe aortic stenosis who are
older than B0 years or for younger patients with a life expec-
tancy less than 10 years. TAVI is also recommended over SAVR
for symptomatic patients of any age with severe aortic stenosis
and a high or prohibitive surgical risk if predicted postproce
dure survival is more than 12 months with an acceptable quality
of life. For symptomatic patients who are aged 65 to 80 years.
either SAVR or TAVI is appropriate following shared decision
making. Neither SAVR nor TAVI is indicated in patients with
limited expectation of survival due to comorbid conditions.
Although the pathophysiologr of aortic stenosis is known
to be inflammatory randomized trials have shown medical
therapy, specifically statins, to be ineffective in slowing disease
progression. For patients with coexistent hypertension or heart
failure, guideline directed medical therapy is recommended.
Vasodilators should be used with caution in patients with
66

79. Valvular Heaft Disease
F I G U R E 3 2. Transcatheter aortic valve implanlation.lop left panel: Balloon aortic
valvuloplasty (anowhead) is fi rst perform ed.Top right panel: Using a transfemoral
approach (anows), a transcatheter aortic value(arrowhead)is positioned at the aortic
annu lus usi ng aortog raphy. Bottom left panel:Ihe prosthesis (arowhead) is then
slowly inflated during rapid pacing from a temporary pacemaker (IP), which creates
ventricula r standstill. Bottom right panel: Ihe prosthesis is ful ly deployed.
aortic stenosis and heart failure symptoms. ln select cases, bal-
loon valvuloplasty may be used to bridge unstable patients to
therapywith TAVI or SAVR.
o The characteristic clinical findings of severe aortic ste
nosis include a late-peaking systolic murmur, a dimin-
ished or absent aortic component of the 52, and a weak
and delayed carotid upstroke.
o Echocardiography is accurate for defining the severity
of aortic stenosis in most patients; when there is a dis-
crepancy between the clinical and echocardiographic
findings, cardiac catheterization should be considered
in patients who are candidates for intervention.
. Patients with low-flow low gradient aortic stenosis have a
small valve area but low velocity and/or low gradient due
to low stroke volume; dobutamine echocardiography or
cardiac catheterization can be used to distinguish pseudo-
severe aortic stenosis from true severe aortic stenosis.
o Aortic valve replacement prolongs life in patients with
symptomatic severe aortic stenosis; the patient's surgi-
cal risk determines whether valve replacement is per-
formed using open surgery or a transcatheter approach.
. Transcatheter aortic valve implantation is indicated for
symptomatic patients with trileaflet aortic stenosis at
any level of surgical risk who do not have concomitant
severe aortic regurgitation.
Aortic Regurgitation
Clinical Presentation and Evaluation
Aortic regurgitation, manifesting acutely or chronically, arises
lrom aortic root pathologr or intrinsic valve disease. Causes of
chronic aortic regurgitation include ascending aortic dilata
tion and valve abnormalities due to bicuspid disease, calcific
degeneration, rheumatic involvement, or chest irradiation.
Acute aortic regurgitation may be caused by endocarditis,
blunt chest trauma, iatrogenic damage (e.g., complications of
balloon aortic valvuloplasty), or aortic dissection.
In chronic aortic regurgitation, volume overload causes
progressive LV dilatation and eccentric hypertrophy. Chronic
aortic regurgitation may be tolerated for many years but may
eventually lead to symptoms, including shortness of breath,
fatigue, or angina. Clinical findings result from the large
stroke volume and LV dilatation and include bounding
peripheral pulses, displacement of the LV apex, and a diastolic
decrescendo murmur heard either along the right sternal
border (suggesting root pathologr) or left sternal border (sug
gesting valve patholory) (see Table 21). The large forward
stroke volume also can cause an early peaking systolic ejec-
tion murmur.
In acute regurgitation, the abrupt onset of volume over-
load may cause acute heart failure or even cardiogenic shock.
Bounding pulses may not be present because stroke volume
has not markedly increased, and murmurs may be softer or
shorter in duration because of the rapid equalization of pres-
sures between the aorta and LV
TTE is indicated for evaluation ofaortic regurgitation and
LV function. In patients with moderate or severe aortic regur
gitation and suboptimal TTE images or a discrepancy between
clinical and TTE fi ndings, transesophageal echocardiography
(TEE), cardiac magnetic resonance (CMR) imaging, or cardiac
catheterization is indicated. When endocarditis is suspected,
TTE is the initial imaging test in most patients. TEE following
TTE is recommended for patients with suboptimal TTE results
or with high initial risk. CT angiography is indicated in patients
with acute AR and aortic dissection because it is highly accu-
rate and usually rapidly available.
Criteria for severe aortic regurgitation include a jet width
that occupies 657, or more of the LV outflow tract, vena con-
tracta (the width of the regurgitant jet at its most narrow por
tion) greater than 0.6 cm, holodiastolic flow in the descending
aorta, regurgitation volume of 60 mL or more, and effective
regurgitant orifice area of 0.3 cm2 or greater. The LV gpically
is dilated in chronic aortic regurgitation. In patients suspected
of having an aortic root abnormality, evaluation with CMR
imaging, CT, or TEE is recommended. Surveillance is based on
severity ofregurgitation and other factors (see Table 23).
Management
In chronic aortic regurgitation, surgery with traditional open
aortic valve replacement is advised for patients with symptoms
(typically, dyspnea or angina) or LV dysfunction (ejection
67

80. Valvular Heart Disease
fraction <55'/.) thought to be due to aortic regurgitation or for
patients with severe aortic regurgitation who are undergoing
other cardiac surgery. Surgical treatment of aortic regurgita-
tion is reasonable in asymptomatic patients with severe AR,
normal left ventricular function, and significant LV dilatation
(end systolic dimension >50 mm or indexed end-systolic
dimension >25 mm/m2). In patients with isolated severe aortic
regurgitation who have indications for SAVR and are candi-
dates for surgery TAVI should not be performed.
Medical therapy, preferably with dihydropyridine calcium
channel blockers, ACE inhibitors, or angiotensin receptor
blockers (ARBs), is indicated in patients with chronic aortic
regurgitation and concomitant hlpertension. Therapy with
ACE inhibitors or ARBs and p blockers is reasonable in severe
aortic regurgitation with symptoms or LV dysfunction when
surgery is not an option.
Significant acute aortic regurgitation due to aortic dissec-
tion is a surgical emergency, requiring aortic dissection repair
and aortic valve replacement or repair. For other acute causes,
the indications for surgery depend on the regurgitation sever
iry presence of symptoms, and hemodynamic stability of the
patient.
t(EY POlltTS
o Characteristic clinical findings of chronic aortic regurgi-
tation include bounding peripheral pulses, displace-
ment of the left ventricular apex, and a diastolic decre-
scendo murrnur heard along the right or left sternal
border.
o In chronic aortic regurgitation, surgery with traditional
open aortic valve replacement is advised for patients with
symptoms or left ventricular dysfunction, or who have
severe asymptomatic aortic regurgitation and are under-
going other cardiac surgery; in slmptomatic patients
who are not surgical candidates, medical therapy is
appropriate.
r Medical therapy with dihydropyridine calcium channel
blockers, ACE inhibitors, or angiotensin receptor block-
ers is recommended for patients with chronic aortic
regurgitation and concomitant hlpertension.
. Emergent surgery is indicated for patients with acute
aortic regurgitation due to aortic dissection.
Bicuspid Aortic Valve Disease
Bicuspid aortic valve disease affects approximately l% to 2'/. of
the general population. Bicuspid morphologr leads to abnor-
mal shear forces and predisposes to early degeneration ofthe
valve, resulting in stenosis in most patients (up to 75%) (see
Figure 30) and pure regurgitation in a small minority of
patients (2%-7O'X,). Patients with a bicuspid aortic valve typi
cally present with an asymptomatic finding of a systolic
ejection murmur in adolescence or young adulthood and
gradually progress to severe disease in the fifth or sixth decade
of life. More than one third of those older than 70 years with
severe aortic stenosis have an underlying bicuspid valve.
Bicuspid valvulopathy is often accompanied by aortic
abnormalities, independent of the severity of aortic stenosis or
regurgitation, and may be associated with aneurysm, dissec
tion, or coarctation. Therefore, in patients '*,ith a bicuspid
aortic valve, the ascending aorta and aortic arch should be
examined for aortopathy with TTE. CMR angiography or CT
angiography is indicated when echocardiographic assessment
is suboptimal. Lifelong serial imaging is indicated if abnor-
malities are detected. The imaging modality and frequency
depend on several factors, including the nature (stenosis.
regurgitation, or aneurysm) and severity of the abnormality.
age of the patient, family history and candidacy for surgery.
Importantly, bicuspid aortic valve is a heritable abnormaliry
and first degree relatives of patients with a bicuspid aortic
valve and aortopathy may be considered for screening with
echocardiography.
Management of bicuspid aortic valve disease is deter-
mined by the predominant lesion Upe
(stenosis or regurgita
tion) and its severity. In patients with a bicuspid valve
undergoing surgery for severe aortic stenosis or regurgitation,
surgical repair ofthe ascending aorta is reasonable'nthen the
aortic dimension is 4.5 cm or greater. In the absence of surgical
indications for a stenotic or regurgitant aortic valve, surgical
repair of the ascending aorta or aortic sinuses is recommended
when the aortic dimension is greater than 5.5 cm and may be
reasonable when the dimension is greater than 5.0 cm with an
additional risk factor for dissection (e.g., family history rate of
progression >0.5 cm/year).
No medical therapies slow aortic dilatation in patients
with aortopathy and a bicuspid aortic valve. Blood pres
sure should be controlled in patients with concomitant
hypertension.
TEY POITIIS
r Bicuspid morphologr predisposes to early degeneration
of the aortic valve, resulting in stenosis in most patients
and pure regurgitation in few patients.
. Patients with a bicuspid aortic valve typically present
with an incidental systolic ejection murrnur in adoles
cence or young adulthood and gradually progress to
severe disease in the fifth or sixth decade of life.
. Management of bicuspid aortic valve disease follows the
recommendations for the predominant valve lesion tlpe
(aortic stenosis or regurgitation) and its severity.
Mitral Stenosis
Clinical Presentation and Evaluation
The leading cause of mitral stenosis is rheumatic heart disease,
which is more common in women than in men (4:1 ratio).
Although relatively rare in the United States, rheumatic heart
disease is frequent in populations with limited access to
!
68

81. I
t
trerturcnt fbr streptococcll pharyngitis. llheur.natic heirrt clis
eirsc results in fusion ol thc rlitral cor.r.rr.r.rissLlres and. in rlrore
ad'tirnced firn-r-rs. calciflcrrtion olthe Virl'u'c irncl abnorrnllities in
the subvirlvular appirrirtus (Figure 33). Othe.r causes ol r.nitral
stenosis arc parachutc rlitral valve, chest irradiation. irr-rd
severe nritrll annular calcitlcation. Nlitral itr-rr-rular calci licirtion
is rttore conrmon ir-r thc clderll'and is ilssociiited u,itl-r inf'lrrnt
mirtoll clisorders. peripheral artery ctiseasc. and chronic kicl
ney discase.
'l'he
natural history of mitral stenosis is chirracterizcd lty
slou' progression over clecades. r,r,ith grrrclual left rtrirl (l.A)
enllrgenrent and lrresen'ation of L\,'tirnction. Sl,mptonrs llav
ari se lionr lor'r' carcl iac or-r tput
( fatiguc). pu l ntonan, con gest ior-r
(dyspnea). and pulnronary hypertension r,r,ith right sicled
heart trrilure (lower extremity edema). I)regnancy, lvith the
resultirrg increased bkrocl volume irncl t'lrclilrc output, r'nirv alscr
precipitltc S)'mptoms. 51'n-rptoms rrrc ty'pical11, exerti0nal
becrrnse erercise shortens diirstolic lilling tin-re ancl increirses
the transval.n,ular 1lolr, and cliastolic ntitral gradient, leirding to
u,orsening of LA hypertension. P:rtients irlso can present with
Valvular Heart Disease
s),stcnric embolizirtion. atrial fibrillrtion. or. in sevcre cases.
hen'rolltvsis. He:irt tirilure is the clusc of death it-t .tpproxi
nratelt'60'li, of prticnts r,t'ith n-ritrirl stenosis. u ith thromboen-t
bolisnr causing nlost others.
(llir.rical finclings r,r'hen the vrtlr,c is pliable include r tap
ping Ili impulse. a loucl S,. an increrrsecl lrulmonic conrponent
ot'S,. ii cliastolic opcning snap. irr.rcl l diastolic rumble or lou,
pitche d llrurnrur.lt tl.re apex (see'lirblc 2l). Signs o{ pulmonary
or systemic congestior-r n-ray be present depending rin lesion
sevcrity ancl the puticnt's volume stirlus.
I'l'lr is highll'ac(urilte fbr irssessing disease severit],,, pul
nronrrl pressures. rrnd RV fur-rction ls u'ell as for iclcntiff ing
cor.rconritilr-rt v:rh,'ular lcsions (scc l'irblc 2tl). Additionirl imag
ir-rg stuclies or cirrclilc catheteriz-ation is rarely recluired for
diirgnosis. Severe mitrirl stenosis is clellned by a nritral valve
iirer o1 1.5 cmr or lcss. u'hich usturlll,corresponcls to a ntean
n-ritrrrl gradierlt of'nrore than 5 to l0 nrnr Ilg at a nurmll heart
rilte. In patients 'nvith ii discrep:rncl betu,een the clinical
lncl ccl.rocardi ogrlp h ic li ndings. exercise ecl.rocardiography
or exercise testir.rg cluring cardiac cirtheterization should be
v
I
FIGURE 33.Echocardiogramsshowinganormal mitralvalve(MV) (toprightpanel)andrheurnatrcmrtral stenosiswith(ommissura lusian(arrowheads,topleftpanel).ln
mitral gradient of 1 3 mm Hg, r0nsistent with severe stenosis. Ao - ascendinq aorta; LA= left atrium; LV= left ventricle.
69

82. Valvular Heart Disease
pursued to assess the response of the mitral gradient and pul
monary pressures.
Management
Percutaneous balloon mitral commissurotomy (PBMC) is the
procedure of choice for patients with significant rheumatic
mitral stenosis when the valve is pliable and not severely calci
fied. PBMC is indicated for symptomatic patients with severe
rheumatic mitral stenosis and favorable valve morphologr and
is reasonable in asymptomatic patients with severe rheumatic
mitral stenosis and a pulmonary artery systolic pressure
greater than 50 mm Hg. PBMC should not be performed in
patients with LA thrombus or moderate or severe mitral regur
gitation, both of which are optimally evaluated with TEE. In
appropriately selected patients, the success rate with PBMC is
95'/,, and complications occur in fewer than 5'1, of patients.
Mitral valve surgery is indicated in patients with severe mitral
stenosis, New York Heart Association functional class III or IV
symptoms, and a nonpliable valve and in asymptomatic
patients with severe mitral stenosis undergoing concomitant
cardiac surgery for another indication.
Nearly 50'2, of patients with mitral stenosis have atrial
fibrillation, and without anticoagulation, these patients have a
risk for thromboembolism of 20'7, to 25')(,. Patients with mod
erate to severe mitral stenosis and concomitant atrial fibrilla
tion should receive a vitamin K antagonist such as warfarin,
with a goal INR of 2.0 to 3.0. Anticoagulation is also indicated
in patients with a history of LA thrombus or systemic emboli
zation. Notably, clinical trials of direct acting oral anticoagu
lants in atrial fibrillation excluded patients with moderate to
severe mitral stenosis; therefore. the efficacy and salety of
these agents have not been demonstrated in this population.
Because the mitral gradient is heavily dependent on
transvalvular flow medical therapy with negative chrono
tropic agents, diuretics, and long-acting nitrates can be effec
tive for symptom palliation in patients who are not candidates
for interventional or surgical therapy.
X EY PO I TITS
. In patients with mitral stenosis, transthoracic echocar
diography is highly accurate for assessing disease sever
ity, pulmonary pressures, and right ventricular function
as well as identiSring concomitant valvular lesions.
. Percutaneous balloon mitral commissurotomy is indi-
cated for patients with symptomatic severe mitral ste
nosis and favorable valve morphologr.
o Anticoagulation is recommended for patients with
mitral stenosis and a history of atrial fibrillation, left
atrial thrombus, or systemic embolization.
Mitral Regurgitation
Clinical Presentation and Evaluation
Mitral regurgitation may arise from dysfunction of any portion
of the complex valve apparatus (leaflets, annulus, chordae.
papillary muscles, or LV free walls) and may present acutell'or
chronically. Causes of acute mitral regurgitation include
infective endocarditis, papillary muscle ischemia or rupture.
trauma
(e. g., injury from PB MC or blunt force). or degenerative
disease with chordal rupture and f'lail leaflet. Chronic mitral
regurgitation is classified as primary. involving any portion of
the mitral annulus. or secondary. invoh,ing causes other than
the annulus (e.g., ventricular dysflunction). Common causes of
primary mitral regurgitation are mitral valve prolapse (also
know,n as myxomatous or degenerative mitral valve disease),
radiation therap)'. rheumatic disease. and cleft mitral valve.
Mitral regurgitation results in volume overload with LV
dilatation and LA hypertension. which may progress and
cause pulmonary hypertension and RV failure. In acute mitral
regurgitation, hearl failure symptoms often occur abruptly
because of insufficient time fbr adaptive chamber dilatation
and, in some patients. can result in cardiogenic shock. The
systolic murrnur in acute mitral regurgitation may be brief
because of the rapid equalization of l.A and LV pressures. and
echocardiography with color flow imaging can underestimate
the severity of the regurgitation. Thus, when acute mitral
regurgitation is suspected, comprehensive assessment to iden
tify the potential causes should be pursued. and additional
imaging with TEE should be considered.
Chronic primary mitral regurgitation is predominantly
caused by mitral valve prolapse. which affects approxi
mately 27, of the general population in the United States.
Echocardiography in patients with chronic primary mitral
regurgitation may show a range of abnormalities, including
prolapse (Figure 34), gross degeneration ofone or both leaf:
Iets (Barlow syndrome). or chordal rupture' ,ith flail leaflet.
Barlow syndrome is more common in young adult patients. In
patients who are relatively older. fibroelastic deficiency pre
dominates and frequently results in chordal rupture. In
patients with chronic secondary mitral regurgitation, ven-
tricular dysfunction causes mitral regurgitation through pap
illary muscle displacement and tethering of the mitral leaflets.
which impairs coaptation. The mitral valve apparatus is nor
mal in patients with chronic secondary mitral regurgitation
(Figure 35).
The physical examination in patients with chronic mitral
regurgitation is notable for a blowing holosystolic murmur at
the apex. In patients with mitral valve prolapse. one or more
systolic clicks may precede the murmur. and variation in
severity, preload, and afterload can lead to differences in
murmur onset (holosystolic, midsystolic. or late systolic).
In patients with LV dilatation, the apical impulse may be
displaced laterally, and an S,, may be audible. especially in
patients with secondarl, n.tt,tr, regurgitation due to LV
dysfunction.
TTE readily and accurately assesses the selerity of pri-
mary mitral regurgitation. Severe primary mitral regurgitation
is defined by several parameters, the most common of which
are an effective regurgitant orifice area of 0.4 cm2 or larger,
regurgitant volume of 60 mL or more, and vena contracta of
x
It
70

83. Valvular Heart Disease
seen\arrowheads), leading to severe regurgitati0n seen on color flow imaging (arrow, bolton right panel) Ao = ascending aorta; LA- left atrium; LV = left ventricle.
0.7 cm or lar[e r. WI]en T1'I pnrvides insr-rtlicient or rliscordant
infirrr.nation,'l'lrl.l or CMIt inrrging is inclicated.
r\pproprirrtc fbllou, u1l of asyn'rlttontiltic piltirltts u,ith
nlitrirl regurgitiltion is outIinerl ir-r'liible 2:1.
Management
l\4edicll therrrpl, rlnd surgical illten'elttion can be lile saving
in l)rltients u ith ircute se\e rc lritrul regurgitirtion. \'rrsoclilator
thcrapy lr,ith I titratiible clrug, such xs nitnrprusside. decreases
iiortic it-npedlncc and mitral regurgitation, therebf irlproving
ftrnrirrcl carcliirc olltput. An intra aortic birlkror-r punrp can be
ttsecl to decreirse irlterloacl irnrl irugr-nent slster-r-ric irr.rrl crrror-rar1
pcrlt-tsion pressLlres. Pronrllt srtrgical correction sl'uukl be con
sirle rerl Ibr iill prrticnts with acutc sevcrc nritral regurgitltion.
[)irtients '"vith chronic scr,e re prinrrrrl' nritral regurgitrition
gencrirlll do poorlv uitholrt surger)l prrrticularll'u'hor there
iire sigt-tiliclnt synlptonrs. llail lerrllct. or LV clilatation. lrr onc
study ol
.15t3 patier-rts r,r,ith iisynrptonrxtic sevcrr primary nritral
regulgitiltion. the 5 y'crrr sun,ivirl rrte \'as on11, 58')i,. Surgical
rep:rir of thc r-r-ritral 'urrlve is inclicrrtccl fbr chronic severr pri
mary mi trll regllrgitrltiolr in syurptonrirtic prrt ients (regr rcl less
ol LV systolic function) and :rsytuptotnatic prltients with lV
dysfttnction (ejection liitction ((;0'1,, and or IV end s1'stolic
clir-nension > 10 mnr). Surgical re prrir is relsonirble in ttsl r-npto
matic paticllts witl.r prcservecl IV function r,r,lren the expectccl
repair succcss ratc is grclrter tl-ran 95',/, and thc operative risk is
less tl'rirn l't,. Surgicrrl rcpair is prrlerred ovcr repltrcerlenl in
irll patie nts. ilnd piiticnts sl-roukl lre re ferrecl to ir surgicril ce ntcr
lvitl-r expcrtise to irrlrrove thc chances o{ re1;lir. Meilicrrl
therapy with vasoclilators is not bcnelicial in patients r,r,,ith
primary nritrirl regurgitrrtion in thc irl-rsence ol slnrptolt-ts or I11
tlvstunction.
i!,1
4r./1c-:
LV
rp;-.ril-
ADULT ECHO
.;;
Ao
I
\LA
TE?,l,fiIiE
HR: f,4bpm
qlfiz
'l40mm
NTHI Generoi
Pwr:0dB l"!l:1 9
c,lds s1/ ltt/ 4
fialn: 1dE a:3
FR 14Hz
r 135,';
1.1'!
rz
",Ao
LV
:ir - g;
5i
20
52";
t
re.
,,
-*
e
';I
"ai:
I
-47 i
1i"
i9r
T
-
- 10
.,9,
97 bpm
71

84. Valvular Heart Disease
FIGURE 35. Echocardiogramsdemonstratingsecondarymitral regurgitationinapatientwithpreviousinferiormyocardial infarction.Tetheringof theposteriorleaflet
aorta; LA = left atrium; LV = left ventricle.
For patients who are not surgical candidates, transcatheter
mitral valve repair with a clip device (transcatheter edge-to-
edge repair [TEER]) improves coaptation of the mitral valve
leaflets, leading to increased valve closure and a reduction in
regurgitation (Figure 36). In selected patients with primary
mitral regurgitation, success rates with TEER are approximately
90%, with a procedural mortality rate of approximately 2'2,.
In patients with chronic secondary mitral regurgitation,
the primary goal of therapy is to address the underlying ven
tricular dysfunction with guideline directed medical therapy
and, ifindicated, cardiac resynchronization therapy (see Heart
Failure). Guideline directed medical therapy for ventricular
dysfunction includes an ACE inhibitor, ARB, or angiotensin
receptor neprilysin inhibitor; a p blocker; a diuretic: andior
an aldosterone antagonist. Benefits ofvalve repair or replace
ment in patients with secondary mitral regurgitation are less
certain, although studies have demonstrated favorable LV
remodeling after surgery. Surgery fbr secondary severe mitral
regurgitation is reasonable fbr those undergoing coronary
artery bypass grafting, but mitral regurgitation may recur after
repair because of primary LV dysfunction. TEER has been
approved by the FDA for patients with chronic secondary
mitral regurgitation and refractory symptoms despite optimal
medical therapy for heart failure; however, the effect of TEER
on mortality and heart failure hospitalization in this popula
tion has varied in studies.
I(EY POITIS
. Patients with acute mitral regurgitation may present
with acute heart failure; these patients may be difficult
to diagnose clinically or with echocardiography.
(Continued)
l( EY PO I t{TS (cotttinued)
. Surgery for chronic severe primary mitral regurgitation
is indicated in the presence of symptoms. left ventricular
dilatation. or decreasing ejection fraction.
. Surgical repair is preferred over replacement in patients
with chronic primary mitral regurgitation; patients should
be referred to a surgical center with expertise to improve
the chances of repair.
o Transcatheter mitral valve repair with implantation of a
clip device may be considered for patients with chronic
severe primary mitral regurgitation who are at high sur-
gical risk.
o Patients with chronic secondary mitral regurgitation
should be treated with guideline directed medical ther
apy for ventricular dysfunction: surgical intervention
may be considered for those undergoing concomitant
cardiac surgery.
Tricuspid Valve Disease
Tricuspid regurgitation. the most common form of tricuspid
valve disease, is frequently functional (or secondary) and clini
cally asymptomatic. Causes of tricuspid regurgitation include
cor pulmonale (or pulmonary hypertension) rvith RV failure.
pacemaker or defibrillator lead placement. trauma. congenital
abnormalities. and infective endocarditis. \[r]ren st'mptomatic.
patients may present with fatigue from [on'cardiac output as
well as signs and symptoms of right sided failure. such as
elevated jugular venous pulse (a large c u wave), a palpable RV
lift, hepatic congestion with pulsatile liver, and peripheral
edema. The murmur ol tricuspid regurgitation is typically a
72

85. Valvular Heart Disease
FIGURE 36.Iranscathetermitralvalveclippingforanteriormitral valveflail.,4,
Atrial view of anterior mitral valve flail segment (arow). B, Ventricular view of
anterior mitral valve f lail segment (arow). ( Fusion imaging (three-dimensional
transesophageal echocardiography, two-dimensional transesophageal
echocardiography, and fluoroscopy) of transcatheter mitral clip (arow) deployment
t0 anterior mitral valve flail segment. D, Postprocedure atrial view after placement
of three transcatheter clip devices (white arrows) with residual double orifice (red
arows) mitral valve. t, Postprocedure ventricular view after placement of three
tra nscatheter cl ip deuices (white arrows) with resid ual dou ble orifice (red arows)
mitral valve. AoV = aortic valve; LA = left atrium; LV = left ventricle.
holosystolic murmur heard along the left sternal border that
increases during inspiration due to increased venous return.
Tricuspid regurgitation should be evaluated with TTE,
which also allows assessment of RV function and estimation of
pulmonary pressures. In patients with tricuspid regurgitation
due to pacemaker or defibrillator lead placement, TEE may be
required to determine the etiologr more clearly.
Medical therapy with loop diuretics and aldosterone
antagonists alleviates symptoms of right-sided congestion;
risks include low-flow state with impaired cardiac output.
Surgery is recommended for patients with severe tricuspid
regurgitation who are undergoing left-sided valve surgery.
Surgery may be considered in patients with severe sympto
matic tricuspid regurgitation refractory to medical therapy.
Tricuspid stenosis is almost always caused by rheumatic
disease. Other causes include radiation therapy, carcinoid
syndrome, and medications (e.g., the ergot agent cabergo-
line). Symptoms of tricuspid stenosis (fatigue, cold skin) are
typically overshadowed by those caused by the left sided
abnormalities of coexistent rheumatic mitral disease. Clinical
findings include signs of right-sided congestion (elevated
jugular venous pulse, hepatic congestion, peripheral edema)
and a diastolic rumble. Surgery for tricuspid stenosis is
typically performed in concert with therapy for rheumatic
mitral disease.
TTY POIXIS
o Loop diuretics and aldosterone antagonists alleviate
symptoms of right-sided congestion in patients with
significant tricuspid regurgitation.
r Tricuspid valve surgery is recommended for patients with
severe tricuspid regurgitation undergoing left-sided lalve
$ugery and may be considered in patients with severe
symptomatic tricuspid regurgitation refractory to medical
therapy.
Prosthetic Valves
The choice ofprosthesis in a patient undergoing surgical valve
replacement is multifactorial, considering the patient's age,
the expected durability ofthe prosthesis, the surgical risk for
reoperation in the event of degeneration, and the ability and
willingness of the patient to take warfarin for anticoagulation.
For patients of any age, a bioprosthetic valve is recommended
for patients who cannot or do not desire to take anticoagula
tion therapy. According to the American College of Cardiologz/
American Heart Association. an aortic mechanical valve is
reasonable in patients younger than 50 years, a bioprosthesis
is reasonable in patients older than 65 years, and either a bio-
prosthesis or mechanical aortic valve prosthesis is reasonable
in those aged 50 to 65 years. In patients older than age
65 years, a mitral valve bioprosthesis is reasonable, as is
mechanical valve prosthesis in patients younger than 65 years.
However, the final decision on valve type should be reached
through a shared decision-making process between the physi-
cian and patient.
The expected durability ofbioprostheses is 15 years, and
in persons younger than 60 years, approximately 40'X, of valves
have evidence of clinically severe deterioration by 15 years.
Data on long-term durability ofTAVI prostheses are currently
limited to a follow-up of 8 to 10 years; however, thus flar, valve
durability is similar to surgical prostheses.
* r-l
C
I
I
\
t.
,1
AoV ,
r' <--
\:
LA
{ +--
-*r
LV
73

86. Valvular Heart Disease
Echocardiography should be performed after implanta
tion to document the baseline hemodynamic performance of
the valve, and repeat evaluations should be performed for
signs or symptoms of prosthetic dysfunction (new dyspnea or
heart failure. louder murmur or new murmur. thromboembo
lism, hemolytic anemia). ln patients with a surgical biopros
thetic valve, TTE at 5 years and 10 years and then annually
after implantation is reasonable, even in the absence of a
change in clinical status. For patients with a transcatheter
bioprosthetic valve, annual TTE is reasonable, even in the
absence of a change in clinical status.
Lif'elong warfarin anticoagulation is indicated fbr all
patients with a mechanical valve. Therapy should target a spe'
cific INR value rather than a range. In patients with a mechan-
ical aortic valve prosthesis (bileaflet or current generation
single-tilting disc) with no additional risk lactors fbr thrombo
embolism (history of embolization. hypercoagulable disorder.
LV dysfunction, atrial fibrillation), the goal INR for warfarin
anticoagulation is 2.5. ln patients with a mechanical aortic
valve prosthesis with heightened risk factors for thromboem
bolism, an older generation aortic valve prosthesis (ball in
cage), or any mitral prosthesis, the target tNR is 3.0. An INR
range of 2.5 to 3.5 is acceptable to balance the risks of under
and over anticoagulation in a patient with a mechanical mitral
valve. Because of the risk fbr valve thrombosis, direct acting
oral anticoagulants should not be used for anticoagulation in
patients with a mechanical valve. Oral anticoagulation with
warfarin should be considered for 3 to 6 months after implan
tation ola mitral or aortic bioprosthesis. An INR of 2.5 should
be targeted in these patients.
Adding aspirin (75 100 mg/d) to warfarin in patients
with a mechanical prosthesis decreases the risk for stroke
and death and may be considered in patients who have an
indication for antiplatelet therapy when the bleeding risk is
low. Low dose aspirin is reasonable for all patients with a
bioprosthesis
rtY POIXTT
o A shared decision-making process should guide the
choice of prosthetic aortic valve type, especially in
patients aged 50 to 65 years, in whom either a mechani
cal valve or bioprosthesis is a reasonable choice.
o Echocardiography is recommended fbr all patients
with a surgical bioprosthesis at baseline, 5 and 10 years
after surgery and then annually thereafter and with
any change in clinical status suggesting prosthetic
dysfunction.
. Echocardiography is recommended for all patients with
a transcatheter bioprosthesis at baseline and annually
thereafter and with any change in clinical status sug-
gesting prosthetic valve dysfunction.
. Lifelong warfarin anticoagulation is recommended in all
patients with a mechanical valve prosthesis; low dose
aspirin is reasonable for all patients with a bioprosthesis.
I nfective Endocarditis
Diagnosis and Management
Infective endocarditis (lE) is a life threatening bacterial or fun
gal inf'ection ol the endocardium involving native valvular
structures or implanted cardiac devices. Such devices include
cardiac valve prostheses, perrnanent pacemakers. implanted
cardioverter defibrillators. and occluders for repair ofcongeni
tal lesions (e.g.. atrial or ventricular septal defect occluders).
Risk factors for IE include advanced age, diabetes mellitus,
immunosuppression. injection drug use. indwelling intrave-
nous catheters. congenital heart disease. cardiac transplanta-
tion with valvulopathy, and an implanted cardiac device. lE
carries significant risk fbr morbidity and mortality. with high
rates of in hospital mortality (zo7.), t year mortality (40'x').
peripheral embolization (23'7,), stroke (17'x,). and need for car
diac surgery (a8%). Early diagnosis. targeted antimicrobial
therapy. and recognition ofthe need for early surgical interven
tion enhance patient survival.
Diagnosis of definite IE requires pathologic confirmation of
microorganisms demonstrated by culture or histologic examina
tion of a vegetation, a vegetation that has embolized. or an intra-
cardiac abscess specimen. Much more commonly. the diagnosis
is made using the modified Duke criteria (Table 24). The diag-
nostic criteria for definite lE include either two major criteria,
one major criterion plus three minor criteria, or five minor
criteria. Possible IE requires one major criterion and one minor
criterion. or three minor criteria. IE is excluded when there is a
firm alternative diagnosis, resolution of IE syndrome with anti-
biotic therapy for 4 days or less, or no pathologic evidence of
IE at surgery or at autopsy with antibiotic therapy for 4 days
or less.
Blood cultures are positive in 90'7, of IE cases. Most cases
of culture negative endocarditis occur in patients who have
received recent pre culture antibiotics. Serologic testing may
be required to identi[z fastidious organisms that are dilficult to
culture. ln patients with a prosthetic valve, IE syndrome is
classified according to the time from surgery as early (within
60 days), intermediate (60 365 days), and late (>365 days).
Early prosthetic valve endocarditis (PVE) is characterized
by infection with hospital acquired microbes. such as
Stophyloc'occus oureus. Coagulase negative staphylococci are
the most common microbes in intermediate PVE. S. oureus and
coagulase negative staphylococci are important causes of late
PVE; however, the microbes in late PVE more typically resem
ble those of'native valve endocarditis.
TTE is indicated to identify vegetations and associated
hemodynamic derangements. TEE is indicated with interme
diate or high suspicion lbr lE when TTE is not diagnostic.
especially in the setting of a prosthetic valve, intracardiac
device, or myocardial abscess, which in turn is suggested by
new conduction abnormalities on ECG or persistent bactere
mia despite antibiotic therapy. In patients with IE who have a
change in clinical signs or symptoms, TTE and/or TEE are
recommcnded fbr reevaluation.
74

87. Major Criteria
Blood culture positive for infeaive endocarditis
Typical microorganisms consistent with infective endocarditis from two separate blood cultures: Viridans streptococci, Streptococcus
bovis, HACEK gToup (Haemophllus spp., Actin obacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella spp., and
Kngella kingae), Staphylococcus aureus; or community-acquired enterococci, in the absence o{ a primary focus; or
Microorganisms consistent with infective endocarditis from persistently positive blood cultures, defined as follows:
At least two positive cultures of blood samples drawn 1 2 h apart; or
All ofthreeoramajorityof atleastfourseparateculturesof blood(withfirstandlastsamplesdrawnatleastl hapart)
Single blood culture positive flor Coxiella burnetii or antiphase I lgG antibody titer >1 :800
Evidence of endocardial involvement
Echocardiogram positive for infective endocarditis (TEE recommended in patients with prosthetic valves, rated at least "possible
infective endocarditis" by clinical criteria, or complicated infective endocarditis Iparavalvular abscess]; TTE as firsttest in other
patients), defined as follows:
Oscillating intracardiac mass on valve or supporting structures, in the path of regurgitant jets, or on implanted material in the
absence of an alternative anatomic explanation; or
Abscess; or
New partial dehiscence of prosthetic valve
New valvular regurgitation (worsening or changing of pre-existing murmur not sufficient)
Minor Criteria
Predisposition, predisposing heart condition, or injection drug use
Fever (temperature >38'C [1 00.4'F])
Vascular phenomena: major arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival
hemorrhages, and Janeway lesions
lmmunologic phenomena: glomerulonephritis, Osler nodes, Roth spots, and rheumatoid factor
Microbiologic evidence: positive blood culture but does not meet a major criterion as noted above" or serologic evidence of active
infection with organism consistent with infective endocarditis
TABLE 24. Major and Minor Criteria Used in the Modified Duke Criteria
Valvular Heart Disease
TEE = transesophageal echocardiography; TTE = transthoracic echocardiography.
uExcludes
single culture positive {or coagulase negative staphylococci and organisms that do not cause endocarditis.
Reproduced with permission from Li JS, Sexton DJ, Mick N, et al. Proposed modifications to the Duke criteria for the diagnosis of infective endocarditis. Clin lnfect Dis.
2000;30:6338.lPMlD:1O770721ldoi:10.1086/313753@2000OxlordUniversityPress.
Appropriate antimicrobial therapy should be promptly
initiated in high risk patients, such as those with severe sep-
sis, once cultures have been obtained. Empiric treatment
should consider patient risk factors, such as intravenous drug
use or the presence of an indwelling intravenous catheter, and
focus on the most common causes of acute endocarditis
Stophylococcus (methicillin susceptible and methicillin
resistant), Streptococcus, and Enterococcus species. Regimens
for empiric antibiotic therapy in critically ill patients with
acute endocarditis are provided in Table 25. Antimicrobials
should not be administered to patients with VHD and unex
plained fever before several blood cultures are drawn. Empiric
antibiotic combinations can be more focused when microbio
logic results are available. Infectious disease consultants
should advise on the duration oftherapy.
The decision to pursue surgery for treatment of IE and the
timing of such surgery is complex and requires a multidiscipli
nary approach. Early surgery (during hospitalization and
before completion of an antimicrobial course) is recom
mended for patients with any of the following:
o Severe valvular dysfunction resulting in symptomatic
heart failure
o Leflt-sided IE caused by S. oureus, fungal infections, or
other highly resistant organisms
o Associated complications, such as annular or aortic ab-
scess, destructive penetrating lesions, or heart block
o Persistent bacteremia or fevers lasting longer than 5 to
7 days after onset of appropriate antimicrobial therapy
Early surgery is reasonable in patients with recurrent emboli
and persistent valve vegetations despite 5 to 7 days of antibiotic
therapy, and early surgery may be considered in the presence ofa
large (>10-mm) left-sided vegetation to prevent embolic events.
When IE is associated with a pacemaker or deflbrillator, the
entire system (generator and leads) must be removed.
Prophylaxis
Endocarditis prophylaxis is recommended for a specific group
of patients before dental procedures that involve manipulation
of gingival tissue or the periapical region of the teeth or
75

88. Valvular Heart Disease
TABLE 25. EmpiricAntibioticTherapyforCritically lll
Patients with Acute Endocarditis
TABLE 26. Prophylactic lnfective Endocarditis Regimens
for Adults at Highest Risk of an Adverse Outcome Before a
Dental Procedure
)
Condition European Society
of Cardiology
(201s)
American
Heart
Association
(2OOs)
Community-
acquired native
valve endocarditis
or late prosthetic
valve endocarditis
(>12 months after
surgery)
Community-
acquired native
valve endocarditis
or late prosthetic
valve endocarditis
(>12 rnonths after
surgery), penicillin
allergic patient
Early prosthetic
valve endocarditis
(<12 months after
surgery) or health
care-associated
endocarditis
with
(Flu)cloxacillin or
oxacillin
with
Gentamicin
Vancomycin
with
Gentamicin
Vancomycin
with
Gentamicin
with
Rifampinu
with
Cefepime or
ceftriaxone (for
prosthetic valve
endocarditis)
No specific
regimen
provided
Vancomycin
with
Gentamicin
with
Rifampinu
with
Cefepime
Situation
Oral
Unable to take oral
medication
Allergic to
penicillin or
ampicillin -
oral
Agent
Amoxicillin
Ampicillin
or
Cefazolin or
ceftriaxone
Cephalexinb,'
or
Clindamycin
or
Azithromycin or
clarithromycin
Cefazolin or
ceftriaxone
or
Clindamycin
Dosage"
2g
2glMorlV
lglMorlV
29
600 mg
500 mg
lglMorlV
Ampicillin V"n.ornvon
-l
i
asome
experts recommend delaying the start of rifampin for 3 5 days in an
attempt to prevent the emergence of resistance to rifampin.
lnformation from Habib G, Lancellotti P, Antunes MJ, et al; ESC Scientific
Document Group. 201 5 ESC
Auidelines
for the management of infective
endocarditis: The Task Force for the Management of lnfective Endocarditis of the
European Society of Cardiology (ESC). Endorsed by European Association for
Cardio Thoracic Surgery (EACTS), the European Association of Nuclear Medicine
(EANI'/). Eur HeartJ.2015;36:3075 3128. IPMID: 26320109]doi:10.1093/
eurheartj/ehv319 and Baddour LM, Wilson WR, Bayer AS, et al; Committee on
Rheumatic Fever, Endocarditis, and Kawasaki Disease. lnfective endocarditis:
diagnosis, antimicrobial therapy, and management of complications: a statement
for healthcare professionals from the Committee on Rheumatic Fever, Endocardrtis,
and Kawasaki Disease, Council on Cardiovascular Disease in the Young, and the
Councils on Clinical Cardiology, Stroke, and Cardiovascular Surgery and Anesthesia,
American Heart Association: endorsed by the lnfectious Diseases Society of America.
Circulation. 2005;1 1 1 :e394-434. [PMID:
1 59561 45] doi:1 0.1 1 61 iCIRCULATIONAHA.
1 05.1 65564
perforation of the oral mucosa (Table 26). Although endocar
ditis prophylaxis was previously advised for a broad popula-
tion, current guidelines recommend its use only for the
following:
. o HistoU olendocarditis
o Cardiac transplantation with valve regurgitation due to a
structurally abnormal valve
. Prosthetic valve
. Prosthetic material used for cardiac valve repair, including
annuloplasty rings and chords
. Unrepaired congenital heart disease
. Repaired congenital heart disease with residual defects at
the site or adjacent to the site ofa prosthetic patch or device
Allergic to
penicillin or
ampicillin and
unable to take oral
medication 600 mg lM or lV
lM = intramusrular: lV intravenous.
'Regimen consists of a single dose 30 to 60 mrnutes before the dental procedure,
or, if inadvertently not administered, drug may be given up to 2 hours after the
procedure.
t'Or
other first- or second generation oral cephalosporin in equivalent adult
d osag e.
'Cephalosporins should not be used in an individual with a history of anaphylaxrs,
angioedema, or urticaria with penicillins or ampicillin.
lnformation from Wilson W Taubert KA, Gewiu N4, et ai; American Heart
Associat on Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee.
Prevention of infective endocarditis: guidelines from the American Heart
Association: a guideline from the American Heart Association Rheumatic Fever,
Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular
Disease in the Young, and the Council on Clinical Cardiology, Council on
Cardiovascular Surgery and Anesthesia, and the Oualrty of Care and Outcomes
Research lnterdisciplinary Working Group. Circulation.2007;116.1136 54. IPMID:
17 4464421 do :1 0.1 1 6 1 i Cl RCULATIONAHA. l 06.1 83095
o A defect that has been repaired with prosthetic mate-
rial (surgical or catheter-based) within the previous
6 months
t(EY P0lltT5
o Initial diagnostic tests for suspected infective endocar
ditis (lE) include blood cultures and transthoracic echo-
cardiography (TTE); TTE is followed by transesophageal
echocardiography in patients with suboptimal TTE results
or with high initial risk for IE.
. Early surgery is indicated for patients with acute infec
tive endocarditis (lE) presenting with acute and wors-
ening valve regurgitation resulting in heart failure;
left-sided IE caused by Stophylococcus oureus, fungal,
or other highly resistant organisms; IE complicated by
heart block, annular or aortic abscess, or destructive
penetrating lesion; and IE with persistent bacteremia
or fevers lasting longer than 5 to 7 days after starting
antibiotic therapy.
76

89. Myocardial Disease
Hypertrophic Ca rdiomyopathy
Clinical Presentation
Hypertrophic cardiomyopathy (HCpf) is an autosomal domi-
nant heritable disorder related to mutations in the genes that
primarily encode sarcomeric proteins. It is characterized by
increased left ventricular (LV) wall thickness in the absence of
loading conditions (e.g., hypertension) or other underlying
causes. HCM affects approximately I in 500 persons and may
be identified in all age groups, although in the United States,
index cases typically present within the third to fourth dec
ades of life.
Most patients with HCM are asymptomatic and have nor-
mal life expectancy, with diagnosis often resulting lrom evalu
ation of a heart murmur or abnormal ECG. However. certain
subsets of patients are more likely to develop symptomatic
disease and are at risk for sudden cardiac death (SCD).
Symptomatic individuals usually present with signs and
symptoms of heart failure (dyspnea, fatigue) or arrhythmias
(palpitations, syncope). SCD may be the initial manifestation
in some patients.
Heart failure symptoms are attributable to abnormal LV fill
ing (diastolic dysfunction) and dynamic left ventricular outflow
tract (LVOT) obstruction. Diastolic dysfunction is multifactorial,
involving increased chamber stiffness, progressive fibrosis, and
myocardial ischemia due to mismatch of coronary flow and LV
mass. Dynamic LVOT obstruction, characterized by asymmet-
ric LV hypertrophy with prominent interventricular septal
thickening, is the classic form of HCM. Outflow tract obstruc
tion in HCM is found on echocardiography in 30'X, of patients
at rest and in an additional 407, of patients with provocation;
30'1, of patients have no obstruction. During ventricular systole,
anterior motion of the mitral valve results in early to midsys
tolic obstruction of the LVOT and subsequent mitral regurgita-
tion related to leaflet malcoaptation ("eject obstruct leak"
Finding or Maneuver Hypertrophic Cardiomyopathy with
Dynamic LVOT Obstruction
Myocardial Disease
triad). Patients with dynamic obstruction may develop dysp-
nea, presyncope, or syncope during periods ofincreased ven
tricular contractility (exercise) or with decreases in ventricular
preload or afterload, all of which may worsen the degree of
obstruction.
Atrial fibrillation is common in patients with HCM, and
risk increases with age. During atrial fibrillation with rapid
ventricular response, diastolic filling periods shorten, worsen
ing diastolic dysfunction; reduced LV filling may exacerbate
the LVOT gradient.
Evaluation
Physical examination findings may be normal in patients
without IVOT obstruction. The most common feature related
to LVOT obstruction is a cardiac murmur, and dynamic
maneuvers during examination are helpful in differentiating
HCM from fixed valvular obstruction (Table 27).
Twelve-lead ECG reveals abnormal findings, such as
increased QRS voltage, evidence of left atrial enlargement, LV
conduction abnormalities, pathologic
Q waves, and significant
repolarization abnormalities, in 75"1, to 95% of persons with
HCM (Figure 37); however, there is substantial interpersonal
variability in the degree of abnormalities.
The diagnosis of HCM is most commonly established
by transthoracic echocardiography (TTE) (Figure 38). Echocar-
diography demonstrates the magnitude and distribution ol
hypertrophy (>15 mm in any LV region or 13 14 mm in a per
son with a first-degree relative with HCM), reveals the pres
ence and degree of dynamic LVOT obstruction and mitral
regurgitation, and characterizes diastolic LV filling. Doppler
echocardiography is the modality of choice for quantiffing the
LVOT gradient to difl'erentiate obstructive from nonobstructive
HCM, an important distinction in symptom management. In
patients with HCM and resting LVOT gradient less than 50 mm
Hg, echocardiography with provocative maneuvers (Valsalva,
squatting) is recommended to establish the diagnosis. ll
provocative maneuvers fail to elicit an outflow gradient in
Valvular Aortic Stenosis
Ejection-quality murmur usually best heard at
left lower sternal border; generally does not
radiate to the carotid arteries
Brisk upstroke; may have two impulses for each
ejection (bifid)
lncrease in intensity of murmur during strain
phase(LR+=14)
lncrease in intensity of murmur with standing
from squat or seated position (LR+
= 6.0);
decrease in murmur with elevation of legs when
supine (LR+ = 7.6)
No change or decrease in intensity of pulse
Ejection-quality murmur usually best heard at
right second intercostal space with radiation to
the carotid arteries
Upstroke is often diminished and delayed
(parvus ettardus)
No change or diminished murmur intensity
during strain phase
No significant change in intensity of murmur
with position
Characteristic of murmur
lncrease in intensity of pulsePeripheral pulse after PVC
Carotid impulse
Valsalva maneuver
Position
TAB LE 2 7. Physical Examination Findings of Dynamic Left Ventricular Outflow Tract Obstruction Versus Fixed
Valvular Aortic Stenosis
LR+ = positive likelihood ratio; LVOT = left ventricular outflow tract; PVC = premature ventricular contraction-
77

90. Myocardial Disease
aVL
VI
w
1
lil aVF V3
tIGURE 37. ECGinapatientwithapical hypertrophiccardiomyopathy. lncreasedORSvoltageandmarkedrepolarizationabnormalities,especiallyinleadsV2throughV5,
are present.
symptomatic patients, exercise echocardiography is recom-
mended. ln patients with inconclusive echocardiographic
findings, cardiac magnetic resonance (CMR) imaging is indi-
cated to clarify the diagnosis.
Upon initial diagnosis of HCM, 24 to 48 hour ambula
tory ECG monitoring should be performed to evaluate for
arrhy.thmias. The presence of nonsustained ventricular tachy
cardia indicates higher risk for SCD. Exercise stress testing is
reasonable to determine lunctional status and to provide prog
nostic information as part of the initial evaluation.
HCM must be differentiated flrom other conditions associ
ated with increased ventricular wall thickness (Table 28).
Particularly challenging is the diflerentiation of HCM from
hypertensive heart disease and from the normal and compensa
tory changes in LV wall thickness seen in competitive athletes
(athlete heart). In patients with hypertension, the likelihood of
concomitant HCM is increased if LV wall thickness is greater than
18 mm and nonconcentric and dynamic LVOT obstruction is
present. In athletes, LV cavity dilatation and normal diastolic fill
ing on echocardiography favor normal physiologic changes,
whereas unusual or asymmetric pattems of hypertrophy favor
HCM. A decrease in wall thickness after a brielperiod of decon-
ditioning favors athlete heart. When the diagnosis remains
unclear, CMR imaging with gadolinium contrast may help with
differentiation.
Risk Stratification
Patients with HCM have an annual risk for death of 1',{,, pri-
marily related to fatal arrhythmia and heart failure. Most
arrhythmic deaths are caused by ventricular fibrillation, and
all patients with HCM, regardless of the presence of obstruc
tion, should undergo assessment for SCD risk factors at the
time of diagnosis and every I to 2 years. Major risk factors for
SCD are listed in Table 29.
An implantable cardioverter defibrillator (lCD) is effec
tive in preventing SCD in appropriately selected high risk
patients. Patients with previous SCD or sustained ventricular
tachycardia have an annual recurrence rate of l0'1, and should
receive an ICD for secondary prevention. In patients with one
or more established risk factors, primary prevention with
an ICD is reasonable. For patients with HCM who are not
j
lt
Clinical FeaturesCondition
Elevated blood pressure
No hypertension; normal or supranormal
exercise capacity
Heartfailure, low voltage on ECG, possible
neuropathy and/or nephropathy; usually
older patients
Male predominance (X-linked), neuropathic
pain, kidney dysfunction, telangiectasias,
angiokeratomas; typically young patients
Ataxia, scoliosis, pes cavus, visual and
hearing impairment
Fabry disease
Friedreich ataxia
Amyloidosis
Hypertension
Athlete heart
TABLE 28. Conditions With lncreased Left Ventricular
WallThickness
7A

91. Myocardial Disease
with peak gradient of 59 mm Hg. LA = left atrium; LV = left ventricle.
otherwise identified as high risk for SCD or in whom a
decision to proceed with ICD placement remains uncefiain,
CMR imaging is beneficial to assess for maximum LV wall
thickness, ejection fraction, LV apical aneurysm, and extent of
HCM = hypertrophic cardiomyopathy; LV = left ventricular; LVEF = left ventricular
ejection fraction.
"For
adults with one or more malor risk factors, it is reasonable to offer an
implantable cardioverteLdefibrillator (ciass 2a recommendation).
lnformation from Ommen SR, N,4ital S, Burke MA, et al; Writing Committee
Members. 2020 AHA/ACC guideline {or the diagnosis and treatment of patients
with hypertrophic cardiomyopathy: a report of the American College of
Cardiology/American Heart Association Joint Committee on Clinical Practice
Guidelines. Circulatioo.2020:1 42:e558-e63 1 . IPMID: 332 1 593 1 ] doi:1 0.1 1 61 /
crR 0000000000000937
myocardial fibrosis with late gadolinium enhancement (LGE).
LGE is associated with increased risk for ventricular arrhlth
mias; in patients with indeterminate risk for SCD, LGE quan
tification of 15'7, or more can serve as a risk modifier in
decision making regarding ICD placement.
Management
Management of HCM includes lifestyle modification; treat
ment of symptoms referable to LVOT obstruction, heart failure,
and atrial fibrillation; and potentially ICD therapy.
In patients with HCM who have overweight or obesity,
therapeutic lifesSzle interventions should be implemented to
achieve weight loss and potentially decrease the risk for LVOT
obstruction, heart failure, and atrial fibrillation. Patients with
symptoms of sleep disordered breathing should be formally
evaluated, including with a sleep study.
Patients with HCM should be advised to avoid dehydra
tion, excessive alcohol intake, and exposures that may cause
vasodilation and decreased preload (e.g., saunas, hot tubs)
because these situations may provoke greater LVOT obstruc
tion. Mild- to moderate intensity recreational exercise is ben
eficial for most patients with HCM. Athletes with HCM should
Sudden death in a first-degree relative or close relative
age (50 y
LV wall thickness >30 mm
>1 Episode of syncope thought to be arrhythmic in nature
LV apical aneurysm
LVEF <50%
TABLU 29" Major Risk Factors for Sudden Cardiac Death in
Patients With HCM"
79

92. Myocardial Disease
undergo a comprehensive evaluation and be engaged in shared
discussion of potential risks of sports participation.
In symptomatic patients, nonvasodilating p blockers
should be initiated. with titration to effectiveness or maxi
mum tolerance. Carvedilol, labetalol, and nebivolol should be
avoided. Alternatively, verapamil or diltiazem may be used in
patients in whom p blockers are contraindicated or not toler
ated. For patients with LVOT obstruction and persistent symp
toms, adding disopyramide. a class IA antiarrhyhmic drug
with potent negative inotropic activity, to one of the other
drugs is a recommended option. Diuretics must be used cau
tiously and only if symptoms of dyspnea cannot be managed
with other therapy. Because oftheir propensity to exacerbate
LVOT obstruction, nitrates and phosphodiesterase 5 inhibitors
should not be used concomitantly.
Invasive treatment of obstruction with surgical septal
myectomy or catheter based alcohol septal ablation is recom
mended in patients with severe obstructive symptoms refrac
tory to maximal medical therapy and a resting or provoked
LVOT gradient of 50 mm Hg or greater. Although both surgical
myectomy and alcohol septal ablation reduce the LVOT gradi
ent and obstructive symptoms, appropriate patient selection
for each procedure is controversial. Surgical myectomy is asso
ciated with a higher likelihood of complete symptom resolu
tion, greater obstruction reliel and a lower rate of repeat
procedures. Alcohol septal ablation carries a higher risk for
atrioventricular block requiring pacemaker implantation; this
risk is higher in older patients. Decisions regarding therapy
must be individualized. Septal myectomy is favored in young
patients and in patients with associated cardiac disease requir
ing surgical treatment, whereas alcohol septal ablation may be
more appropriate for patients with comorbid conditions and
increased surgical risk. Outcomes with either procedure are
best when the procedure is performed in a center with exper
tise in HCM management. Surgical myectomy is associated
with low operative mortality (O.a'2,) in such centers.
Atrial fibrillation should be managed with rate control and
anticoagulation, regardless of the patient's CHATDS, VASc score.
Direct oral anticoagulants are considered first line anticoagulant
therapy, with warfarin as a second line option. Low molecular
weight heparin or warfarin (maximum dose <5 mg/day) is rec
ommended for pregnant women with HCM and atrial fibrillation.
Anticoagulation is also recommended in patients with an epi-
sode of subclinical atrial fibrillation of more than 24 hours'dura
tion detected by ambulatory monitoring. Patients with HCM and
concomitant atrial fibrillation often remain symptomatic despite
rate control, and rhl.thm control in conjunction with anticoagu
lation should be considered early (see Arrhythmias). Digoxin
should be avoided in patients with atrial fibrillation because the
positive inotropic effects may worsen the LVOT gradient.
Fewer than 5'1, of patients progress to end stage HCM,
manifesting as dilated cardiomyopathy with systolic dysfunc
tion. For patients with HCM who develop systolic dysfunction
(LVEF <50'7,), guideline-directed therapy is recommended (see
Heart Failure).
Surveillance
An ECG should be obtained every 1 to 2 years in asymptomatic
patients with HCM to screen for changes in rhythm or conduc
tion. Patients should also undergo 24 lo 48 hour ambulatory
ECG monitoring at diagnosis and every I to 2 years thereafter
if asymptomatic or with the development of symptoms that
suggest arrhyhmia, such as palpitations or syncope.
Repeat TTE is recommended with any change in clinical
status or new cardiac event. In asymptomatic patients. TTE is
recommended every 1 to 2 years to assess for mitral regurgita
tion and changes in LV hypertrophy. function, and degree of
obstruction. Although most patients with HCM have normal
LV function, a decrease in LV systolic function is associated
with worse outcomes, including death.
Role of Genetic Testing and Counseling
Patients known or suspected to have HCM should undergo an
evaluation ol familial inheritance with a three-generation
family history and receive genetic counseling. Genetic testing
is recommended in patients who meet the clinical definition
of HCM to aid screening of family members, but results must
be interpreted carefully. A pathogenic sarcomeric mutation
may be identified in up to 60%, of patients with a family his
tory of HCM; the incidence is lower (2o'X, 3O'X') in isolated
cases.
First degree relatives of patients with HCM should
undergo screening with ECG and echocardiography. ln first
degree relatives, ECG and cardiac imaging should be per
formed every 1 to 2 years in children and adolescents and every
3 to 5 years in adults or with a change in clinical status.
Alternatively, genetic testing should be offered to first degree
family members if a causative sarcomeric mutation is identi
fied in the index case. Genetic testing may identi$r genotype
positive persons who do not express phenotypic features of
HCM, and these persons should be followed with clinical
examinations at the same intervals described. The absence of
an identified sarcomeric mutation does not exclude HCM in
the index patient, and first degree relatives should continue
clinical screening. Genorype negative relatives in families
with genotlpe positive HCM do not require ongoing clinical
screening. The use of genetic testing in the assessment of SCD
risk and need for ICD placement is uncertain.
TEY POIXT'
. Doppler echocardiography, at rest and with provoca-
tion, is the modality of choice for quantification of the
left ventricular outflow tract gradient in patients sus-
pected of having obstructive hypertrophic cardiomyo
PathY.
o Patients with hlpertrophic cardiomyopathy who have
experienced sudden cardiac death or sustained ventricu
lar tachycardia or who have major risk factors should
receive an implantable cardioverter-defibrillator for
preventionofsuddencardiacdeath.
(Continued)
80

93. Myocardial Disease
l(EY P0lllIS kominued)
. In patients with hypertrophic cardiomyopathy who
have symptoms of left ventricular outflow tract obstruc
tion, nonvasodilating B blockers are first line therapy.
. Transthoracic echocardiography should be performed
every 1to 2 years in asymptomatic patients with hyper-
trophic cardiomyopathy to assess for mitral regurgitation
and changes in left ventricular hypertrophy, function,
and degree of obstruction.
HVC o Genetic testing is not indicated in first-degree relatives of
patients with hypertrophic cardiomyopathy unless a path
ogenic genetic variant is identified in the index patient.
Cardiac Amyloidosis
Cardiac amyloidosis results from deposition of misfolded pro
teins within the myocardium, leading to restrictive physiologr
and heart failure symptoms. There are two major forms-light
chain amyloid (AL) amyloidosis and transthyretin amyloid
(AITR) amyloidosis (Table 3O). Cardiac amyloidosis is sug
gested by increased wall thickness on echocardiogram accom
panied by a low voltage ECG; however, these findings are not
diagnostic. CMR imaging is highly sensitive and specific for
cardiac amyloidosis, but it does not distinguish betvveen types.
AL amyloidosis is a multiorgan disease associated with
plasma cell dyscrasias. The diagnosis is based on the presence
of an amyloid-related systemic syndrome; amyloid deposits
on fat pad aspiration, bone marrow biopsy, or organ biopsy;
abnormal serum immunoglobulin light chains; and monoclo-
nal gammopathy (see MKSAP 19 Hematologr). Endomyocardial
AL [Yl
=
F, age 40-80 y
tIGURE 39. 99m-Technetium pyrophosphate scan in a patientwith hereditary
transthyretin cardiac amyloidosis. Cardiac uptake of the radiotracer is denser than
bone uptake at both t hour(toppanel) and 3 hours (bottom pahel\.
biopsy is diagnostic but unnecessary in the presence of extra-
cardiac amyloidosis with abnormalities on CMR imaging.
Treatment is directed toward the hematologic malignancy.
Cardiac transplantation can be considered selectively.
Hereditary ATTR (h-ATTR) amyloidosis results from a
genetic mutation in the TTR gene, most commonly Val122lle,
which occurs in 3.4"/,, of Black persons Iiving in the United
States. Wild-type ATTR (wt ATTR) amyloidosis is an aging
linked process that may be responsible for up to 10% to 15'2, of
cases ofheart failure in the elderly. In addition to heart failure,
ATTR amyloidosis may cause sensory and autonomic neuropa
thy and carpal tunnel syndrome. In the setting of abnormal
CMR imaging results, 99m technetium pyrophosphate scin
tigraphy of the heart may be diagnostic (Figure 39). Tafamidis,
t
ATTR h-ATTR: M >> F,
age 55-75 y
wt-ATTR: M >>> F
age 65-95 y
Nephrotic syndrome,
cardiomyopathy,
h e patosp le n om eg a ly,
neuropathy, carpal tunnel
syndrome, bleeding
diathesis, periorbital
purpura, macroglossia
Cardiomyopathy, ca rpal
tunnel syndrome, spinal
stenosis, neuropathy
Analysis for clonal plasma
cell dyscrasias with SPEP
and UPEP
Serum and urine
immunofixation
Serum FLC testing
Bone marrow biopsy
CMR imaging with LGE
Endomyocardial biopsy or
other tissue biopsy
CMR imaging with LGE
Techneti u m pyrophosphate
scintigraphy
Endomyocardial biopsy
(controversial)
Genetic testing
Treatment of plasma cell
dyscrasias
Cardiac transplantation in
selected cases
Tafamidis
Diflunisal (off label)
Patisiran ( neuropathy)
lnotersen (neu ropathy)
Liver or heart-liver
transplantation
AL=amyloidlightchain;ATTR=amyloidtransthyretin;CMR=cardiacmagneticresonance;F=females;FLC=freelightchain;hATTR=hereditaryamyloidtransthyretn;
LGE = late gadolinium enhancement; Ny' = males; SPEP = serum protein electrophoresis; UPEP = urine protein electrophoresis; wt ATTR = wild'type amyloid transthyretin.
Subtype Demographics Clinical Manifestations Diagnostic Tests Therapy
TABLE 30. Subtypes of Cardiac Amyloidosis
81

94. Myocardial Disease
a protein stabilizer, is an FDA approved therapy that reduces
mortality and cardiovascular hospitalization in patients with
ATTR cardiac amyloidosis. Patisiran and inotersen, which
reduce hepatic production of transthyretin, are indicated for
ATTR polyneuropathy and may have benefit for ATTR cardio
myopathy. Liver or combined heart liver transplantation may
be considered for patients with h ATTR amyloidosis.
ftY P0txrs
o Cardiac amyloidosis is suggested by heart failure with
restrictive physiologr, increased wall thickness, and low
ECG voltage.
. Light chain amyloidosis is a multisystem disease for
which treatment is directed toward underlying plasma
cell dyscrasias.
. Hereditary transthyretin amyloidosis causes heart fail-
ure and sensory and autonomic neuropathy; treatment
with tafamidis, a protein stabilizer, reduces mortality.
Restrictive Ca rd iomyopathy
Clinical Presentation and Evaluation
Restrictive cardiomyopathy (RCM) is a rare disorder charac-
terized by abnormally stiff, noncompliant ventricles. RCM
was once considered idiopathic; however, increasing evidence
suggests that gene mutations in sarcomeric proteins and
abnormalities in desmin, an intermediate filament that regu-
lates sarcomeric architecture, play an important role in famil
ial and sporadic cases. The sarcomeric gene mutations of RCM
are similar to or the same as those linked to HCM, raising the
possibility that these disorders represent different phenotypic
expressions of the same heritable defect. Histologically, RCM
is characterized by patchy interstitial fibrosis and myocyte
disarray, which are also seen in HCM. With increasing inter
stitial fibrosis, the ventricles stiffen, resulting in increased
pressure during normal diastolic fi1ling.
Patients with RCM can present at any age, usually with dysp
nea, peripheral edema, and exercise intolerance. Hepatomegaly
and ascites may be present late in the disease course. RCM should
be suspected when echocardiognphy reveals biatrial enlarge
ment and severe diastolic dysfunction in the setting of normal
ventricular size, wall thickness, and systolic function. Pulmonary
hypertension is commonly present, as are tricuspid and mitral
valve regurgitation.
Primary RCM must be differentiated from other condi-
tions involving restrictive physiologr. These include conditions
in which wall thickness is typically normal (radiation induced
fibrosis, eosinophilic diseases, hemochromatosis) as well as
infiltrative diseases with increased wall thickness (see Cardiac
Amyloidosis). A complete blood count with manual differen
tial, serum ferritin, and transferrin saturation are reasonable
as part of the evaluation. CMR imaging with gadolinium con
trast may help distinguish between conditions. When the
diagnosis remains unclear, endomyocardial biopsy is reason-
able for establishing a diagnosis.
Differentiating Restrictive Cardiomyopathy
From Constrictive Pericarditis
RCM and constrictive pericarditis present with similar symp-
toms and echocardiographic findings. Differentiating between
the two disorders is essential because surgical pericardiectomy
may relieve symptoms and prolong life in patients with con
striction. In patients with previous cardiac surgery pericardi
tis, or chest irradiation, constrictive pericarditis should be
strongly considered.
On physical examination, patients with RCM or con
striction demonstrate elevated central venous pressure.
Increase in the height of the jugular venous waveform during
inspiration (Kussmaul sign) is more commonly associated
with constriction. Both RCM and constrictive pericarditis
may be associated with a diastolic sound an S, gallop in
RCM and a pericardial knock in constrictive pericarditis.
Differentiating between these sounds may be dilficult (see
Pericardial Disease).
A multimodality approach. including noninvasive imag
ing and invasive hemodynamic evaluation, may be required to
distinguish RCM lrom constrictive pericarditis. Clues to the
presence of constrictive pericarditis include pericardial calci
fication on chest radiography or CT, pericardial thickening on
CT or CMR imaging, and a B type natriuretic peptide level
below 100 pg/ml (100 ng/L) (usually >400 pgrml [400 ngi L]
in RCM). A hallmark feature of constrictive pericarditis is ven
tricular interdependence, whereby total cardiac volume is
limited by the rigid pericardium. With ventricular interde
pendence. increased filling ol the right or left ventricle can
occur only with reciprocal decreased filling of the other ven
tricle. Ventricular interdependence may be demonstrated by
Doppler echocardiography, CMR imaging, or invasive hemo
dynamic evaluation in patients with constriction; however. it
is not present in patients with RCM.
Management
There are no medical therapies specifically for RCM. Loop diu
retics are usually necessary for relief of congestive symptoms.
especially in late stage disease. However, patients with RCM
require relatively high filling pressures to maintain cardiac
output, and balancing relief of congestion with adequate car
diac output is often challenging. Volume status should be
monitored carefully, because even small changes in volume
may lead to renal hypoperfusion.
Atrial fibrillation is common in patients with RCM and is
poorly tolerated because of increased heart rate and reduced
ventricular filling. Anticoagulation and rate control are indi
cated. and rhythm control should be considered early in
symptomatic patients.
Survival is poor in patients with RCM. r.r'ith a 5 year mor
tality rate of 36'7, and a l0 year mortality rate of 63'X,. Cardio
vascular mortality is predominantly related to progressive
heart failure and arrhythmias. Cardiac transplantation may be
considered in select patients who remain symptomatic despite
maximal therapy. There is no accepted indication for primary
t
82

95. prevention ICD placement in patients with RCM who have
preserved systol ic function.
xtY torf,rs
. Restrictive cardiomyopathy should be suspected when
echocardiography reveals biatrial enlargement and
severe diastolic dysfunction in the setting of normal
ventricular size, wall thickness, and systolic function.
o Differentiating between restrictive cardiomyopathy and
constrictive pericarditis is essential because surgical
pericardiectomy may relieve symptoms and prolong life
in patients with constriction.
Cardiac Tumors
Most cardiac tumors are metastatic. Neoplasms with the high
est metastatic potential are melanoma, malignant thymoma,
and germ cell tumors. Common tumors with an intermediate
risk for cardiac involvement include carcinoma of the lung,
stomach, and colon. Therapy is directed at systemic treatment
of the underlying neoplasm, with cardiac surgery reserved for
patients with obstructive symptoms.
Primary cardiac tumors, which are exceedingly rare, are
benign in two thirds of patients. Nearly 50'X, of primary cardiac
tumors are atrial mlxomas. Myxomas may occur in either
atrium but are most commonly attached to the fossa ovalis
within the left atrium (Figure 4O). Myxomas are usually soli
tary and discovered at a mean age of 50 years, often after a
systemic embolic event. Patients with a mlxoma may present
with constitutional symptoms related to interleukin produc-
tion, embolic phenomena from tumor fragmentation, or
symptoms referable to intracardiac obstruction (dyspnea, syn
cope). When mitral valve obstruction is present, auscultatory
findings are similar to those of mitral stenosis; however, find
ings may vary with position or be associated with an early
diastolic sound, known as the tumor plop. Surgical removal is
indicated to prevent embolic events, as is subsequent surveil-
lance echocardiography for detection of recurrence.
Papillary fibroelastomas usually occur on the surface of
the aortic and mitral valves and are commonly discovered in
the eighth decade ollife. Although most papillary fibroelasto
mas do not cause symptoms, they may be associated with
stroke, transient ischemic attack, and, rarely, coronary embo
lization with infarction. On echocardiogram, these tumors
often have a heterogeneous globular shape or a mobile frond-
like appearance. Patients with embolic symptoms are treated
surgically. There are no randomized data comparing surgical
therapy with antithrombotic or antiplatelet therapy to prevent
embolic events.
Angiosarcomas are the most common primary malignant
tumor. Angiosarcomas typically arise in the right atrium and
are often associated with pericardial effusion (Figure 41).
Dyspnea and chest pain are common presenting symptoms.
Angiosarcomas are highly vascular tumors, and CT or CMR
imaging with contrast may help differentiate an angiosarcoma
Myocardial Disease
tlGU RE 40. 4,Transthoracic echocardiogram during diastole demonstrating a
left atrial myxoma originating from the interatrial septum and prolapsing across the
mitral valve (MV) into the left ventricle (LV). B, Parasternal long'axis view. ( Excised
myxoma with portion of the lossa ovalis (arow). LA = left atriu m; RA = rig ht atriu m;
RV= rightventricle.
83

96. Pericardial Disease
TABLE 31 . Causes of Pericardial Disease
F
\
Category
lnfectious
Viral infection
Cause
Enterovirus, herpesvirus, adenovirus,
parvovirus
Myco b a cte ri u m tu be rcu I o si s,
Pneu mococcu s spp., Staphy/ococcus spp
Coxiel I a bu rnetii, Borre I i a bu rgdo rfe ri
H i sto p I a sm a, Aspe rg i I I u s, B I a stomyce s,
Candida spp.
Systemic lupus erythematosus,
rheumatoid arthritis, Sjogren syndrome,
systemic sclerosis, sarcoidosis, familial
Mediterranean fever
Metastatic lung cancer, breast cancer, and
melanoma; lymphoma and leukemia
Uremia, myxedema
Bacterial
infection
Fungal infeaion
Noninfectious
Autoimmune
diseases
Cance
F I G U R E 4 1 . Cardiac magnetic resonance image in a patient with an angiosarcoma.
A heterogeneous m asslwhite anow)isinfiltrating the right atrial wall. A pericard ial
eflusion(yellow arrow) is also present.
from a right atrial mlxoma. Most patients die in 6 to 12 months.
Even when complete surgical extirpation is possible, survival
remains less than 2 years for most patients.
r(EY P0rt{Ts
. An atrial myxoma should be considered in patients with
auscultatory findings of mitral stenosis that vary with
position.
. Atrial myxomas should be surgically removed to prevent
embolic events.
Pericardial Disease
Acute Pericarditis
Clinical Presentation and Evaluation
Pericarditis is inflammation of the pericardium, the thin
fibrous sac surrounding the heart. It may be subclinical or
present as sharp precordial pain of acute onset. Acute pericar-
ditis has many causes (Table 31), but it is most often idiopathic
or presumed viral in origin.
Acute pericarditis is diagnosed clinically by the presence
of at least two of four criteria: chest pain typical for pericardi-
tis, a pericardial friction rub, new ECG changes, or a new peri-
cardial effusion (Table 32). Chest pain attributable to acute
pericarditis is typically sharp, severe, and positional. It is not
exacerbated by exertion nor relieved by rest or nitrogfcerin,
unlike anginal pain. Pain is characteristically worse in the
supine position and improves with sitting up and leaning
forward. These pain features may be related to tension of the
pericardium at its sternal and diaphragmatic attachments.
A pericardial friction rub is a hallmark of acute pericarditis
and is frequently present on auscultation. The harsh, scratchy
Metabolic
conditions
Drug-related
causes
Hydralazine, procainamide, minoxidil,
all-trans retinoic acid
Cardiac surgery (postpericardiotomy
syndrome), coronary perforation during
percutaneous intervention, pacemaker
lead penetration, radiofrequency ablation
lrradiation, aortic dissection, pulmonary
arterial hypertension
latrogenic causes
Other
sound classically has three components corresponding to the
cardiac rycle during norrnal sinus rhythm: atrial systole. \,en-
tricular systole, and ventricular filling. The three phases ofthe
pericardial friction rub differentiate it from a pleural friction
rub, which has two components linked to respiration. Less
commonly, the rub of pericarditis may be monophasic or bipha
sic, but it is not affected by respiration. Auscultation should be
performed during end expiration n ith the patient in the supine
position or sifting upright.
The tlpical ECG feature of acute pericarditis is concave
ST-segment elevation in multiple leads that does not corre
spond with a single coronary artery distribution. PR segment
depression in lead II or reciprocal PR segment eler'ation in
lead aVR also may be present (Figure 42). In contrast. ECG
findings of acute myocardial infarction are hyperacute T
waves, ST-segment elevation consistent with a single coronary
distribution, reciprocal ST-segment depression, pathologic
Q
waves during myocardial infarction evolution, and absence of
PR-segment change.
Echocardiography should be performed to evaluate fbr
pericardial effusion; however, the absence of effusion does not
exclude acute pericarditis. When the diagnosis remains uncer
tain, cardiac magnetic resonance (CMR) imaging u'ith intrave
nous gadolinium contrast may be used to identifi'evidence of
pericardial inflammation, characterized by pericardial thicken
ing and late gadolinium enhancement (Figure 43). Alternatively,
gated cardiac CT may demonstrate pericardial inflammation.
84

97. I
!
l
3
t
I
I
Pericardial Disease
Physical
examination
Chest radiography
Echoca rd iog raphy
CMR imaging
Cardiac
catheterization
Concave ST-seg ment elevation
in multiple leads, PR-segment
depression in lead ll or
PR-segment elevation in aVR
Usually normal
Possible pericardial effusion
Pericardial inflammation with
late gadolinium enhancement
Pericard ial inflammation
Not applicable
Sinus tachycardia, electrical
alternans, possible low voltage
Possible enlarged cardiac silhouette
(water bottle heart), clear lungs
RV early diastolic collapse, RA late
diastolic collapse, abnormal
interventricular septal motion,
inspiratory decrease in mitral inflow
velocity, IVC plethora
Usually not necessary
Usually not necessary
Usually not necessary
Elevated CVP, increasing CVP with
inspiration, pericardial knock, right-
sided HF
No pathognomonic findings
Possible pericardial calcification,
more often normal
lncreased pericardial thickness,
inspiratory decrease in mitral inflow
velocity, biatrial enlargement, normal
ventricular size and systolic function,
IVC plethora
Pericardial thickening and
calcification
Pericardial thickening and
ca lcification
Ventricular interdependence, square
root signs in RV and LV pressure
tracings
Three-component friction rub Tachycardia, elevated CVB
diminished y descent, pulsus
paradoxus, pericardial rub if
inflammatory etiology
ECG
CT
t
t-
i
t
,-
a
'1
CMR= cardiac magnetic resonance; CVP=central venous pressure; HF = heartfailure; IVC = nferlorvena cava; LV= eftventric!lar; RA= right atrial; RV= right ventricular
I
Additional findings that support acute pericarditis include
fever and serologic evidence of inflammation (leukocl,tosis,
elevated erythrocyte sedimentation rate or C-reactive protein
[CRP] lerel). Serum cardiac troponin levels are normal or may
be slightly elevated if there is a component of myopericarditis.
Although most cases of acute pericarditis are idiopathic
or viral, a search fbr alternative causes is appropriate (see
Table 31). Additional testing beyond the initial evaluation is
based on suspicion for a specific cause. Tuberculosis should be
considered in hospital workers, persons who are incarcerated
;
{
FIGURE 42. ECGchangesofacutepericarditis.ConcaveST-segmentelevationispresentinmostoftheleads(arowheads).ThePRsegmentisdepressedinallleadsexcept
aVR (arrows).
Evaluation Acute Pericarditis Cardiac Tamponade Constrictive Pericarditis
TABLE 32. Evaluation of Pericardial Disease
85

98. Pericardial Disease
F I G U R E 4 3. Cardiac magnetic resonance image obtained after intravenous
administration of gadolinium. A pericardial effusion ( red anow is present, and
there is late gadolinium enhancement of the pericardium consistent with
inflam mation (white a rrowl.
or residing in chronic care facilities, and residents ofor travel
ers to an endemic area. Treatment of tuberculosis may reduce
the risk for reactive pericardial constriction from greater
than B0'1, to less than 10'){,. Other causes of acute pericarditis
include uremia, recent cardiac surgery. and chest irradiation.
Postpericardiotomy syndrome, characterized by pericardial
inflammation that is likely autoimmune in nature, may occur
after cardiac surgery with a latency period of several weeks.
The presentation and treatment of postpericardiotomy syn
drome are similar to those ol idiopathic pericarditis.
Management
Most patients with acute pericarditis can be managed as out
patients; however, patients with accompanying high risk
features may require hospitalization for treatment and moni
toring. These predictors of poor prognosis include tempera
ture higher than 38'C (100.4 "F), subacute onset (days to
weeks without acute chest pain at onset), a large pericardial
effusion (>20 mm diastolic echo-free space) or tamponade at
presentation, oral anticoagulation therapy, or lack ofresponse
(no relief in symptoms and/or inflammatory markers) after
1 week of treatment.
First line therapy for acute idiopathic pericarditis is
aspirin (750 1000 mg) or NSAIDs (ibuprofen 600 mg) every
B hours for 1to 2 weeks. Colchicine (0.5 mg once or twice daily
for 3 months) is recommended as adjunctive therapy to
shorten symptom duration and reduce treatment failure and
recurrence. Patients who respond to therapy initially but
develop recurrent pericarditis after treatment completion may
benefit from a longer course of standard therapy with slow
tapering. CRP may be useful as a marker of treatment response,
with tapering initiated after the CRP level normalizes.
Glucocorticoid therapy is reserved for patients with per-
icarditis that is recurrent, incessant (>4 6 u,eeks'duration).
or chronic (>3 months' duration) despite standard therapy:
uremic pericarditis not responsive to intensive dialysis; con
traindications to NSAID therapy; or autoimmune-mediated
pericarditis. Prednisone is added to standard therapl, (aspi
riniNSAIDs and colchicine) at a dosage of 0.25 mg,'kg to
0.5 mg/kg and continued for 3 months. Tapering should not
be initiated until after the first 2 to 4 weeks of therapy or until
the CRP level normalizes. Targeted inhibition of interleukin I
with rilonacept is an effective glucocorticoid sparing therapy
fbr idiopathic recurrent pericarditis refractory to standard
treatment.
Expert consensus opinion is that athletes should not
return to competitive exercise for 3 months from initial onset
ofpericarditis and that nonathletes restrict strenuous activity
until symptoms resolve.
xtY Potilrs
o Acute pericarditis is diagnosed by the presence ofat
least tvvo offour criteria: chest pain typical for pericar
ditis, a pericardial friction rub, new ECG changes. or a
new pericardial effusion.
o First line therapy for acute idiopathic pericarditis is
high dose aspirin or NSAIDs and adjuvant colchicine
therapy.
o Glucocorticoid therapy is reserved for patients with
incessant, recurrent, or chronic pericarditis despite
standard therapy; uremic pericarditis not responsive to
intensive dialysis; contraindications to NSAID therapy;
or autoimmune-mediated pericarditis.
Pericardial Effusion and
Cardiac Tamponade
Pericardial Effusion
Pericardial effusions are often asymptomatic, with incidental
discovery on chest radiograph, CT. or echocardiogram. ln
asymptomatic patients, most effusions are idiopathic: how
ever, malignancy, infections, autoimmune disease. hlpothy
roidism, and iatrogenic causes (medications, anticoagulation
therapy) should be considered (see Table 31). ln countries
where tuberculosis is endemic. more than 60'/. of effusions are
caused by tuberculosis.
Pericardiocentesis should be considered for diagnostic
and therapeutic purposes if cancer or bacterial infection is
strongly suspected. In patients with pericardial effusion of
unknown cause and elevated inflammatory markers. empiric
treatment of pericarditis may be reasonable. Drainage should
be considered for large idiopathic effusions ol more than
3 months' duration, because one in three patients progress to
cardiac tamponade.
I
l
t
i
'!
i
I
i
I
I
i
:
t
:
;
;
85

99. Pericardial Disease
n
tt
tt
aVL
VI
I
tt
n
V5
FIGURE 44. ECGdemonstratingelectrical alternans,acyclicbeat-to-beatshiftinthe0RSaxisthatmayrepresentswingingof theheartwithinalargepericardial effusion,
as well as supraventricular tachycardia.
v4
v5
v0
Cardiac Tamponade
Clinical Presentation and Evaluation
Cardiac tamponade occurs when tluid accumulation within
the pericardial space compresses the heart and impedes dia-
stolic filling. With rapid fluid accumulation, as may occur
with trauma, aortic dissection, or invasive cardiac procedures,
tamponade may arise at relatively low pericardial volumes.
Subacute or chronic processes, such as neoplastic disease or
hypothyroidism, may be associated with much larger efl'u-
sions (several hundred milliliters in volume).
Clinical signs o1'tamponade include tachycardia, muffled
heart sounds, and elevated central venous pressure (see
Table 32). Blood pressure may be elevated or normal early in
the course due to adrenergic response, with ensuing hypoten-
sion as intrapericardial pressures rise.
'l'he y descent of the
jugular venous pulse may be absent because passive filling of'
the ventricles is impeded by the intrapericardial pressure. This
finding may be difficult to appreciate, especially in patients
with tachycardia. Pulsus paradoxus represents exaggerated ven
tricular interdependence and is a key clinical feature ofcardiac
tamponade. It is characterized by a tall in systolic pressure o1
greater than 10 mm Hg during inspiration. Pulsus paradoxus is
not specific for tamponade and must be interpreted in conjunc-
tion with other clinicai and echocardiographic findings.
The ECG in patients with tamponade may demonstrate
sinus tachycardia, electrical alternans (related to a swinging
motion of the heart within the pericardial Iluid) (Figure 44), or
low voltage. If fluid has accumulated slowly, the cardiac sil
houette is typically enlarged on chest radiograph (Figure 45).
Echocardiography is an essential tool in the diagnosis of
cardiac tamponade because it defines the presence, distribu-
tion, and relative volume of pericarditrl fluid (Figure 46). Early
diastolic collapse ofthe right ventricle, late diastolic collapse of'
the right atrium, and abnormal interventricular septal motion
are echocardiographic I'eatures associated with cardiac tam
ponade. Doppler evaluation may additionally demonstrate a
decrease in mitral intlow velocity of more than 25'1, with
FIGURE 45. Chest radiograph demonstrating an enlarged cardiacsilhouette
("water bottle heart") in cardiac tamponade.
1
tt ltt
I
iln
.l_,l^)l-, _l-,
I
^l^,
l-,Jl^
t-I
^
l_
"1"
^l^
87

100. Pericardial Disease
cardiac tamponade lrightpanel). LA= left atrium; LV= left ventricle; RA = right atrium; RV= right ventricle
t
I
n
I
i
I
l
i
I
I
1
i
1
I'
I
I
;
i
inspiration, which is the echocardiographic equivalent of
pulsus paradoxus (Figure 47).
Cardiac catheterization is rarely necessary for diagnosis.
Hemodynamic hallmarks of tamponade include blunting or
loss of the
U descent within the right atrial pressure waveform
and elevated and equalized diastolic pressures. The latter reflects
the transmilted effect of the intrapericardial pressure. Invasive
arterial pressure recordings also show pulsus paradoxus.
Management
Cardiac tamponade is life-threatening, and urgent pericar
dial drainage is required after diagnostic confirmation.
Fluid removal can be accomplished r,r,ith pericardiocentesis
or a surgical subxiphoid approach. Pericardiocentesis is
often performed using echocardiographic or fluoroscopic
guidance. Surgical therapy is indicated to drain fluid when
pericardiocentesis cannot be performed safely, to obtain
pericardial tissue for diagnostic purposes, or to prevent
recurrent pericardial effusion by creating a pericardial u'in-
dow (often used in cases of malignant pericardial effusion).
In hemodynamically unstable patients, intravenous normal
saline is used to stabilize the patient as a temporizing meas
ure or bridge to definitive therapy.
After pericardial drainage, hemodynamic and clinical
evaluation may occasionally disclose findings of underlying
pericardial constriction, termed eflusive constrictive pericar-
ditis. If clinical evaluation and imaging studies (such as CMR
imaging) suggest an active inflammatory process. medical
therapy for acute pericarditis may be considered. with surgery
reserved for refractory cases.
I(EY POITIS
o Clinical features of cardiac tamponade include tachy-
cardia, hypotension, muffled heart sounds, elevated
central venous pressure, and pulsus paradoxus.
o Cardiac tamponade requires urgent drainage of pericar
dial fluid with either pericardiocentesis or surgery.
Constrictive Perica rditis
Clinical Presentation and Evaluation
Constrictive pericarditis is characterized by pericardial thick
ening, fibrosis. and sometimes calcification that impair dia
stolic filling and limit total cardiac volume. Within developed
countries, most cases are idiopathic. Other causes include
cardiac surgery chest irradiation, autoimmune disease. and
tuberculosis or other bacterial inf'ection. Tuberculosis remains
a major cause of constrictive pericarditis in developing
countries.
Constrictive pericarditis most commonly presents as indo
lent progression of right-sided heart failure symptoms. includ
ing peripheral edema and abdominal sr.relling (see Table 32).
Exertional dyspnea and fatigue are caused by increased dia-
stolic pressures and limited ability to augment cardiac output
due to the fixed stroke volume.
'l
I
,
l
I
I
i
I
i
1
't
I
i
I
)
FIGURE 47. Pulsed-waveDopplerevaluationofmitralinflowvelocity.Achangein
the flow between expiration and inspiration of greater than 250/o is present, consistent
with enhanced ventricular interdependence.
a
t
1?A
80
l0
Ittspiration Expiration
0
5
10
15
?0
?5
FR 29Hz
20
.t i!
I I l'*,,* lu ,[
li l,p l$ 't, i* I *
- {u
,-4,
'r,
,t ,l l-i'l
r..r flr, l?
dt.r..l I I
r& lr tr l[ Il;-""
88

101. Pericardial Disease
On physical examination, the central venous pressure is
elevated in nearly all patients, with prominent x and y
descents. The height of the waveform does not fall or may
increase during inspiration (Kussmaul sign), reflecting the
fixed diastolic volume of the right heart. Early diastolic filling
is unimpaired or even accentuated and is followed by sudden
cessation when total acceptable volume is met, resulting in a
high-frequency early diastolic sound (the pericardial knock).
Characteristics that may be used to differentiate a pericardial
knock from other diastolic sounds are listed in Table 33. Pulsus
paradoxus is less frequent in constrictive pericarditis than in
cardiac tamponade. Peripheral edema, ascites, hepatomegaly,
and pleural effusions are common. Muscle wasting may be
evident in advanced cases.
An ECG is obtained as part of the initial evaluation and
may reveal low voltage; however, no ECG findings are specific
to constrictive pericarditis.
Diagnosis of constrictive pericarditis is made with imaging
studies and hemodynamic evaluation. Transthoracic echocar-
diography reveals normal right and left ventricular size and
systolic function despite prominent symptoms and findings
suggestive of heart failure. Dilatation ofthe inferior vena cava
reflects elevated right-sided filling (right atrial) pressure. Dop-
pler echocardiography and tissue Doppler velocity are required
to differentiate constrictive pericarditis from restrictive cardio-
myopathy (see Myocardial Disease). Myocardial relaxation is
unimpaired or even enhanced in constrictive pericarditis, in
which early diastolic filling is rapid and unimpeded, whereas
relaxation is impaired in restrictive cardiomyopathy.
Chest radiography or CT in constrictive pericarditis may
demonstrate partial or circumferential pericardial calcification
(Figure aS), and pericardial thickening (>3 mm) may be visible
on CT or CMR imaging. Importantly, constriction may exist in
the absence of these findings. In one case series, hemody-
namically proven constrictive pericarditis occurred with nor
mal pericardial thickness in 18% of cases. CMR imaging also
may demonstrate an inspiratory septal shift, a sign of ventricu-
lar interdependence.
Cardiac catheterization can be performed when con
strictive pericarditis is suspected but not confirmed by
echocardiography. Invasive hemodynamic findings of con-
strictive pericarditis include a prominent y descent in the
right atrial waveform, which corresponds with the dip of
the right ventricular dip and plateau waveform (square
root sign). The significant
U descent and the right ventricu
lar dip both represent unimpeded or rapid early diastolic
ventricular filling. As inflow volume reaches the fixed
Opening snap
Pericardial
knock
Tumor plop
High frequency
Heard best at the left lower
sternal border and the apex
Associated with a loud S1, a
loud P2 with associated
pulmonary hypertension,
and a low-pitched diastolic
rumble at the apex
High frequency
Heard throughout the
precordium
Earlierthan an 53
Associated with deep x and
y descents in the jugular
venous pulse waveform
Low frequency
Heard best at the apex
May be positional
Low frequency
Heard best at the apex and
the left lateral decubitus
position
Displaced point of maximal
impulse
s3
Mitral
stenosis
Constrictive
pericarditis
Atrial
myxoma
Heart failure
circumferential pericardial calcification (arorvs).
Diastolic
Heart Sound
Condition Characteristics
Heart Sounds
TABLE 33. Characteristics of the Selected Diastolic
89

102. Adult Congenital Heart Disease
pericardial constraint, pressure rises rapidly until maxi-
mum volume is achieved; pressure then remains constant,
causing the plateau phase of the square root sign. A more
specific finding is ventricular interdependence during
simultaneous right and left ventricular systolic pressure
measurement. During inspiration, right ventricular inflow
is enhanced, and right ventricular systolic pressure rises;
however, these changes occur with a concomitant decrease
in left ventricular filling and reduction in left ventricular
stroke volume and systolic pressure (Figure 49). The con
verse is seen during expiration.
Pericardial thickening and impaired distensibility may
occur without librosis or calcitication in the setting of acute or
subacute inflammation. In these patients, constriction may be
transient and resolve spontaneously. Patients with transient
constrictive pericarditis present most commonly with symp
toms olright sided heart failure, although fever and chest pain
may indicate active inflammation. Most cases are idiopathic;
other causes include recent cardiac surgery. acute pericarditis,
autoinlmune disease, and chemotherapy. Systemic inflamma
tory markers (erythrocyte sedimentation rate and CRP) may
be elevated in transient constriction but are generally normal
with fixed constriction. Echocardiographic features are similar
F I G U R E 4 9. Ihe hemodynamics of constrictive pericarditis (top panel) versus
restrictive cardiomyopathy (boftom panel\. ln constrictive pericarditis, there is
significant enhancement of ventricular interdependence leading to discordance of
the left ventricular and right ventricular pressures during respiration. Arrows
indicate onset of inspiration and subsequent respective changes in left ventricular
and right ventricular systolic pressures. ln the top panel, note the diastolic dip and
plateau (arowhead), or "square root sign," characteristic of constrictive pericarditis.
ln restrictive cardiomyopathy, there is evidence of early rapid ventricular filling, but
the ventricular pressures concordantly rise and fall during respiration. LV= left
ventricle; RV = right ventricle.
to those of fixed constriction, although pericardial effusion is
more likely in patients with transient constrictive pericarditis.
Management
Treatment of transient constrictive pericarditis is the same
as for acute pericarditis. Anti-inflammatory therapy for 2 to
3 months is reasonable in hemodynamically stable patients
before recommending surgical pericardiectom)'. Diuretics
improve symptoms by reducing volume overload and cardiac
filling pressures but may have only mrtdest benefit. Response
to therapy is monitored clinically. echocardiographically. and.
if initial inflammatory markers are elevated. serologicallli
Patients with chronic pericardial constriction should be
referred fbr surgical pericardial stripping (pericardiectomy
performed via median sternotomy). In advanced cases. ade
quate resection of the pericardium may be difficult. leading to
incomplete resolution of symptoms. Diuretic therapy may be
useful to relieve congestive symptoms in patients who are
not deemed surgical candidates or in whom stripping r'r'as
incomplete.
f,EY POII'TS
. Constrictive pericarditis is characterized by pericardial
thickening, fibrosis, and sometimes calcification that
impair diastolic filling and limit total cardiac volume.
o Transthoracic echocardiography is the initial diagnostic
test for evaluating constrictive pericarditis; however,
additional imaging may be required to diflerentiate con
strictive pericarditis from restrictive cardiomyopathy.
. In patients with constrictive pericarditis, anti inflammatory
therapy for 2 to 3 months is reasonable before consider-
ing surgical pericardiectomy.
Adult Congenital
Heart Disease
lntroduction
Medical and surgical advances have resulted in more adults
than children with congenital heart disease (CHD) in North
America. Cardiovascular residua are common in patients with
previous intervention for congenital cardiac lesions, under
scoring the importance of periodic follow up by a team ide
ally consisting of the internist and a cardiologist trained in
adult CHD. Specialized care is critical fbr patients born with
complex and cyanotic congenital cardiac disease, sympto-
matic patients, and patients who desire pregnancy. The lre
quency offollow up depends on the underlying disorder and
patient's status.
Women with CHD should be offered reproductile health
counseling. The use of contraceptive agents in women with
CHD must be balanced against the risks of pregnancy; how
ever, there are no safety data on the various contraceptives to
lnspiration +
I
lnspintion +
90

103. help guide choice. l.strogen containing contraceptives may
pose a risk in women with CHD who are already at high risk
lbr venous thromboenrbolic diserse, including patients with
cyanosis, Fontan physiologr, mechanical valves, prior throm
botic events, and pulmonary arterial hypertension. In preg
nant patients, anticoagulation is associated witl-r r-naternal and
fetal risks. Prepregnancy counseling is recommended lbr all
wonlen requiring long term anticoagulation to enable them to
make infbrn-red decisions and to understand the risks (see
Pregnancy and Cardiovascular Disease).
Adults with CHD are at risk lbr hepatitis C, and screening
is recornnrended. Hepatitis B vaccination is recontmended lbr
all nonimmune patients at high risk for infection, includir.rg
patients with reprrired CHD.
Anxiety ar-rd depression are prevalent but underrecog
nized in patients with CHD. and screening tbr these mood
disorders should be a routine aspect ol care.
Patent Foramen Ovale
'l'l-re
firramen ovale is a passage in the superior portion ol the
lbssa ovalis that allows oxygenated placental blood to transfer
to the fetal circulation. It norr.nally closes within the lirst
rteeks ol life; however. ir-r 25',1, to 30',1, of the population, it
renrains patent (Figure 50). A patent fbramen ovale (PFO) is
usually tbund incidentally on echocardiography or during
evaluation fbr a cerebrovascular event.
A PFO is typically diagnosed by transesopl.rageal echocar
diography (TEE). less commclnly by transthoracic echocardi
ography (TTE). Right to-left shunting of blood across the PFO
is demonstrated by color flow Doppler imagir-rg or by intrave
nously ir.rjecting agitated saline and identifying its subsequent
transfbr througl.r the PFO from the right atriur.n to the left
atriunr.
Patients with a PFO and embolic stroke ol undetermined
source should be treated with antiplatelet therapy. In patients
younger than 60 years with a PFO and embolic irppearing
infarct and no other mechanism of stroke identified. current
guidelines recommend PFO ckrsure following a discussion of
potential benefits (absolute recurrent stroke risk reduction of
3.4')(, at 5 years) and risks (periprocedural conrplication rate
oI' :1.9"1, and increased absolute rate of non periprocedural
RA
RV
F I G U R E 5 0. Patent foramen ovale. The arrows demonstrate the mechanism of
right to left shunting through the patent foramen ovale. LA- lelt atrium; LV= left
ventricle; RA = right atrium; RV = right ventricle.
Redrawn from origina supplled counesy 0i Dr. Wi llam D tdwards, Department 0f Labordtory lVedicine afd
Pathology, [,4ayo Cl.i., Roihester, [,4N
Adult Congenital Heart Disease
atrial flbrillation of0.33'/, peryear). Data regarding the benefit
of PFO closure in patients older tharr 60 years are lacking. For
these patients and fbr younger patients who do not select PFO
closure, antiplatelet therapy or anticoagulation is reasonable.
Limited data support PFO closure in an ef'fort to decrease the
frequency of migraine. No treatment or fbilow-up is needed in
asymptomatic patients with an incidentatly detected PFO.
Platypnea-orthodeoxia syndrome is a rare acquired disor-
der characterized by cyanosis and dyspnea in the upright posi
tion resulting from right to lelt shunting across a PFO or, less
commonly. through an atrial septal defect (ASD) or pulmonary
arteriovenous fistulae. A transient increase in right atrial pres-
sure or change in right atrial anatomy resulting from myocar-
dial inlarction, pulmonary embolisnr, tricuspid regurgitation,
or acute right sided heart failure n.ray precipitate this syn
drome. Device closure of the PFO may relieve symptoms and
improve oxygen saturation.
Atrial septal aneurysm is characterized by mobile, redun
dant atrial septal tissue that is often associated with a PFO.
Atrial septal aneurysm with a PFO reportedly increases the
risk lbr stroke compared with PFO alone. Results of a ran-
domized, open label trial demonstrated that patients present-
ing with en.rbolic stroke of undetermined source in the setting
of an atrial septal aneurysm with PFO had a lower rate of
stroke recurrence when treated with PFO closure combined
with antiplatelet therapy than with antiplatelet therapy alone.
Rarely, surgical excision and defect closure is considered based
on anatomic features.
IEY POIlITS
o No treatment or follow-up is needed in asymptomatic HVC
patients with a patent foramen ovale.
. In patients younger than 60 years with a patent foramen
ovale (PFO) and embolic stroke of undetermined source,
PFO closure is recommended following a discussion of
potential benefits and risks.
Atrial Septal Defect
Pathophysiology and Genetics
An ASD is a defect in the atrial septum resulting in a left to-
right shunt with eventual right-sided cardiac chamber dilata
tion in most patients. ASDs are generally classified by their
krcation (Figure 51). Ostium secundum defects, the most
common type of ASD (75'1, of cases), are typically located in
the mid portion of the atrial septum and are usually isolated
anomalies. Located in the lowest portion of the atrial septum,
ostium primum defects (15,1,-20"/. of ASDs) are a component
of endocardial cushion defects. Associated lesions include
mitral valve, ventricular septum, and subaortic anomalies.
Sinus venosus defects (5'u, 10'/,, o1'ASDs) are located near the
superior vena cava or, rarely, the inferior vena cava; anoma-
lous pulmonary venous connection (typically involving the
right upper pulmonary vein) occurs in essentially all patients
with this defect. A coronary sinus ASD (<1'l. of cases) is a
+
LA
LV
RA
LA
LV
91

104. Adult Congenital Heart Disease
F I G U R E 5 1 . Positions of various atrial septal defeds viewed from the right side
of the heart. (1) Ostium secundum; (2) ostium primum; (3) superior sinus venosus;
(4) i nferior si nus venosus. The coronary si n us atrial septa I defect is not visua I ized i n
this image; it is a communication between the coronary sinus and left atrium.
PI= pulm0nary trunk; RV= rightventricle.
Redrawn from original supplied courtesy of Dr. William D. Edwards, Department of Laboratory Medicine and
Pathology, Mayo Clinic, Rochesler, MN.
communication betvveen the left atrium and the coronary
sinus. These defects are commonly associated with a persis
tent left sided superior vena cava or complex CHD.
ASDs are rarely associated with genetic syndromes.
Familial ostium secundum ASDs may be autosomal dominant
or linked to chromosome 5. Congenital heart defects are rela
tively common in patients with Down syndrome; the most
frequent abnormalities reported are atrioventricular septal
defects, including ostium primum ASD.
Clinical Presentation
ASDs may be suspected in patients with unexplained right
heart enlargement or atrial arrhythmias. Atrial fibrillation is a
common finding, particularly in older patients with an ASD.
Atrial fibrillation risk decreases but does not normalize after
ASD closure. ASD size and associated defects influence the age
of presentation. Symptoms include fatigue, exertional dyspnea,
arrhlthmias, and paradoxical embolism. Rarely, patients with
pulmonary hypertension (PH) are found to have isolated ASDs.
Examination findings in patients with an ASD include a
parasternal impulse, fixed splitting of the Sr, and a pulmonary
outflow murrnur. A diastolic flow rumble across the tricuspid
valve can occur with a large left to right shunt.
Diagnostic Evaluation
ECG and radiographic findings in patients with an ASD are
presented in Table 34.
TTE is the preferred imaging modality for identification of
ostium secundum and primum ASDs. TTE also measures asso-
ciated features, such as right-sided cardiac chamber enlarge-
ment, tricuspid regurgitation related to annular dilatation, and
right ventricular systolic pressure elevation. Agitated saline
contrast injection in a peripheral vein during TTE may help
identify an atrial level shunt. Sinus venosus and coronary
sinus ASDs are less readily diagnosed by TTE in adults and
often require other imaging modalities, such as TEE, cardiac
magnetic resonance (CMR) imaging, or CT. CMR imaging and
CT are rarely used as the primary imaging modality when an
ASD is suspected but can identify anomalous pulmonary
veins and quantify right ventricular volume and ejection
fraction.
Cardiac catheterization is the only method for accurately
calculating pulmonary-to systemic blood flow ratio (Qp:Qs).
It may be recommended in patients with an ASD and PH when
ASD closure is being considered.
Treatment
In asymptomatic patients with a small ASD and no right heart
enlargement, periodic clinical monitoring and echocardio
graphic imaging are recommended. The main indications for
ASD closure include right-sided cardiac chamber enlargement
and dyspnea. Some experts recommend closure for platypnea
orthodeoxia syndrome.
Percutaneous device closure is indicated for patients with
an isolated ostium secundum ASD causing functional and
hemodynamic consequences. It is a reasonable option for
asymptomatic patients with shunt related hemodynamic con
sequences in the absence of severe PH. Surgical ASD closure is
indicated for nonsecundum ASDs, large secundum ASDs,
unfavorable anatomy for device closure, and coexistent cardio
vascular disease that requires operative intervention, such as
coronary artery disease, partial anomalous pulmonary venous
return, or tricuspid regurgitation.
Patients with an ASD and PH require specialized care;
ASD closure may be considered for persistent left-to right
shunting without fixed PH. Medical therapy targeted at PH
should also be considered.
Patients with an isolated anomalous pulmonary venous
connection may present with clinical findings and TTE fea
tures similar to an ASD. Surgical redirection of the pulmonary
vein is the only feasible treatment and requires surgical exper
tise in CHD.
Activity restriction is not required for patients lvith small
ASDs. Large left-to-right shunts result in self-limited exercise
restriction. Patients with severe PH are advised to avoid iso
metric or competitive exercise.
Pregnancy is generally well tolerated in patients with an
ASD in the absence of PH. The risk for CHD transmission with
a sporadic ASD is estimated to be around 5')6. Other genetic
syndromes have variable inheritance, and genetic counseling
is suggested if a syndrome is suspected.
Follow-up After Atrial Septal Defect Closure
Follow up after surgical or percutaneous ASD closure includes
TTE and clinical assessment within the first year after closure
92

105. Adult Congenital Heart Disease
ECG and CXR Findings Late ComplicationsLesion
TABLE 34. lmaging Findings and Late Complications in Adult Congenital Heart Disease
Patent foramen ovale
Ostium secundum ASD
Ostium primum ASD
Sinus venosus ASD
SmallVSD
Large VSD
Small PDA
Pulmonary valve stenosis
Aortic coarctation
Repaired tetralogy of Fallot
Eisenmenger syndrome
Large PDA
Normal
ECG: lncomplete RBBB, RA enlargement, right axis
deviation
CXR: Right heart enlargement, prominent
pulmonary artery, increased pulmonary vascularity
ECG: Left axis deviation, first-degree
atrioventricular block
CXR: Right heart enlargement, prominent
pulmonary artery, increased pulmonary vascularity
ECG: Abnormal P-wave axis
CXR: Right heart enlargement, prominent
pulmonary a rtery, increased pulmonary vascularity
Normal
ECG: RV or RV/LV hypertrophy
CXR: RA and RV enlargement, increased
pulmonary vascular markings; with PH: prominent
centra I pulmonary arteries, red uced periphera I
pulmonary vascular markings
Normal
ECG: LA enlargement, LV hypertrophy; with PH: RV
hypertrophy
CXR: Cardiomegaly, increased pulmonary vascular
markings, calcification of PDA (occasional); with
PH: prominent central pulmonary arteries, reduced
peripheral pulmonary vascular markings
ECG: lf RV systolic pressure >60 mm Hg: RA
enlargement, right axis deviation, RV hypertrophy
CXR: Pulmonary artery dilatation, calcification of
pulmonary valve (rare); RA enlargement may be
noted
ECG: LV hypertrophy and ST-T wave abnormalities
CXR: Dilated ascending aorta, "figure 3 sign"
beneath aortic arch, rib notching from collateral
vessels
ECG: RBBB, increased ORS duration (QRS duration
reflects degree of RV dilatation)
CXR: Cardiomegaly with pulmonary or tricuspid
valve regurgitation; right aortic arch in 25o/" of
ECG: Right axis deviation, RA enlargement, RV
hypertrophy
CXR: RV dilatation, prominent pulmonary artery,
reduced pulmonary vascularity
Paradoxical embolism, platypnea-orthodeoxia
syndrome
Right heart enlargement, AF, PH (rare)
Post-repair: residual shunt (rare)
Right heart enlargement, AF, mitral regurgitation
(from mitralvalve cleft), PH (rare)
Post-repair: residual shunt (rare), mitral
regurgitation (from mitral valve cleft), LV outflow
tract obstruction
Right heart enlargement, AF, PH (rare)
Post-repair: residual shunt (rare), residual
anomalous pulmonary venous connection
Endocarditis
PH with associated RA and RV enlargement, RV
hypertrophy, Eisenmenger syndrome
Post-repair: residua I VSD, residual shu nt (rare)
Endocarditis
Endocarditis, right-sided heart failure, PH
Eisenmenger syndrome
Post-repair: residual shunt (rare)
Post-repa i r: Severe pu I mona
ry
va lve re g u rg itation
after pulmonary valvotomy or valvuloplasty
Hypertension (75% of cases), bicuspid aortic valve
(>50% of cases), increased risk for aortic aneurysm
(ascending or at repair site) and intracranial aneurysm
Post-repair: Recoa rctation, hypertension, aortic
aneurysm
Post-repair: I ncreased atrial and ventricu lar
arrhythmia risk, pulmonary valve regurgitation or
stenosis, tricuspid regurgitation
ORS >180 ms increases risk for ventricular
tachycardia and sudden death
Right-sided hea rt failure, hemoptysis, stroke
KtY POIilTS
o Clinical findings in patients with an atrial septal defect
include a parasternal impulse, fixed splitting of the 52,
and a pulmonary outflow murrnur.
(Continued)
AF=atrialfibrillation;ASD=atrialseptaldefect;CXR=chestradiography;LA= eftatrial;LV=leftventricular;PDA=patentductusareriosus;PH=pulmonaryhyperension;
RA = right atrial; RBBB = right bundle branch block; RV = right ventricular; VSD = ventricular septal defect.
and then periodically afterward. Endocarditis prophylaxis is
indicated for 6 months alter closure. Atrial fibrillation risk
persists after closure. and frequency increases related to age at
the time of ASD closure. Rare complications after device clo-
sure include device migration, erosion into the pericardium or
aorta, and sudden death.
93

106. \
Adult Congenital Heart Disease
XtY P0ltIS (ominued)
r The main indications for atrial septal defect (ASD) clo
sure include right-sided cardiac chamber enlargement
and dyspnea; asymptomatic, small ASDs without right
heart enlargement can be monitored with clinical and
echocardiographic surveillance.
Ventricular Septal Defect
Pathophysiology
Ventricular septal defects (VSDs) are delined by their loca
tion on the ventricular septum (Figure 52). Membranous
VSDs are most common (80'X, of cases) and are usually iso
lated abnormalities. Muscular VSDs (10'/, of cases) can be
located anltvhere in the ventricular septum and often close
spontaneously. Subpulmonary VSDs (also called outlet or
supracristal VSDs) account for approxir.r.rately 6'X, of defects
in non Asian persons and 33'1, in Asian persons and are
associated with aortic regurgitation caused by aortic cusp
distortion. Inlet VSDs (4'X, of cases) occur in the superior
posterior portion of the ventricular septum adjacent to the
tricuspid valve. They occur as part of the atrioventricular
septal defect complex, characteristically seen in patients
with Down syndrome.
VSDs are common at birth. but many small VSDs close
spontaneclusly, resulting in lower prevalence by adulthood.
Clinical Presentation
The presentation ofan isolated VSD depends on the VSD size
and pulmonary vascular resistance. A snrall VSD without PH
presents with a loud (otten palpable) holosystolic nlurmur
FIGURE 52. Positionsofvariousventricularseptaldefectsviewedfromtheleft
side of the heart. (1
) Membranous; (2) subpulmonary; (3) muscular; (4) inlet.
Ao = aorta; LA = left atrium.
Redrawn irom origina supp ied coudesy of Dr Willianr D Edwardg, Depa(ment of Laboratory Medic ne and
Pathology, [,'layo Clinic, Rochester, lN.
located at the left sternal border that obliterates Sr. Small VSDs
do not cause lelt heart enlargement or PH.
A VSD u'ith a moderate left to right shunt may cause left
ventricular (LV) volume overload and PH. Patients are asymp
tomatic for many years but eventually present u'ith heart fail
ure s)'mptoms. A displaced LV apical impulse suggests rolume
overload. A holosystolic murmur at the left sternal border is
noted; the pressure gradient between the ventricles deter
mines the murmur quality and duration. Progressive PH
results in reduced murmur ir.rtensity.
VSDs associatecl with large left to right shunts usually
are detected by the presence of a murmur, heart failure. and
failure to thrive in infanclr Failure to close the defect early in
life usually causes flxed PH within several years with subse-
quent development ol Eisennrenger syndrome (see later dis
cussion) and shunt reversal.
Diagnostic Evaluation
ECG and radiographic tindings in patients \'ith a VSD are pre
sented in Table 34.
TTE is the imagir.rg modality of choice for identilication
of VSD location, size. and hemodynamic impact. Rarely. TEE.
CMR imaging. or CT is needed to delineate cardiac anatomy
when T'fE is unsatisfactory Cardiac catheterization is primar
ily performed to delineate the Qp:Qs ratio and pulmonary
pressures in select patients.
Treatment
VSD closure is indicated in the absence of PH when the Qp:Qs
ratio is 1.5 or greater with evidence clf LV volume overload.
Most patients are treated surgicallyr but percutaneous device
closure is an option lbr select VSDs.
VSD closure is not indicated for patients with a small left
to right shunt and no chamber enlargement or valve disease.
but periodic clinical evaluation and imaging are recom
ntended. Large VSDs lvith shunt reversal (right to lett shunt
ing) and PH (Eisenmenger syndrome) should not be closed
because this causes clir-rical deterioration owing to reduced
cardiac output.
Patients with small VSDs do nclt require activity restric
tions. If the pulmonary artery pressure is greater than 50')1, ot'
systolic blood pressure. isometric or competitire exercise is
discouraged.
Pregnancy in women with VSDs is generally lrell tolerated
in the absence of P[{: women with VSDs and associated fixed
PH should be counseled to avoid pregnanc,'.
Follow-up After Ventricular Septal Defect Closure
Residual clr recurrent VSD. arrhythmias. PH. endocarditis.
and valve regurgitation are recognized complications fbllolv
ing VSD closure. Endocarditis prophylaxis should be pro
vided for 6 months lbllorving closure. Clinical assessment
and TTE are recommended within 1 year of VSD closure.
Subsequent fbllow up frequency depends on clinical and
cardiac status.
:
94

107. XEY POIXTS
. A small ventricular septal defect without pulmonary
hypertension presents with a loud holosystolic murmur
located at the left sternal border that obliterates Sr.
e Ventricular septal defect closure is indicated when the
pulmonary to systemic blood flow ratio is 1.5 or greater
with evidence of left ventricular volume overload.
Patent Ductus Arteriosus
Pathophysiology
A patent ductus arteriosus (PDA) is a persistellt fetal connection
between the aorta and the left pulmonary artery. It may be an
isolated abnormality or associated with other congenital cardiac
defects. Prematurity and maternal ruhella predispose to a PDA.
Clinical Presentation
The typical murmur of a PDA is a continuous "machinery"
murmur that envelops the Sr, making it inaudible; the mur
mur is heard beneath the left clavicle. A tiny PDA is generally
asymptomatic and inaudible. Patients with a moderate sized
PDA may present with bounding pulses, wide arterial pulse
pressure, left heart enlargement and dysfunction, and, rarely,
clinical heart failure. A large unrepaired PDA may c:ruse PH
with eventual shunt reversal (Eisenmenger syndrome); char
acteristic features of an Eisenmenger PDA are clubbing and
oxygen desaturation afl'ecting the feet but not the hands,
owing to desaturated blood reaching the lower extremities
preferentially (di lferential cyanosis).
Diagnostic Evaluation
ECG and radiographic findings in patients with a PDA are pre-
sented in Table 34.
TTE is the imaging modality of choice for identification of
a PDA. The PDA may be difficult to visualize in patients with
severe PH owing to equalization of pressures between the
aorta and pulmonary artery. Intravenously injected agitated
saline may demonstrate the shunt trom the pulmonary artery
to the descending thoracic aorta via the PDA. [n patients with
a PDA and PH, cardiac catheterization is used to determine
shunt size and reversibility of PH. Angiography confirms PDA
morphologz and helps determine rvhether percutaneous clo
sure is feasible. TEE, CT, and CMR imaging may identify a PDA
but are not the primary diagnostic techniques.
Treatment
PDA closure is indicated for left sided carcliac chamber
enlargement as long as pulmonary artery systolic pressure is
less than 50'/. systemic. Percutaneous closure is usually per
formed; referral to a congenital cardiac center for considera
tion of closure options is recommended.
Patients with a small PDA u'ithout PH do not require
physical activity restrictions, and women should be able to
tolerate pregnancy.
Adult Congenital Heart Disease
rtY P0rtrs
. A continuous "machinery" murmur heard beneath the
left clavicle that makes the 52 inaudible is typical for a
patent dttctus arteriosus.
. Patent ductus arteriosus closure is indicated for left
sided cardiac chamber enlargement as long as pulmo
nary artery systolic pressure is less than 50'/. systemic.
Pulmonary Stenosis
Pathophysiology
Pulmonary stenosis (PS) causes obstruction to right ventricu
lar outflow and is usually an isolated valve lesion. Isolated PS
is associated with Noonan syndrome, an autosomal dominant
disorcler characterized by short stature, variable intellectual
capacity, neck webbing, and ocular hypertekrrism (abnormally
increased clistance befween the orbits).
Clinical Presentation
Mild and moderate PS is generally asymptomatic. On physical
examination, mild PS is associated with a normal jugular
venous wavefbrm and precordial impulse.
Severe PS can cause exertional dyspnea. Right ventricular
hypertrophy caused by pressure overload results in a promi
nent o wave on the jugular venous waveform and a palpable
right ventricular lift.
Auscultatory findings in PS include an early systolic
ejection click, which is the only right sided heart sound to
decrease during inspiration, followed by a crescendo
decrescendo murmur. In severe PS, the systolic ejection
murmur at the lef t sternal border increases in intensity and
duration, and the pulmonary component of S, is delayed
(causing a split S,) and eventually disappears. A right ven-
tricular S, is oflen heard in severe PS.
Diagnostic Evaluation
ECG and radiographic findings in patients with PS are pre-
sented in Table 34.
T1'E is the imaging modality of choice for identification of
PS. Severe PS is present with a peak gradient greater than
64 rnm Hg and mean gradient greater than 35 mm Hg.
Treatment options depend on valve mobility, calcification, and
the eftbcts ofobstruction on the right ventricle. PS causes right
ventricular hypertrophy rather than enlargement. Right ven
tricular dilatation should prompt a search fbr an associated
lesion, such as pulmonary regurgitation or an ASD.'lEE, CMR
imaging, and CT are not routinely used in patients with PS.
Cardiac catheterization is performed when percutaneous
intervention lbr PS is considered.
Treatment
Pulmonary balloon valvuloplasty is the pref'erred treatment
for valvular PS. lt is indicated for symptomatic patients with
appropriate valve morphology who have moderate or severe
95

108. Adult Congenital Heart Disease
valvular PS (moderate peak gradient, 36 64 mm Hg; severe
peak gradient, >64 mm Hg; mean gradient, >35 mm Hg) and
otherwise unexplained symptoms of heart failure, cyanosis
from interatrial right to left communication, and/or exercise
intolerance. Surgical intervention is recommended for PS
associated with a small annulus, more than moderate pulmo-
nary regurgitation, severe subvalvar or supravalvar PS, or
another cardiac lesion that requires operative intervention.
Patients with mild or moderate PS do not require exercise
restriction. Patients with severe PS should participate in low-
intensity sports only.
Pregnancy is generally well-tolerated with PS; percutane-
ous valvotomy has been performed during pregnancy for
severe symptomatic PS. Sporadic CHD recurrence in offspring
is rare. Noonan syndrome should be suspected with PS recur
rence in offspring.
Follow-up After Pulmonary Stenosis Repair
Patients with previous PS intervention (balloon or surgical) often
have severe pulmonary regurgitation; thus, long term clinical
and TTE follow up is recommended. The frequency of follow-up
depends on regurgitation severity and impact on the heart.
r(EY P0t 1{IS
o Auscultation findings in pulmonary stenosis include an
early systolic ejection click followed by a crescendo
decrescendo murmur.
o Pulmonary balloon valvuloplasty is the preferred treat-
ment for valvular pulmonary stenosis.
Aortic Coarctation
Pathophysiology
Aortic coarctation is a discrete narrowing of the aorta. usually
located just beyond the left subclavian artery causing hyper-
tension proximal and hypotension distal to the narrowing.
Clinical Presentation
Aortic coarctation should be suspected in all patients with
severe hypertension, especially young patients. Severe aortic
coarctation may present as heart failure early in life. Adults are
usually asymptomatic, but exertional leg fatigue or headaches
may occur. Upper extremity hypertension and reduced blood
pressure and pulse amplitude in the lower extremities cause a
radial artery-to femoral artery pulse delay. A systolic or con
tinuous murmur is heard in the left infraclavicular region or
over the back. A murmur from collateral intercostal vessels also
may be audible and palpable over the chest wall. Fifty percent
of patients with aortic coarctation have a bicuspid aortic valve.
Auscultation of the heart may reveal an ejection click, a systolic
murmur at the cardiac base, or, sometimes, an S..
Turner syndrome, a chromosomal abnormality secondary
to partial or total loss of chromosome X, is often associated
with coarctation and bicuspid aortic valve. Turner syndrome is
characterized by short stature, webbed neck, broad chest, and
widely spaced nipples. Aortic coarctation is also associated
with bicuspid aortic and subaortic stenosis. mitral valve
abnormalities (Shone syndrome), ventricular septal defect.
and cerebral aneurysms.
Diagnostic Evaluation
ECG and radiographic findings in patients with aortic coarcta
tion are presented in Table 34.
The characteristic radiographic features of aortic coarctation
include the "figure 3 sign" (Figurr 53), which is caused by dilata
tion ofthe aorta above and below the area ofcoarctation. Dilatation
of intercostal collateral arteries because of aortic obstruction may
lead to the radiographic appearance ofrib notching.
TTE is often the initial diagnostic test in patients sus-
pected of having aortic coarctation; it usually identifies the
coarctation and associated features, such as bicuspid aortic
valve and LV hypertrophy. CMR imaging and CT are recom
mended to identif,i the anatomy, severiry and location of the
coarctation; the presence of collateral vessels; and associated
abnormalities, such as aortic aneurysm. Cardiac catheteriza
tion is primarily used in patients in whom percutaneous inter
vention is being considered.
Treatment
Severe aortic coarctation is associated /ith excess morbidity
and mortality, including hypertension, coronary artery dis
ease, stroke, aortic dissection, and heart failure. Age at the
Fl G U RE 53. Chest radiograph of a patient with aortic coarctation exhibiting
the "figure 3 sign," caused by dilatation of the aorta above and below the area of
coarctation (blue anow), and rib notching (white arrowsl.
96

109. Adult Congenital Heart Disease
time of coarctation repair is the most important predictor of
long term survival.
Indications for intervention in patients with coarctation
include upper extremity hypertension and features of significant
aortic coarctation (upper exlremity/lower ertremi$z resting sys
tolic peak [peak to peakl pressure gradient >20 mm Hg or mean
Doppler gradient >20 mm Hg with normal LV systolic function;
upper extremity/lower extremigr gradient >10 mm Hg or mean
Doppler gradient >10 mm Hg plus either decreased LV systolic
function, aortic valve regurgitation, or collateral flow) and radio
logic (CT or CMR) evidence of severe coarctation with collateral
flow. The choice between percutaneous or surgical intervention
depends on the length, location, and severity of coarctation and
the presence of associated cardiovascular lesions.
Physical activity restriction is recommended for patients
with severe postintervention residual or unrepaired coarcta
tion, aoftic stenosis, or a dilated aorta; these patients should
avoid contact sports and isometric exercise.
A comprehensive preconception evaluation is warranted
in all patients with coarctation who are considering preg
nancy. Pregnancy is reasonable in women with repaired aortic
coarctation without significant residua. Women with mild or
moderate residua or unoperated coarctation will generally
tolerate pregnancy well but should undergo blood pressure
monitoring and cardiovascular evaluation during pregnancy.
Pregnancy should be avoided by patients with severe unre
paired coarctation.
Follow-up After Aortic Coarctation Repair
Following coarctation repair, hypertension occurs in up to 75% of
patients and should be treated. Additional intervention following
repair may be required for bicuspid aortic valve, aortic aneurysm
or dissection, recoarctation, coronary artery disease, systolic or
diastolic hearl failure, or intracranial aneurysm. Regular follow
up should include TTE, periodic aortic imaging by CT or CIr4R,
and evaluation by a cardiologist specializing in CHD.
KEY POIT{T5
o In patients with aortic coarctation, findings may include
upper extremit5z hypertension and reduced blood pressure
and pulse amplitude in the lower extremities, causing a
radial artery-to femoral artery pulse delay.
. The "figure 3 sign'is a characteristic radiographic feature
of aortic coarctation.
. Intervention is indicated in patients with upper extrem
ity hlpertension, features of significant aortic coarcta-
tion, and radiologic evidence of severe coarctation with
collateral flow.
Tetralogy of Fallot
Tetralogz of Fallot (TOF) is characterized by a large subaortic
VSD, infundibular and/or valvular PS, aorlic override, and
right ventricular hypertrophy (Figure 54). It is the most
FIGURE 54,TetralogyofFallot.Asubarterialventricularseptaldefect(asterlsk)
and subpulmonary and pulmonary valve stenosis (arrow) are associated with
secondary aortic override and right ventricular hypertroptry. Ao = aorta; LA= left
atrium; LV= leftventricle; RA= right atrium; RV= rightventricle.
Redrawn from origina supplied c0udesy 0f Dr. William D. Edwards, Departmert of Laboratory Medicine and
Pathology, [,4ayo Clinic, Rochester, l]N.
common cyanotic congenital cardiac lesion. Repair is usually
performed eariy in life; adults who have not undergone an
operation are rarely encountered in developed countries.
Genetic screening is reasonable for patients with
conotruncal defects for recognition of comorbidities and
counseling for risk of recurrence in offspring. Approximately
15% of patients with TOF have the22qll.2 chromosome micro-
deletion (DiGeorge syndrome); when present, CHD inherit
ance is approximately 50%, compared with 5% in unaffected
patients. TOF is common in persons with Down syndrome.
TOF repair involves VSD patch closure and relief of PS/
right ventricular outflow tract obstruction by transannular
patch placement; the transannuiar patch disrupts integrity of
the pulmonary valve, causing severe pulmonary regurgitation
(distended neck veins, loud Sr, diastolic murmur at the second
left intercostal space that increases with inspiration). Severe
long standing pulmonary regurgitation causes right heart
enlargement, tricuspid regurgitation, exercise limitation, and
both atrial and ventricular arrhy'thmias and is the most com-
mon reason for reoperation after TOF repair. The incidence of
sudden cardiac death after surgical repair of TOF is approxi-
mately 2"/n pe.r decade. Primary prevention of sudden cardiac
death with an implantable cardioverter defibrillator should be
considered in patients with ejection fraction of 35'/. or less and
New York Heart Association functional class II or III symp
toms. Annual congenital cardiology follow up is recom-
mended for patients with repaired TOF to determine optimal
timing for intervention.
97

110. Adult Congenital Heart Disease
Diagnostic Evaluation After Repair
of Tetralogy of Fallot
ECG and radiographic findings in patients with repaired TOF
are presented in Table 34.
Symptoms, arrhythmias, or right heart enlargement
should prompt a search for severe pulmonary regurgitation.
Prolongation of the QRS complex reflects the degree of right
ventricular dilatation;
QRS duration of 180 ms or longer and
nonsustained ventricular tachycardia are risk factors for sud
den cardiac death.
TTE is the imaging modality of choice for identifying valve
dysfunction, residual VSD, LV dysfunction, and aortic dilata
tion. CMR imaging or CT is preferred for assessment of right
ventricular size and function, which helps determine appro
priate timing for pulmonary valve replacement. Cardiac cath
eterization may be required to assess hemodynamics and
residual shunts and to delineate coronary artery and pulmo
nary artery anatomy.
Treatment of Tetralogy of Fallot Residua
Indications for pulmonary valve replacement in patients r,tith
repaired TOF and severe pulmonary regurgitation include
symptoms, decreased exercise tolerance, more than moderate
right heart enlargement or mild or moderate right or left heart
dysfunction, arrhythmias, and development of tricuspid regur-
gitation. Tricuspid valve repair may also be needed. Pulmonary
valve replacement is reasonable in asymptomatic patients with
repaired TOF and ventricular enlargement or dysfunction and
moderate or greater pulmonary regurgitation. Percutaneous
pulmonaryvalve replacement is possible in select patients with
previous TOF surgery and a native pulmonary valve.
Physical activity restriction is recommended for patients
with repaired TOF and residual sequelae; contact sports and
healy isometric exercise should be avoided.
t([Y POtXTS
o Genetic screening is recommended for all patients with
tetralogr of Fallot who are considering pregnancy
because the presence of the 22q11.2 chromosome
microdeletion (15'/" of patients) results in congenital
heart disease inheritance of approximately 507,.
o Indications for pulmonary valve replacement in patients
with repaired tetralogr of Fallot and severe pulmonary
regurgitation include syrnptoms, decreased exercise tol
erance, more than moderate right heart enlargement.
mild or moderate right or left heart dysfunction, arrhyth-
mias, and development of tricuspid regurgitation.
Adults With Cyanotic Congenital
Heart Disease
General Management
Right to left cardiac shunts, such as palliated or unrepaired TOF
and Eisenmenger syndrome, result in hypoxemia, erythrocltosis,
and cyanosis. Physical leatures include digital clubbing and
central cyanosis. Patients are predisposed to arthropathll gout.
scoliosis, gallstones. pulmonary hemorrhage or thrombus.
paradoxical cerebral emboli or abscess. kidnel, d1'sfunction.
hemostatic problems, and pheochromocvtoma paragangli
oma. Patients with cyanotic CI ID should be evaluated annualll,
by a congenital cardiac specialist.
Perioperative complications are common in patients u'ith
cyanosis: thus. elective procedures and operations should be
performed at specialized multidisciplinary care centers. A con
genital cardiac specialist should be consulted rt hen patients are
hospitalized. and anesthesia should be administered b1'provid
ers with expertise in anesthetic management of CHD. Addi
tional considerations include endocarditis prophl'laxis ftrr
nonsterile procedures: placement of intravenous line filters to
prevent paradoxical air embolism; and early ambulation.
pneunratic compression devices. and anticoagulation to pre
vent venous thrombosis and paradorical enrbolism. Venous
thromboembolism prophylaxis is especially important in these
patients because ofthe risk for paradoxical embolism ifvenous
thromboembolism were to occur.
Most patients with cyanosis have compensated, stable
erythrocytosis to improve oxygen transport. Phlebotomy is
recommended for patients with symptomatic hypen'iscosity
(headaches. reduced concentration) with a hemoglobin level
greater than 20 gidl (200
Bi L) and hematocrit greater than
65'/. in the absence of dehydration. Phlebotomy should be
performed no more than three times each year and should be
follor.r,ed by fluid administration. Repeated phlebotomies
deplete iron stores, with increased risk for stroke as a possible
consequence of iron deficiency. Iron therapy is indicated in
cases of deflciency.
Maternal and fetal morbidity and mortality increase
related to the degree of cyanosis and pulmonary pressures.
and all pregnancy in women with cyanosis is considered high
risk. Reproductive counseling is recommended.
Eisenmenger Syndrome
Eisennrenger syndrome is severe PH with cardiac shunt rever
sal (right to left shunting) caused by long standing. unre
paired VSD. PDA. or ASD or another lesion. TTE evah.ration and
appropriate intervention has decreased the frequency of'
Eisenmenger syndrome, but PH related to complex CHD is
increasingly identifi ed.
Consenative medical measures fbr patients with Eisen
menger syndrome include avoiding iron deficiency. dehldration.
acute exposure to excess heat, and moderate or severe strenuous
or isometric exercise. Phlebotomy is rarely performed. Long
term altitude exposure should be avoided or limited because it
results in a reduced partial pressure of orlgen. Air trarel should
be undertaken in pressurized aircrafts; supplemental oxygen
may be beneflcial with prolonged air trarel.
All patients',tith Eisenmenger syndrome should undergo
annual evaluation by a congenital cardiac specialist. Noncardiac
surgery should be performed at centers with experts in
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98

111. complex CHD. Patients with progressive cardiovascular symp
toms may benefit from pulmonary vasodilator therapy or. in
rare cases, heart and lung transplantation.
Women with Eisenmenger syndrome should be cau
tioned about the high risk for maternal mortality during
pregnancy.
rtI PortTS
. In patients with cyanotic congenital heart disease, phle
botomy is recommended only for symptomatic hyper
viscosity with a hemoglobin level greater than 20 g/dl
(200 g/L) and hematocrit greater than 65% in the absence
ofdehydration; iron deficiency should be avoided.
. Experienced cardiologists and anesthesiologists should
manage patients with cyanotic congenital heart disease
who are undergoing surgery or invasive procedures;
these patients require endocarditis prophylaxis for non-
sterile procedures, intravenous line filters to prevent air
embolism, and measures to prevent venous thrombo-
embolism (early ambulation, pneumatic compression
devices, and, occasionally, anticoagulation).
Diseases of the Aorta
lntroduction
Diseases of the aorta comprise chronic conditions, such as
thoracic and abdonrinal aortic aneurysms and aortic athero-
mas, and potentially lii'e threatening acute conditions, such as
aortic dissection and aneurysm rupture. Appropriate treat,
ment and surveillance of aortic disease are critical to prevent
ing disease progression, complications, and mortality.
Thoracic Aortic Aneu
rysm
Thoracic aortic aneurysm (TAA) is defined as an increase in
the thoracic aortic diameter of greater than 50'1, relative to the
expected or normal aortic dimension, which is influenced by
age, sex, and body type. TAAs may occur at the level of the
aortic root, ascending aorta, aortic arch, or descending aorta.
Most commonly, they involve the aortic root and ascending
aorta, often forming at the site of aortic atherosclerosis.
Common causes of TAA are summarrized in Table 35. TAAs
are usually the result of cystic medial degeneration and weak
ening o1'the aortic wall due to loss of smooth muscle flbers and
elastic fiber degeneration. TAAs occurring in patients younger
than 50 years often are caused by connective tissue disorders,
such as Marfan syndrome or Ehlers Danlos syndrome.
Bicuspid aortic valve morphologr is an important risk factor
fbr TAA lbrmation, with TAA occurring in approximately 50'7,
of patients with a bicuspid aortic valve. Other risk factors for
'lAA
include hypertension, smoking, and advanced age.
TAAs often are asymptomatic and detected incident:rlly
during echocardiographic evaluation of left ventricular function
Atherosclerosis
Connective tissue
d isorders
Other genetic
and/or
congenital
conditions
Vascu litis
lnfectious
Diseases of the Aorta
Marfan syndrome
Ehlers-Danlos syndrome type lV
Loeys-Dietz syndrome
Ankylosi ng spondylitis
Familial thoracic aortic aneurysm and
aortic dissection syndrome
Bicuspid aortic valve
Turner syndrome
Coarctation of the aorta
Takayasu arteritis
Giant cell arteritis
Nonspecific (idiopath ic) aortitis
Other autoimmune conditions (BehEet
syndrome, relapsing polychondritis, lgG4-
related disease)
Septic embolism
Direct bacterial inoculation
Bacteremia
Contiguous infection
Syphilis
Prior acute aortic syndrome
Chest trauma
Aortic injury
or murmurs. Infrequently, dysphagia and hoarseness occur
when an aneurysm compresses surrounding structures. If the
aneurysm ruptures, patients may have severe chest pain, back
pain, sudden shortness ofbreath, or sudden death. A diastolic
heart murmur, wide pulse pressure, or symptoms ol heart fail
ure may signal aorlic regurgitation, which olten occurs in com
bination with TAA.
Screening and Surveillance
Screening for abnormalities of the thoracic aorta with aortic
imaging is indicated in asymptomatic patients with a bicuspid
aortic valve, a genetic condition that predisposes to aortic
aneurysms and dissections (e.g., Marfan syndrome, Ehlers
Danlos syndrome), or a family history of TAA or aortic dissec
tion. Screening is not recommended in other asymptomatic
persons.
If TAA is identified, the aortic cross sectional area should
be determined using noninvasive imaging (Table 36). Aortic
diameter measurement often varies substantially depending
on the type of imaging study used. Care must be taken to
measure the dimension perpendicular to the long axis of the
aorta because oblique measurements may overestinlate the
true diameter. The maximum aortic diameter at the site of
aneurysm (measured in centimeters) generally is included in
the criteria fbr surveillance and treatment.
i
Syndromescategory
TABLE 35" Causes of Thoracic Aortic Aneurysm
99

112. Diseases of the Aorta
TABLE 36. Thoracic Aortic lmaging Modalities
Modality
Transthoracic
echocardiography (TTE)
Transesophageal
echocardiography (TEE)
CT angiography
Magnetic resonance
angiography
Advantages
Good visualization of aortic rooVproximal
ascending aorta
Sensitivity and specificity for identifying proximal
aortic dissection of 77%-807o and93%-96o/o,
respectively
No exposure to radiation or contrast dye
Allows definition of valvular pathology, myocardial
function, pericardial disease
Bedside diagnosis
Superior image quality compared with TTE
Excellent visualization of the aorta from its root to
the descending aorta
Sensitivity and specificity for identifying proximal
aortic dissection of 887o-98% a nd 90/"-957",
respectively
No exposure to radiation or contrast dye
Allows definition of valvular pathology, myocardial
function, pericardial disease
Bedside diagnosis
Visualization of entire aorta and side branches
Sensitivity, 1 00%; specificity, 98o/o-99o/"
Rapid imaging
M u lti planar reconstruction
Visualization of entire aorta and side branches
Sensitivity and specificity >98%
No exposure to radiation or iodinated contrast
dy"
Visualization of aortic lumen, side branches, and
collaterals
Provides exact information on aorta size and
shape and any anomalies
Disadvantages
Requires experienced operator
Limited visualization of the distal ascending aorta
and aortic arch and branches of the great vessels
A negative TTE result does not rule out aortic
dissection, and other imaging techniques must be
considered
Diagnosing intramural hematoma may be
challenging
Requires experienced operator
lnvasive procedure
Diagnosing intramural hematoma may be
cha llenging
Exposes patientto radiation and iodinated
contrast dye
For acute disease, prolonged image acquisition
away from acute care area
Contraindicated in patients with implanted
pacemaker or defibril lator
Gadolinium contrast dye contraindicated in
patients with kidney diseaseu
Diseases of the aortic wall and thrombus-filled
discrete aortic aneurysms may be missed
lnvasive procedure that requires power injection
within the aorta
Requires dye load and may be nephrotoxic
Catheter manipulation can worsen dissection
Poor ability to diagnose intramural hematoma
given a lack of luminal disruption
Aortography
gadolinium based contrast agents are likely safe in this population. The safety of group lll gadolinium-based contrast agents in this population is unknown.
Because the leading cause of death in patients with TAA is
rupture, surveillance and treatment depend on aneurysm size
and subsequent rupture risk. The average expansion rate of
TAA is 0.1 cm/year but is influenced by patient-specific fac-
tors. Expansion rate increases with increasing diameter of the
aneurysm and is faster during pregnancy, when involving the
ascending aorta, and in patients with either Marfan syndrome
or bicuspid aortic valve. Annual aneurysm imaging is recom-
mended for patients with degenerative conditions of the aortic
root or ascending aorta measuring 3.5 to 4.4 crn in diameter;
imaging should be performed every 6 months if 4.5 to 5.4 cm
in diameter. In patients with Marfan syndrome, repeat assess
ment should be done 6 months after diagnosis to determine
the rate of aortic enlargement. Thereafter, imaging is recom
mended every year for aneurysms measuring 3.5 to 4.4 cm in
diameter and every 6 months if 4.5 to 5.0 cm. In all patients,
more frequent imaging is recommended if rapid expansion is
documented (>0.5 cm/year) or the aneurysm is approaching
the threshold for repair. Aneurysms smaller than 5.0 cm are
most often imaged with transthoracic echocardiography, pro
vided an adequate image can be obtained. Other imaging
modalities include CT angiography (CTA) and magnetic
100

113. resonance angiography (MRA). Echocardiography is obtained
in patients with a bicuspid aorlic valve.
Treatment
TAAs with a diameter smaller than 5.0 cm usually can be man
aged with medical therapy and active surveillance. Medical
therapy includes aggressive blood pressure control with a goal
blood pressure below 130/80 mm Hg. B
Blockers are the pre
f'erred antihypertensive agents in patients with TAA. In patients
with Marfan syndrome, B-blockers and losarlan have been
associated with a reduced rate of aneurysm growth.
When an ascending aortic aneurysm due to degenera
tive disease exceeds 5.5 cm in diameter or has rapid growth
(>0.5 cm/year), surgical repair is warranted to prevent the
morbidity and mortality associated with rupture. In patients
with an ascending aorta or aortic root larger than 4.5 cm in
diameter who require surgery tbr coronary artery disease
(CAD) or valve pathology, aortic repair should be performed
at the time of cardiac surgery. Current guidelines recom
mend that all patients with a bicuspid aortic valve and
ascending aortic aneurysm undergo aortic repair when the
aneurysm exceeds 5.5 cm in diameter, unless an additional
risk factor for dissection is present or the patient is at low
operative risk, in which case aortic repair is reasonable at
.5.0 cm. In patients with Marfan syndrome or another genet
ically mediated disorder, the American College of Cardiologi/
American Heart Association guidelines suggest elective aor'
tic repair at a lower threshold (4.0 5.0 cm, depending on the
condition).
Aneurysm location, aortic valve pathologr, and the pres-
ence of concomitant CAD dictate the type of thoracic aofiic
repair performed. Open surgical repair is indicated for TAAs
that involve the aortic root, ascending aorta, and aorlic arch.
Thoracic endovascular aortic repair (TEVAR) with stent graft
ing should be used when a descending aortic aneurysm has a
diameter greater than 6.0 cm, has exhibited rapid growth
(>0.5 cm/year), or has caused end organ damage, because
TEVAR has a lower morbidity and shorter hospital stay relative
to open surgical repair. TEVAR offers the advantage ofavoiding
open surgery although complications, such as stroke, spinal
ischemia, and aortic graft endoleaks, can occur.
r(EY P0r1{rS
o Asymptomatic patients with a bicuspid aortic valve,
genetic condition that predisposes to aortic aneurysms
and dissections, or family history of thoracic aortic
aneurysm or aortic dissection should undergo screening
for abnormalities of the thoracic aorta.
. In patients with an ascending aortic diameter exceeding
5.5 cm or demonstrating rapid growth (>0.5 cm/year),
aortic repair is warranted to prevent the morbidity and
mortality associated with aneurysm rupture; patients
with genetically mediated disorders should undergo
aortic repair at a lower threshold.
(Continued)
Diseases of the Aorta
l(EY POIilTS ((J,ntinufd,)
o Thoracic endovascular aortic repair is recommended in
patients with a descending aortic aneurysm when the
diameter is greater than 6.0 cm, has exhibited rapid
growth (>0.5 cm/year), or has caused end-organ damage.
Abdominal Aortic Aneurysm
Abdominal aortic aneurysm (AAA) is abnormal dilatation of the
abdominal aorta with an anteroposterior diameter greater than
3.0 cm. Risk factors include male sex (6:1 male to female inci
dence ratio), advanced age, smoking, atherosclerosis, hypefien
sion, and a family history of AAA.
Screening and Surveillance
AAA most often is diagnosed incidentally by CTA or abdominal
ultrasonography. Approximately 75')(, of patients with AAA are
asymptomatic at the time of diagnosis. Because of the high
mortality rate associated with aneurysm rupture, the U.S.
Preventive Services Task Force recommends one-time screen
ing with duplex ultrasonography in men aged 65 to 75 years
who have smoked at least 100 cigarettes during their lifetime
and selective screening for men in this same age group who
have never smoked.
Estimated annual risk for aoftic rupture according to AAA
dimension is shown in Table 37. In patients with AAA diameter
smaller than 4.0 cm, surveillance with duplex ultrasonography
every 2 to 3 years is warranted. In patients with AAA diameter of
4.0 to less than 5.5 cm, surveillance with CIA or duplex ultra
sonography should be perlormed every 6 to 12 months. Once the
aortic diameter meets the threshold for aortic repair (>5.5 cm),
CTA or MRA is indicated to determine the exact location of the
AAA (suprarenal, juxtarenal, or infrarenal) for planning repair'
Treatment
Medical treatment of AAA involves risk factor reduction to
decrease risk for rupture, cardiovascular morbidity, and
Aneurysm Diameter Annual Rupture Risk
<0.5%
0.57o-57"
3/o-15"/"
10%-20%
20%-407"
307" 50"/"
<4.0 cm
4.0-4.9 cm
5.0-5.9 cm
6.0-6.9 cm
7.0-7.9 cm
>8.0 cm
Reproduced with permission from Brewster DC, Cronenwett JL' Hallett JW Jr, et al;
Joint Counci of the American Association forVascular Surgery and Societyfor
Vascular Surgery. Guidelines for the treatment o{ abdominal aortic aneurysms'
Report of a subiommittee o{ the Joint Council o{ the American Association for
Vaicular Su rgery and Society for Vascular Su rgery. J Vasc Surg.2003;37:1 106 11
'
IPM lD: 1 27563631 d oiiAl06l /mva.2O03.363. 02003 Society for Vascular Surgery
ind The American Association for Vascular Surgery. Published by Elsevier lnc' All
rights reserued.
TABLE 37. Annual Rupture Risk of Abdominal Aortic
Aneurysm by Diameter
101

114. Diseases of the Aorta
overall mortality. Aortic repair should be performed in patients
with AAA diameter of 5.5 cm or larger, rapid expansion in
AAA size (>0.5 cm/year), or symptoms resulting from AAA
(e.g., abdominal or back pain). In patients with an indication
for aortic repair, the choice between open surgery and endo
vascular aneurysm repair (EVAR) is driven by the location of
the AAA and involvement of the renal and mesenteric arteries.
Suprarenal and juxtarenal aneurysms most often necessitate
open surgical repair. Patient age, comorbid conditions, and
ability to tolerate open surgical repair determine which proce
dure is performed in patients with an infrarenal AAA. EVAR is
associated with lower short term (30-day) morbidity and
mortality but no significant differences in long term mortality.
Additionally, EVAR is associated with greater need for repeat
intervention and significantly higher rates of endoleak, device
failure, and postimplantation syndrome (fever, leukocy,tosis,
elevated serum C reactive protein level). These complications
necessitate diligent follow up with noninvasive imaging tests
(CTA or ultrasonography) to evaluate the stent graft.
xEY POtilrt
o Risk factors for abdominal aortic aneurysm include
male sex, advanced age, smoking, atherosclerosis,
hypertension, and family history.
o One-time screening for abdominal aortic aneurysm
with duplex ultrasonography is recommended in men
aged 65 to 75 years who have smoked at least 100 ciga
rettes during their lifetime; selective screening is rec
ommended for men in this age group who have never
smoked.
. Aortic repair should be performed in patients with an
abdominal aortic aneurysm (AAA) diameter of 5.5 cm
or larger, rapid expansion in AAA size, or symptoms
resulting from AAA (abdominal or back pain).
Aortic Atheroma
Aortic atheromatous plaques (atheromas) commonly occur in
patients with evidence of atherosclerosis in other vascular
beds. The most frequent complication of aortic atheroma is
systemic thromboembolism resulting in transient ischemic
attack or stroke. Aortic atheromas greater than 4 mm in diam-
eter and those with a mobile component are more likely to be
associated with thromboembolism compared with smaller
atheromas.
Aortic atheromas often are detected incidentally on imag
ing studies. Their presence represents a CAD risk equivalent,
and patients should be considered for antiplatelet and statin
therapies in addition to other risk factor interventions.
rtY PottI
o Patients with an aortic atheroma should be treated with
antiplatelet and statin therapies to reduce cardiovascu
1ar risk.
Acute Aortic Syndromes
The most common and life threatening acute aortic syn-
dromes are acute aortic dissection and aortic aneurysm mp
ture. Other acute aortic syndromes include aortic intramural
hematoma and penetrating atherosclerotic ulcer.
Pathophysiology
Acute aortic dissection involves tearing of the aortic intima,
leading to passage of blood from the true lumen of the aorta
into a false lumen (Figure 55). Dissection of the aorta can
propagate in an antegrade or retrograde fashion, mainly due to
shear forces. Propagation of the dissection can cause cardiac
Acute aortic dissection
Acute intramural hematoma
Penetrating atherosclerotic ulcer
tl G U R E 5 5. Cross-sectional representation of acute aortic syndromes. Acute
aortic dissection: interruption of the intima (b/ue) with creation of an intimal flap
and false lumen formation within the media (red). Colorflow by Doppler
echocardiography or intravenous (lV) contrast by CI is present within the false
lumen in the acute phase. Acute intramural hematoma: crescent-shaped
hematoma contained within the media without interruption of the intima (b/ue).
No color flow by Doppler echocardiography or lV contrast by CT within the crescent.
Penetrating alherosclerotic ulcer: atheroma (yel/ow) with plaque rupture
disrupting intimal integrity; the blood pool is contained within the intima-medial
layer (pseudoaneurysm). Color flow by Doppler echocardiography or lV contrast by
CI enters the ulcer crater.
102

115. Diseases of the Aorta
tamponade, acute aortic regurgitation, compromise of arterial
side branches (carotid, mesenteric, renal, or iliac arteries), and
underperfusion of organs such as the brain, intestines, or
kidneys.
Aortic dissections are categorized according to their loca-
tion of origin using the Stanford classification, which describes
fype A dissections as originating within the ascending aorta or
aortic arch and type B dissections as originating distal to the
Ieft subclavian artery. Type A dissections require surgical
intervention because of risk for rupture and death, whereas
type B dissections often can be managed initially with medical
stabilization and blood pressure control.
lntramural hematomas result from microtears in the aor
tic intima and rupture of the vasa vasorum (Figure 56).
Penetrating atherosclerotic ulcers are caused by erosion of the
internal elastic membrane of the aorta at the site of atheroscle-
rotic plaque, leading to a blood-filled false space within the
wall of the aorta (Figure 57). Both intramural hematomas and
penetrating atherosclerotic ulcers are more common in type B
dissections.
Diagnosis and Evaluation
The diagnosis of an acute aortic syndrome requires a high index
of suspicion because of its life-threatening complications. Acute
aortic dissection classically presents as severe, sudden onset
chest or back pain that has a tearing or ripping quality. Other
presenting features may include hypertension, syncope, a mur
mur of aortic regurgitation, and heart failure. Asymmetric blood
pressures in the upper extremities, asymmetric pulses, or pulsus
paradoxus should raise suspicion for acute aortic dissection.
Abnormalities may be present on chest radiography (wid
ened mediastinum) and ECG (ST-segment depression), but
these findings are not diagnostic. In patients with a high likeli
hood of acute aortic dissection, diagnostic imaging should not
be delayed based on results ofchest radiography, ECG, or labo
ratory testing.
_l
at --_.
tIGURE 56. lntramural hematomademonstratedasalow-attenuationbandof
hematoma(aror'vs) intheaorticwall onCTimages Axial imagesatthelevel of theaortic
arch(topleft),throughthemid thorax(topmiddle),andatthelevel of thesuperiormesentericarterywithnarrowingoftheaorticlumen(topnght)'0bliquesagittal
reformatted iruge t-h,orgh the thorax (note ba nd a rtifact evident without the use of ECG gating
)
(bottorn /eft) Coronal reformatted image throug h the a bdomen
demonstrates thi length of the hematoma and an incidental infrarenal aortic aneurysm (bottom right)'
PubIshed by Ekevier lnc All Iighls le5erued.
103

116. Diseases of the Aorta
FIGU R E 5 7. Penetrating atherosclerotic ulcer of the proximal descending
thoracic aorta. Axial CT images at the level of the aortopulmonary window (top) and
at the level of the left pulmonary artery (bottom) demonstrate a small penetrating
ulcer (arow) that extends beyond the expected confines of the aortic lu men with
adjacent i ntramura I hematoma both at the level of the u lcer itself and that extends a
few centimeters caudally in the wall of the descending thoracic aorta. U =
penetrating
u lcer.
Reproduced with permission from Hiratzka LF, Bakris GL, Beckman JA, et al; American Coilege of Cardiology
Foundation/American Heart Association Task Force on Practice Guidelines.2010 ACCF/AHA/AATS/ACR/ASA/SCIV
SCAI/SlR/STS/SVM Guidelines for the diagnosis and management of patients with thoracic aortic disease. A report
of the American College of Cardiology Foundation/American Heart Association Task Force 0n Practice Guidelines,
American Associati0n f0rTh0racic Surgery, American College of Radiol0gy, American Str0ke Association, Society
of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and lnteruentions, Society 0f
l0teruentional Radiology,Society ofThoracic Surgeons, and Society forVascular Medicine. J Am Coll Cardiol.
2010;55:e27-e129.IPMID:20359588]doi:10.1016/j.jaa.2010.02.015.@20l0AmericanCollegeofCardiology
Foundation and the American HeartAss0ciati0n, lnc. Published by Elsevier lnc. All riqhts reserued.
CTA, MRA, and transesophageal echocardiography have
similar sensitivity and speciflcity in diagnosing acute thoracic
aortic disease; however, CTA is the imagrng modality of choice
because it provides important information for procedural plan-
ning and has widespread availability. Compared with CTA and
MRA, the primary advantages of transesophageal echocardiog
raphy in patients suspected of having aortic dissection include
portability for an unstable patient and lack of iodinated con-
trast. Transthoracic echocardiography often is used initially but
is limited by inability to image the distal ascending aorta,
transverse aortic arch, and descending aorta. Invasive aortogra-
phy rarely is indicated for the diagnosis of acute aortic disease;
however, it is performed at the time of endovascular repair or
when noninvasive testing is contraindicated or unavailable.
Treatment
Patients with acute aortic dissection without evidence of car
diogenic shock should be treated with medical therapy to
lower heart rate and blood pressure. Current guidelines rec-
ommend reducing systolic blood pressure to 120 mm Hg or
less in the first hour. Intravenous p-blockers are first-line
treatment. For hypertension that does not respond adequately
to p blocker therapy, an intravenous vasodilator (e.g., nitro-
pmsside, nicardipine) should be administered. Pain control is
often necessary and is best accomplished with intravenous
opioids.
An algorithm for the management of acute ascending
aortic dissection is shown in Figure 58. Emergency surgery
should be considered for acute aortic dissection complicated
by cardiogenic shock, type A dissection, and type A intramural
hematoma, given the very high mortality rate associated with
these conditions. Decisions regarding concomitant aortic arch
reconstmction, aortic valve replacement, branch vessel repair,
and/or coronary artery bypass graft surgery or coronary artery
reimplantation depend on the anatomy of the dissection,
involvement of the aortic valve or branch vessels. and other
patient characteristics.
Patients with uncomplicated type B aortic syndromes
may be treated with medical therapy initially. Compared with
medical therapy, TEVAR is associated with similar clinical
outcomes (overall survival) but improved aortic specific death
rates and disease progression measures at 5 years. Patients
with type B dissection and refractory chest/back pain or
hypertension, rapid aortic expansion, or organ malperfusion
should undergo aortic repair.
In patients with an aortic intramural hematoma or pen
etrating atherosclerotic ulcer, treatment choices depend on the
location of the hematoma or ulcer, progression to aortic dis
section, and evidence of aortic enlargement. Immediate aortic
repair is indicated in patients with a type A aortic intramural
hematoma or penetrating atherosclerotic ulcer and in those
with enlargement or progression of disease after detection.
f,EY POIIIIT
o Acute aortic dissection classically presents with the
sudden onset ofsevere tearing pain in the chest, back,
or abdomen.
o Clinical examination findings that increase the index of
suspicion for an acute aortic syndrome include pulsus
paradoxus, asymmetric blood pressures in the upper
extremities.andasymmetricpulses.,^ontinued)
:
:
j
:
:
104

117. Diseases of the Aorta
Ascending aortic dissection
by imaging study
STEP 1
Determine
suitability for
surgery
No
Yes
ls patient a suitable
candidate for surgery?
Begin medical
management
STEP 2
Determine
stability for
preoperative
testing
Yes
STEP 3
Determine
likelihood of
coexistent
CAD
Yes
No
No
Yes
ls patient stabl€ enough
to allow preoperative testing?
Age >40 years?
Assess need for
preoperative coronary
angiography
. Known CAD?
o Significant risk
factors for CAD?
Significant CAD by
angiography?
Plan for CABG if
appropriate" at
time of AoD repair
Urgent operative management
lntraoperative assessment
of aortic valve by TEE:
Aortic regurgitation?
or
Dissection of aortic sinuses?
Graft replacement
of ascending aorta
+/- aortic arch
Graft replacement
of ascending aorta
+/- aortic arch
and
repair/replacement
of aortic valve or
aortic root
STEP 4
lntraoperative
evaluation of
aortic valve
STEP 5 Yes No
Surgical
interuention
FIGURE 58.Acutesurgical managementpathwayforAoD.AoD=aorticdissection; CABG=coronaryarterybypassgrafting; CAD=coronaryarterydisease;TEE=transesophageal
echocard iog ra phy.
JAmCollCardiol.2009;53:53053.IPMID:19195618]d0i:10.1016/jjarc.2008.10.005
Published by Elsevier lnc. All riqhts reserued.
105

118. Peripheral Artery Disease
l(EY POltlTS (ondnued)
. CT angiography is the imaging modality of choice in
patients in whom acute thoracic aortic disease is suspected.
o Intravenous p-blockers are first-line treatment to
reduce heart rate and blood pressure in patients with
acute aortic dissection without cardiogenic shock.
. Emergency surgery should be considered for acute aor
tic dissection complicated by cardiogenic shock, type A
aortic dissection, and type A intramural hematoma.
Role of Genetic Testing and
Family Screening
Genetic conditions that predispose patients to TAA syndromes
include Marfan syndrome. Ehlers Danlos syndrome, and
Loeys Dietz. syndrome (see 'fable
35). Clinical findings and
family history olconnective tissue disease olten trigger genetic
testing for either diagnostic confirmation or screening of fam
ily members. Noninvasive in.raging of the aorta should be
performed if a pathogenic genetic mutation is found. Routine
surveillance (initially at 6 months ar.rd then annually if find-
ings are stable) should be perfbrmed to ensure that the aorta is
not enlarging rapidly.
In first degree relatives of patients with TAA and/or dis
section, noninvasive aortic imaging should be perfornted to
identify those with asymptomatic disease. Screening echocar
diography might be considered in first degree relatives of
patients with a bicuspid valve. l'irst degree relatives of patients
with a mutant gene (e.g., F8,ry1, TGFBRl, TGFBR2, COL3A1,
MYHLL) associated with aortic aneurysm andior dissection
should undergo genetic counseling and testing. Relatives with
the identified genetic mutation should then undergo noninva
sive aortic imaging.
Peripheral Artery Disease
Epidemiology and Screening
Peripheral artery disease (PAD) is typically characterized by
narrowing of the aortic bilurcation and arleries of the lower
extremities, including the iliac, femoral, popliteal, and tibial
arteries. Atherosclerosis is the most common cause. Risk fac
tors for PAD include smoking (current or past), diabetes mel-
litus, hypertension. hyperlipidemia, increasing age, and family
history of atherosclerosis. PAD occurs at a later age in women
than in men, and because women have a longer life span, the
overall prevalence is higher in women. The incidence of PAD
begins to increase at around age 40 years and rises to approxi-
mately 10'1, at age 70 years.
PAD is considered a coronary heart disease risk equiva-
lent, and both asymptomatic and symptomatic patients with
PAD are at increased risk fbr ischernic events, including
myocardial infarction, stroke. and cardiovascular death. Thus.
early recognition of PAD provides a unique opportunity to
identiff persons at increased cardiovascular risk and to mod
ify risk factors. According to guidelines from the American
Heart Association (AHA) and the American College of
Cardiology (ACC). <-rbtaining a screening ankle brachial index
(ABI) to identify PAD is reasonable in asymptomatic persons
with one of the following characteristics that signify increased
risk: (l) age 65 years or older. (Z) age so to 64 years rt'ith risk
tactors for atherosclerosis (e.g., smoking. diabetes. hyperten
sion, dyslipidemia) or family history t-rf PAD, (3) age younger
than 50 years with diabetes and one additional risk factrtr for
atherosclerosis. or (4) known atherosclerotic disease in
another vascular bed (coronary carotid, subclavian. renal. or
mesenteric artery stenosis or abdominal aortic aneurysm). The
U.S. Preventive Services Task Force concluded that there is
insufficient evidence to support screening for lower extremity
PAD with an ABI.
t(EY P0t1{IS
o The primary risk factors for peripheral artery disease
include smoking, diabetes mellitus, hypertension,
hyperlipidemia, and advanced age.
o Screening for peripheral artery disease (PAD) with
ankle brachial index testing may be considered in
patients at increased risk for PAD.
Clinical Presentation
Lower extremity PAD, by virtue of being defined by an abnor
mal ABI value rather than by symptoms. has a lvide spectrum
ol clinical manifestations. Patients may present with exer
tional leg pain relieved by rest (intermittent claudication),
atypical exertional leg pain. ischemic rest pain. nonhealing
wounds, ischemic ulcers. or gangrene.
Approximately 25'X, to 30'7, of patients with lower
extremity PAD present with intermittent claudication.
Patients with intermittent claudication often have reduced
exercise capacity and functional status compared with
age- and sex-matched controls, and their annual risk lor
myocardial infarction, stroke. or cardiovascular death is
approximately 5'){, to 77,. Most patients with intermittent
claudication have stable symptoms; however. symptoms
worsen in approximately 25'7, of patients. and 10'2, to 20'l,, of
patients will undergo lower extremity revascularization
procedures over a 5-year period.
Fewer than 5'X, of patients with PAD present u,ith chronic
limb threatening ischemia (CLTI; also termed critical limb
ischemia), the most severe fbrm of PAD. CLTI n-ranifests as
ischemic rest pain, tissue ulceration, and gangrene. Arterial leg
ulcers commonly occur on the distal toes, plantar aspect of the
foot, anterior portion of the lower leg where minimal collateral
arterial circulation is present, and sites of trauma. Arterial
ulcers usually are painful and have sharply demarcated
borders with a dry. pale gray or yellow wound base witl.rout
106

119. Peripheral Artery Disease
Evaluation
History and Physical Examination
A detailed history review of symptoms, and physical examina-
tion are essential in the evaluation for vascular disease.
Patients should be asked about walking impairment, a[zpical
limb symptoms (leg weakness, paresthesia), intermittent clau
dication, and ischemic rest pain. In patients with ex.ertional
leg symptoms, intermittent claudication should be differenti-
ated from pseudoclaudication (symptoms that arise from spi-
nal stenosis) (Table 38). Patients also should be questioned
about skin breakdown and loot ulcers and educated on the
importance of foot protection and wearing shoes (specifically,
hard soled shoes) when walking outside the home.
Components of the physical examination in patients with
suspected PAD are listed in Table 39. Vascular examination of
patients suspected of having lower extremify PAD should
include comprehensive pulse examination, auscultation for
bruits, and inspection of the feet for skin and toenail changes.
Patients with PAD may exhibit diminished, absent, or asym
metric pulses, and bruits may be heard at or near sites of arte-
rial stenosis. Patients with CLTI may have coolness in the
Characteristic Claudication Pseudoclaudication
t
Nature of
discomfort
Location of
discomfort
Exercise-ind uced
Walking distance
at onset of
symptoms
Discomfort
occurs with
standing still
Action for relief
Cramping,
tightness,
aching, fatigue
Same as for
claudication plus
tingling, burning,
numbness, weakness
Same as for
claudication; most
often bilateral
Variable
Variable
f I G U R E 5 9, Arterial insufliciency ukers (top panel) appear sharply demarcated
or "punched out" with a dry pale, gray, or yellow base, and the surrounding skin is
red, taut, and tender. Arterial ulcers are more likely to occur on the toes, plantar
surfaces of the foot, and the heel. Venous stasis ulcers(boftom panel) are typically
less painful and ocrur on the medial side of the lower leg (between the cal{ and
the ankle) in areas of hyperpigmentation. Venous ulcers are typically irregularly
shaped and shallow with yellow granulation tissue. They often weep serous fluid;
the ulcers are rarely necrotic.
evidence of granulation tissue (Figure 59). Patients with CLII
often have reduced exercise capacity and functional status,
and these patients have a 30'/. rate of major amputation and a
20% mortality rate within l year of diagnosis. Surgical or endo
vascular revascularization is usually necessary to salvage the
foot or limb.
IEY POIf,TS
o Peripheral artery disease may be asymptomatic or pre-
sent as intermittent claudication, atypical exertional leg
pain, or, rarely, chronic limb-threatening ischemia.
. Patients with peripheral artery disease have reduced
exercise capacity and an elevated risk for cardiovascular
morbidity and mortality.
. Ulcers due to peripheral artery disease often cause sig-
nificant pain; ulcers are usually sharply demarcated and
located on the distal extremities, such as the distal toes
and anterior portion of the lower leg, where minimal
collateral arterial circulation is present.
No
Stand or sit Sit, flexion at the
waist
<30 minTime to relief <5 min
Measure blood pressure in both arms (systolic blood pressure
difference >1 5 mm Hg suggests subclavian stenosis)
Auscultate for presence of arterial bruits (e.9., femoral artery)
Palpate for presence of abdominal aortic aneurysm
Palpate and record pulses (radial, brachial, carotid, femoral,
popliteal, posterior tibial, dorsalis pedis)
Evaluate for elevation pallor and dependent rubor of foot
lnspect feet for ulcers, fissures, calluses, tinea, and tendinous
xanthoma; evaluate overall skin care
Buttock, hi
thigh, calf,
Yes
Consistent
p,
foot
Yes
TABLE 38. Discriminating Claudication from
Pseu d ocla u d icatio n
TABLE 39. Physical Examination of Patients for Peripheral
Artery Disease
107

120. Peripheral Artery Disease
affected extremity as well as evidence of poor wound healing
or active ulceration. Clinicians should distinguish CLTI from
chronic venous disease (leg edema; pigmented, brawny indu
ration of the gaiter zone; ulceration of the shin or ankle)
because these conditions are treated differently (see MKSAP l9
General Internal Medicine 1).
Diagnostic Testing
The most commonly used diagnostic modality for identiffing
lower extremity PAD is measurement of the ABI, the ratio of
lower extremity to upper extremity systolic blood pressures.
Guidelines recommend ABI testing in all patients with history
or physical examination findings suggestive of PAD. ABI meas
urement is simple, inexpensive, and noninvasive, with a sen
sitivity and specificity approaching 90'7,. ABI values also have
prognostic importance; low values are associated with higher
rates ofmyocardial infarction, stroke, and death.
When undergoing ABI testing, patients should rest for
10 minutes in a supine position before the physician measures
the ankle pressures and brachial pressures with a Doppler
machine. Blood pressures should be measured in both arms
and in both legs at the dorsalis pedis and posterior tibial ankle
locations. To calculate the ABI for each leg, the higher ankle
pressure in that leg is divided by the higher brachial artery
pressure (regardless ofside). The ankle pressure is the same as
or slightly higher than the brachial pressure in healthy per
sonsi therefore, a normal resting ABI is between 1.00 and 1.40
(Table 4O). In the presence of atherosclerotic narrowing of the
limb arteries, the downstream blood pressure, and thus the
ABI value, is lower. A resting ABI of 0.90 or less is diagnostic
for PAD and correlates with abnormalities seen on imaging of
the arterial tree. Generally. patients with claudication have an
ABI of 0.4 to 0.9, whereas patients with ischemic rest pain,
ulceration, organgrene have an ABI ofless than 0.4. A resting
ABI greater than 1.40 indicates the presence ofnoncompress-
ible, calcified arteries in the lower extremities and is consid
ered uninterpretable. A toe-brachial index is used for diagnosis
in these patients, with a value of less than 0.70 indicating PAD.
Exercise ABI testing is useful in patients with ABI values
between 0.91 and 1.40 and high pretest probability. It requires
ABI measurements at rest and after treadmill walking or plan-
tar flexion exercises. A postexercise ankle pressure drop of
30 mm Hg or more or significant decline in the ABI suggests PAD.
Ankle-Brachiallndex lnterpretation
: 1.00-1.40
Noncompressible (calcified) vessel
(uninterpretable result)
Normal
Borderline
Mild to moderate PAD
Severe PAD
0.91-0.99
PAD = peripheral anery disease.
CT angiography Widely available
Useful in defining
the severity of PAD
Magnetic
resonance
lmaging
: fUodality
Arterial duplex
ultrasonography
Advantages Limitafions
Widely available
Does not require
administration of
contrast dye
lnexpensive
Useful in defining
the severity of
PAD
Limited abilityto
detect stenosis in
the pelvis and in
patients with severe
calcifications
Poor utility for
infrapopliteal stenosis
Risk for contrast-
induced
nephropathy
Very expensive
Contraindicated in
patients with
implanted pacemak-
ers or defibrillators
Risk for nephrogenic
systemic fibrosis in
patients with severe
kidney disease"
Very expensive
.40
-0.900.4
0.00-0.40
angiography
PAD = penpheral aftery disease.
"Group
I gadolinium based contrast agents are contraindicated in patients with an
estlmated glomerular filt.ation rate less than 30 mUmin/1.73 m2 or acute kidney
injury;
safety
Segmental pressure measurements in a vascular laboratory
may be used to localize diseased vessels. The procedure involves
pulse volume recordings (measurement of the magnitude and
contour of blood pulse volume in the lower extremities) and
blood pressure measurements at several locations in the lower
extremities (high thigh, low thigh, calf. posterior tibial artery.
and dorsalis pedis artery) (Figure 60).
Other imaging studies used to delineate the anatomic
location and severity of lower extremity PAD include arterial
duplex ultrasonography, CT angiography, and magnetic reso
nance angiography (Table 4l). These imaging modalities are
most often used to plan for endovascular or surgical revascu-
Iarization procedures. Invasive angiography is often preferred
because endovascular revascularization procedures can be
performed concurrently.
fEY POITIS
r An ankle-brachial index of 0.90 or less is diagnostic of
peripheral artery disease.
. An ankle-brachial index greater than 1.40 indicates the
presence of noncompressible arteries in the lower
extremities and is considered uninterpretable; a toe
brachial index is used for diagnosis ofperipheral artery
disease in these patients.
o In patients with peripheral artery disease, imaging with CT
angiography or magnetic resonanc€ angio,gaphy is usefuJ
in identiSing the location and severity of stenosis and
planning for endo scular or surgical revascularization.
TABLE 41 . lmaging Modalities for the Diagnosis of PAD
TABLE 40. Interpretation of the Ankle-Brachial lndex
108

121. Peripheral Artery Disease
Segmented BP
I S"g..nt-Brachial lnd
119 Brachial
ex
121
t
145
1.20
129
1.07
8s (PT)
0.70
80 (DP)
0.66
Medical Therapy
Treatment of PAD focuses on reducing cardiovascular risk,
improving functional status and quality of life, decreasing
claudication symptoms, and preventing tissue injury and
amputation.
Cardiovascular Risk Reduction
Cigarette smoking is the most important modifiable risk factor
for PAD. Smoking cessation is imperative to lowering the risk
for myocardial infarction and stroke and improving overall
survival in patients with PAD. Smoking cessation is also asso
ciated with decreased risk for major amputation, improved
patency rates following revascularization, and less disease
progression.
Diabetes is also a strong risk factor for PAD; however,
intensive glucose control has not been demonstrated to reduce
macrovascular complications, including myocardial infarc
tion, stroke, or amputation. Regardless, patients with PAD and
concomitant diabetes should adhere to American Diabetes
1 6s (PT)
1.36
1s1 (DP)
1.25
Association recommendations on diabetes management, with
particular attention to foot care (see MKSAP l9 Endocrinologz
and Metabolism).
Dyslipidemia has a mild effect on the development of
PAD. According to AHA/ACC guidelines, patients with PAD
should be treated with high intensity statin therapy, and
patients with PAD who are older than 75 years or intolerant of
high intensity statins should be treated with moderate inten
sity statin therapy (see MKSAP 19 General Internal Medicine 1).
Blood pressure control has been associated with a reduc
tion in cardiovascular events in patients with PAD. The
AHA/ACC recommends a blood pressure target of less than
130/80 mm Hg in patients with PAD. Adults with hypertension
and PAD are treated similarly to patients without PAD (see
MKSAP 19 Nephrologi).
Although older studies linked nonselective p blockers
(such as propranolol) with increased complications in patients
with severe PAD, fi selective B blockers appear to be better
tolerated and can be used with caution if otherwise indicated
for cardiovascular risk reduction. A meta-analysis showed no
175
1.45
170
1.40
153
1.26
96
0.79
0.70 Ankle-Brachial lndex 1.36
FIGURE 60.Apulsevolumerecordingdemonstratingdecreasedperfusioninthepatienttrightside.Anankle-brachial indexvalueof0.T0ontherightsideisconsistent
upstroke and downstroke (the width of the waveform is much narrower compared with the width of the waveform on the right side). When the amplitude of the waveform is
significantly blunted and the width is broader, it suggests moderate to severe disease. BP = blood pressure; DP = dorsalis pedis; PT= posteriortibial.
Gain:100% Gain
R) Dorsalis Pedis
Gain:100%
L) Dorsalis
Gain: 95%
109

122. Peripheral Artery Disease
increased risk for symptom exacerbation in patients with mild
to moderate PAD treated with selective B-blockers.
Antithrombotic Therapy
Current guidelines recommend antiplatelet monotherapy in
patients with PAD to reduce risk for myocardial infarction,
stroke, and peripheral arterial events, including CLTI. Despite
little supporting evidence, aspirin has been recommended by
experts as the primary antiplatelet agent in patients with PAD.
In patients who are aspirin intolerant, clopidogrel is recom
mended as an acceptable alternative. There is no compelling
evidence lor the use of dual antiplatelet therapy with aspirin
plus clopidogrel in patients with PAD alone. ln the CHARISMA
trial, patients with PAD who were treated with aspirin plus
clopidogrel had a reduced rate of hospitalization lor myocar
dial infarction and ischemic events, which was mitigated by a
higher rate of bleeding.
Evidence from the COMPASS trial demonstrated that
aspirin (100 mg/d) plus rivaroxaban (2.s mg twice daily). a
direct factor Xa inhibitor, was associated with improved major
adverse cardiovascular and limb end points when compared
with aspirin plus placebo in patients with coronary artery
disease and PAD. Major bleeding, primarily gastrointestinal
bleeding, was increased in patients assigned to receive aspirin
plus rivaroxaban; however, fatal or critical organ bleeding was
not significantly different between the groups. In the VOYAGER
trial, patients undergoing endovascular or surgical revascu
larization for PAD had a significantly lower incidence of the
primary efficacy outcome (a composite of acute limb ischemia,
major amputation for vascular causes, myocardial infarction.
ischemic stroke, or death from cardiovascular causes) when
assigned to rivaroxaban (2.5 mg twice daily) plus aspirin com
pared with aspirin alone.
There is no evidence that oral anticoagulation with a
vitamin K antagonist (warfarin) is more effective than anti
platelet monotherapy in patients with PAD. and warfarin
antico:rgulation is associated rn,ith an increased risk for
major bleeding, including intracerebral bleeding. There is
also no evidence that direct acting oral anticoagulant ther
apy alone is superior to antiplatelet monotherapy in patients
with PAD.
Symptom Relief
Improving functional status and quality of life is ol high
importance for patients with PAD. In patients who can exer
cise, supervised exercise training has been associated with
improved functional performance and is recommended fbr
patients with intermittent claudication. Systematic reviews
comparing supervised exercise with home exercise have
reported improvements in maximal walking distance and ini-
tial claudication distance that favor supervised exercise; how-
ever, no statistically significant differences in quality of life
were observed. The Centers for Medicare & Medicaid Services
approved coverage of supervised exercise training for patients
with claudication in 2017.
Cilostazol. a phosphodiesterase inhibitor $'ith antiplatelet
and vasodilator activiry increases pain free walking distance
and overall walking distance in patients with claudication. and
clinical practice guidelines recomrnend that a therapeutic trial
of cilostazol be considered in patients with claudication. As
with other oral phosphodiesterase inhibitors (e.g.. inotropes
such as milrinone). the FDA has placed a black box warning on
use of cilostazol in patients with heart failure. There is no
approved pharmacotherapy lor patients with CLTI.
TtY POITII
. Smoking cessation is essential to reducing cardiovascular
risk in patients with peripheral artery disease.
. Antiplatelet monotherapy, typically low-dose aspirin.
reduces risk for myocardial infarction, stroke, and
peripheral arterial events, including chronic limb
threatening ischemia, in patients with peripheral artery
disease.
o Supervised exercise training is the most effective treat
ment for improving functional status in patients with
peripheral artery disease.
o Cilostazol is recommended for patients with intermit-
tent claudication but is contraindicated in patients with
heart failure.
I nterventional Therapy
Endovascular or surgical revascularization is eflective in
relieving symptoms and improving functional capacity and
wound healing in patients with intermittent claudication or
CLTL Referral for revascularization is indicated in patients
with life limiting claudication, rest pain, ulceration, or gan
grene, especially if there has been an inadequate response to
exercise training, antiplatelet medication. cilostazol, and/or
wound treatment. Patients with CLTI (ABI <0.40, a flat wave
form on pulse volume recording, and low or absent pedal flow
on duplex ultrasonography) should be considered for urgent
revascularization. Revascularization should also be considered
in patients with a favorable risk benefit ratio. as determined
by patient factors (age,
f railty, comorbid conditions). anatomic
factors (severity and burden of atherosclerotic disease. loca
tion of disease in the lower extremities), operator expertise.
and type olprocedure. Revascularization is not recommended
in asymptomatic patients.
Endovascular revascularization use has dramatically
increased in recent years because it is minimally invasive and
confers a lower risk for perioperative adverse events compared
with surgical revascularization. Endovascular techniques
include balloon angioplasty, stenting (bare metal or drug elut
ing), and atherectomy (laser or various cutting options). In
patients with isolated iliac disease. endovascular revasculari
zation is favored over surgery because of lower morbidity and
mortality, high procedural success, and high patency rates over
time. Most patients undergo balloon angioplasty with stenting
110

123. of the iliac arteries, which lras a sigr.rificantly higher long term
success rate than angioplasty al<tne.
In patients with f'emoral, popliteal, or tibial artery
(infrainguinal) disease, the patency rates for endovascular
revascularization are not as high as for iliac disease. Although
infrainguinal disease was traditionally treated with angio-
plasty alone, the advent ot atherectomy devices, nitinol
(nickel titanium) stents, and paclitaxel coated devices has
changed management. However, despite early efficacy data in
randomized controlled triirls. paclitaxel coated devices have
been associated with higher all cause death rates after 2 years
compared with standard treatments.
Surgical revasculariz:rtion is still recommended for
patients with complex anatomy that may limit percutaneous
procedural success and long term patency (e.g., long chronic
total occlusions, multisegment disease). The traro most com
mon surgical techniques are endarterectomy and surgical
bypass.
Hybrid revascularization, whicl'r combines surgical and
endovascular approaches in a single setting or finite time
frirme. has been used more fiequently in conjunction with the
rise in endovascular revascularization; however, it is not cur
rently recommended by clinical practice guidelines.
KEY POIl{IS
. In patients with life limiting claudication and inadequate
response to exercise or pharmacologic therapy, endo
vascular or surgical revascularization is indicated.
. Patients with chronic limb threatening ischemia should
be considered for urgent revascularization.
Acute Limb lschemia
Acute limb ischemia is an infic.quent but life threatening
manifestation of PAD. Patients classically present with at least
one of the "6 P's": paresthesia, pain, pallor, pulselessness,
poikilothermia (coolness), and paralysis. The most common
cause is acute thrombosis of a lower extremity artery stent, or
bypass graft. Other causes include thromboembolism, vessel
dissection (usually occurring periprocedurally), or trauma.
Acute limb ischemia represents a trne medical emergency;
l0'7, to 157, olpatients undergo amputation during initial hos
pitalization, and 207, of patients die within 1 year.
Anticoagulation, typically with unfractionated heparin,
should be initiated as soon as the diagnosis is suspected.
Specialists with expertise in revascularization should be con
sulted, and diagnostic angiography should be performed
immediately to define the anatomic level of occlusion. In addi
tion to surgical and endovascular revascularization options,
catheter directed thrombolysis improves outcomes in patients
with acute limb ischemiu.
Careful monitoring is required after limb reperflsion
because of frequent reocclusion, limb edema, and the possi
bility of compartment syndrome. Signs and symptoms of com
partment syndrome include severe pain, hypoesthesia, and leg
Cardiovascular Disease in Cancer Survivors
weakness. lf compartment syndrome occurs, surgical fasciot
omy is indicated to prevent irreversible neurologic and soft
tissue damage.
r(EY P0r{TS
o Acute limb ischemia is characterized by at Ieast one of
the "6 P's": paresthesia, pain, pallor, pulselessness,
poikilothermia (coolness), and paralysis.
. Patients with acute limb ischemia should receive emergent
anticoagulation therapy and diagnostic angiography in
preparation for endovascu lar or surgical revascularization.
. Careful monitoring after limb reperfusion is required
because of frequent reocclusion, limb edema, and the
possibility of compartment syndrome.
Cardiovascu la r Disease
in Cancer Survivors
Cardiotoxicity of Radiation
Therapy to the Thorax
Radiation therapy improves survival in patients with Hodgkin
lymphoma, early stage breast cancel and other thoracic
malignancies. With higher survival rates, cardiovascular dis
ease has emerged as the most common nonmalignant cause of
death in patients treated with chest radiation therapy, account
ing fbr 25'){, of deaths in survivors of Hodgkin lymphon.ra.
Radiation therapy causes a wide spectrum of cardiovas
cular diseases (Table 42).
'lhoracic
irradiation damages all
cells, including those of the pericardium, myocardium, valves,
coronary and peripheral vasculature, and conduction systenl.
with clinical disease usually presenting two to three decades
after treatment. The risk fbr radiation induced cardiac injury
is increased further in patients taking concomitant anthracy
clines or trastuzumab. Contemporary techniques that limit
total dosage and field size have decreased the risk for cardiac
conlplications following radiation therapy.
Acute pericarditis is the most common early manifesta
tion of radiotoxicify, affecting 2.5')(, of patients. The presenta
tion, diagnosis, and treatment are similar to those of idiopathic
acute pericarditis. Chronic or constrictive pericarditis develops
Cardiomyopathy
Conduction defects (atrioventricular block, bundle branch block)
Coronary artery disease
Coronary microvascular injury
Pericardial disease (acute pericarditis, chronic constrictive
pericarditis, pericardial effusion)
Peripheral artery disease
Valvular disease
L
TABLE 42, Cardiovascular Diseases Related to
Radiation Therapy
111

124. Cardiovascular Disease in Cancer Survivors
in 10'7, to 20'7, ofpatients 5 to 10 years after radiation therapy.
Pericardial calcification is not always present radiographically.
Late constriction can occur in those who have not experienced
acute pericarditis.
Radiation also damages the microvasculature, causing
endothelial dysfunction and ischemia that result in myocardial
fibrosis, diastolic dysfunction, and restrictive physiologr.
Radiation-induced cardiomyopathy presents similarly to pri
mary restrictive cardiomyopathy. Diff'erentiating cardiomyo
pathy due to myocardial fibrosis fiom pericardial constriction
is essential because the conditions have dilferent treatments
and outcomes (see Myocardial Disease).
Although all cardiac l'alves may be affected by radiation
therapy, left sided involvement predominates. Valvular regur-
gitation due to tissue retraction is the most common valvular
lesion in the first two decades after therapy, with later fibrosis
and calcification leading to mixed regurgitation and stenosis
(Figure 61).
Radiation causes fibrosis of the conduction system and
may lead to sinus node dystunction, lascicular and bundle
FIGURE 61. Transesophageal echocardiogram in a patientwith previ0us
mantle irradiation for Hodgkin lymphoma. The aortic valve leaflets are thickened,
and excursion is limited during systole (top panel). During diastole (bottom panel),
central aortic regurgitation (arow) is present.
branch blocks, and complete heart block. Up to 75% of long
term survivors of mediastinal radiation demonstrate conduc-
tion abnormalities on ECG. The need for permanent pacing is
more common after valve replacement surgery in patients who
have received radiation therapy.
Coronary artery disease (CAD) occurs earlier and with
increased incidence in patients treated with radiation ther-
apy. Coronary artery Iesions are typically ostial. long. smooth.
and concentric and have higher tibrotic content than typical
atherosclerotic lesions. The incidence ofCAD is increased by
traditional risk factors (e.g., smoking. dyslipidemia. and
hypertension). and aggressive therapy to address these risk
factors is indicated.
Surgical outcomes for patients with radiation associated
cardiovascular disease are significantly worse than in matched
cohorts. and radiation-associated aortic stenosis is associated
with rnorse short-term and long-term cardiovascular mortal-
ity. Recent trials have suggested that percutaneous approaches
to aortic valve replacement may be preferable in this group.
There is no consensus on cardiac testing after chest irra-
diation in asymptomatic patients. Baseline evaluation includ
ing echocardiography is reasonable, and several organizations
have recommended stress echocardiography at 5 to 10 years
after completion of therapy or at age 30 years, whichever
comes first. The role of serum biomarkers in surveillance is
unclear, and their use is not recommended. Routine screening
with nuclear medicine testing or coronary CT should be
avoided.
Statins. ACE inhibitors, and aldosterone antagonists.
although effective for risk factor reduction. have not been
proved to prevent radiation-induced cardiovascular disease.
TTY POITTS
o Thoracic irradiation damages all cells, including those
of the pericardium, myocardium, valves, coronary vas
culature, and conduction system, with clinical disease
usually presenting t'vvo to three decades after treatment.
. In patients with a history of chest irradiation, tradi-
tional cardiovascular risk factors, such as smoking, dys
lipidemia, and hypertension, should be aggressively
managed because of the increased risk for coronary
artery disease.
Card iotoxicity of Chemothera py
Chemotherapy may cause reversible or dose dependent,
irreversible cardiac injury (Table 43). Strategies to minimize
cardiovascular risk should be considered before initiation of
chemotherapy, including optimizing treatment of traditional
risk factors and identifying patients with the greatest long
term risk (Table a4). Cardiotoxic therapies should be mini-
mized if there are alternative therapies with equivalent
outcomes.
Acute anthracycline toxicity, which can present as heart
block, arrhythmias, heart failure, myocarditis, or pericarditis,
d
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112

125. Cardiovascular Disease in Cancer Survivors
TABTE 43. Cardiovascular Toxicities Associated with Chemotherapy
Toxicity Class
schemia
Left ventricular dysfunction
Hypertension
Venous th rom broembolism
Bradycardia/atrioventricular block
OT prolongation resulting in torsades de pointes
Atrial fibrillation
Alkylating agents
Anthracyclines
Antimicrotu bu lar agents
Monoclonal antibody
Proteasome inhibitor
Tyrosine kinase inhibitor
Antimetabolites
Antimicrotubular agents
Monoclonal antibody
Tyrosine kinase inhibitors
Monoclonal antibody
Tyrosine ki nase inhibitors
Alkylating agent
Angiogenesis inhibitor
Tyrosine kinase inhibitor
Angiogenesis inhibitor
Antimicrotubular agent
Alkylating agent
Anth racycline
Miscellaneous
Tyrosine kinase inhibitor
Alkylating agents
Anthracyclines
Antimetabolites
Histone deacetylase inhibitor
Microtubule agents
Monoclonal antibodies
Tyrosine kinase inhibitors
Anthracycl i nes
Antimetabolite
Miscellaneous
Monoclonal antibodies
Drugs
Cyclophosphamide, ifosfamide
Doxorubicin, epirubicin, idarubicin
Paclitaxel, docetaxel
Trastuzumab
Bortezomib, carfilzomib
Sunitinib
5-Fluorouracil, capecitabine
Paclitaxel, docetaxel
Bevacizumab
Erlotinib, sorafenib
Bevacizumab
Sorafenib, sunitinib
Cisplatin
Thalidomide
Erlotinib
Thalidomide
Paclitaxel
Oxaliplatin
Aclarubicin
Arse n ic
Vandetanib
Cisplatin, cyclophosphamide, ifosfamide
Doxorubicin, aclacinomycin A, mitoxantrone
5-Fluorouracil, leucovorin
Belinostat
Paclitaxel, docetaxel, gemcitabine
Trastuzumab, etaracizumab
Cetuximab, sunitinib, sorafenib, ibrutinib
Doxorubicin
5-Fluorou raci I
Arsenic
Nivolumab, trastuzu mab
Monomorphic ventricula r tachycardia
lnformat on from Tisdale JE, Chung MK, Campbeli KB, et al; American Heart Association Clinical Pharmacology Committee of the Council on Clinical Cardiology and Council on
doi:1 0.1 1 61 /CIR.00OOOOOOOO0OOiO5
Anthracycline ortrastuzumab in patients with multiple
cardiovascular risk factors
High-dose anthracycline (>-250 mg/m2 doxorubicin or
>600 mg/m2 epirubicin)
Combination anthracycline and trastuzumab
Combination anthracycline and radiation
Radiation of >30 Gy with the heart in the treatment field
occurs in fewer than 1'2, of patients and may be reversible.
Chronic progressive anthracycline toxicity usually presents as
dilated cardiomyopathy and is typically irreversible. It is most
closely linked with doxorubicin but may occur with epirubicin
and idarubicin. Chronic progressive toxicity has an early onset
(within 1 year of treatment) in 1.6"1' to 2.1% of patients and a late
onset (after 1 year) in up to 5'7, ofpatients. Late onset chronic
progressive toxicity is related to total cumulative dose. ln
patients with a cumulative anthracycline dose of 550 mg/m2,
the incidence of heart failure is up to 26'ln, and toxicity may not
l
TABLE 44. CancerTherapy-Associated FactorsThat
lncrease Long-Term Cardiovascular Risk
113

126. Pregnancy and Cardiovascular Disease
become clinically evident until 10 to 20 years after treatment.
Dexrazoxane, an iron chelator, reduces the incidence of
anthracycline cardiotoxicity and should be considered for
those at increased risk. Continuous anthracycline administra
tion, as opposed to bolus, and liposomal formulations of
doxorubicin also should be considered to lower risk for
anthracycline toxicity. The use of ACE inhibitors, angiotensin
receptor blockers, and p-blockers to prevent cardiotoxicity is
an ongoing area of interest, with conflicting results from small
studies.
Reversible injury is more commonly associated with tar
geted molecular therapy, such as trastuzumab, multitargeted
tyrosine kinase inhibitors, and anti vascular endothelial
growth factor antibodies. Trastuzumab toxicity causes Ieft
ventricular (LV) systolic dysfunction, with symptoms of heart
failure in 3'/" to 7"/,, of patients. It is more common in patients
older than 50 years and with concomitant anthracycline use.
Multitargeted tyrosine kinase inhibitors also cause cardiotox-
icity, although not all tyrosine kinase inhibitors carry the same
risk. Sunitinib is associated with up to a 50% incidence of new or
worsened hypertension and up to a 15% incidence of decreased
LV ejection fraction (LVEF). These effects may be reversible with
early recognition. Bevacizumab, an anti-vascular endothelial
growth factor antibody, is associated with significant but
reversible hypertension.
In patients preparing to receive chemotherapy associated
with possible LV dysfunction, baseline echocardiography is
recommended. Measurement of global longitudinal strain (GLS)
as part of surveillance echocardiography has been recom-
mended by some expert consensus panels. GLS expresses longi-
tudinal myocardial shortening as a simple percentage and may
be more sensitive than other echocardiographic techniques in
detecting ear$ myocardial dysfunction. For patients in whom
echocardiography is technically diffi cult, multigated acquisition
and cardiac magnetic resonance imaging are alternatives.
Although the frequency of surveillance of asymptomatic
patients must be based on clinical judgment, it is reasonable to
repeat echocardiography at a cumulative anthracycline dose of
250 mg/m2 and before each dose in patients with pre existing
LV dysfunction or those receiving higher cumulative doses.
The frequency of echocardiographic surveillance in patients
receiving trastuzumab who are at high risk for cardiac dys-
function is based on clinical judgment. Trastuzumab is tempo
rarily stopped if the LVEF drops by more than 157, or drops
10% to 15% and below the normal level. Patients who demon
strate normalization of LV function after discontinuation of
trastuzumab may receive additional therapy, and retrospective
data has shown that patients whose LVEF remains greater than
50% may continue treatment without long term cardiac effects.
A relative decline in GLS ofgreater than 15'7, is considered sub
clinical LV dysfunction and should prompt consultation with a
cardiologist. In patients taking sunitinib, surveillance with
baseline N-terminal pro-B-type natriuretic peptide measure
ment and LVEF assessment at baseline, 1 month, and every
3 months thereafter has been advocated.
Echocardiography may be repeated at therapy completion
and at 6 to 12 months in asymptomatic patients at increased risk.
Currently, there are no guidelines for long-term suneillance of
patients with normal LVEF after cardiotoxic chemotherapl:
In patients \vith clinical signs or symptoms of cardiac
dysfunction. measurement of cardiac biomarkers (e.g.' tro
ponin, N terminal pro-B type natriuretic peptide) and echo
cardiographf including GLS are recommended in identiffing
early toxicity and guiding individual therapy. At present. treat-
ment of patients with chemotherapy induced heart failure
follows standard paradigms, and decisions regarding continu
ation of chemotherapy must be individualized.
I(EY POIilTS
. Traditional cardiovascular risk factors increase the car
diotoxic risk of chemotherapy and should be managed
aggressively.
o Chronic anthracycline toxicity usually presents as irre
versible dilated cardiomyopathy.
o Cardiotoxicity from trastuzumab manifests as reversible
left ventricular systolic dysfunction.
Pregnancy and
Card iovascu lar Disease
Maternal mortality has increased in the United States while
decreasing in other Western countries o\er the past t$/o dec
ades. Acquired cardiovascular disorders (cardiomyopathy,
coronary artery disease, aortic disorders) are the most com
mon cause of maternal mofiality.
Card iovascu la r Cha nges
During Pregnancy
Knowledge of the cardiovascular changes of pregnancy is nec
essary to distinguish betr,teen normal and pathologic signs and
symptoms in the pregnant patient (Table 45). During a normal
pregnancy, patients develop a relative anemia due to increases
in plasma volume and, to a lesser degree. erythrocyte mass.
Mean arterial pressure slightly decreases in the setting of
reduced systemic vascular resistance and increased heart rate
and cardiac output. By the 32nd week ofpregnancy. maternal
cardiac output peaks at approximately 407, to 50'X, above the
prepregnancy level owing to increased heart rate and stroke
volume. During delivery the cardiac output may increase to as
much as BO'X, above the prepregnancy level.
Prepreg na ncy Eval uation
A1l women with cardiovascular disease should receive preg
nancy counseling. If appropriate, women should receive
genetic counseling and possibly testing. Women who are
anticipating pregnancy should undergo multidisciplinary
114

127. TABLE 45. Normal Versus Pathologic Signs and
Symptoms in Pregnancy
I
:
Blood pressure Low blood pressure
associated with
symptoms, high
blood pressure
(>140 mm Hg
systolic or >90 mm
Hg diastolic)
Systolic murmur
grade >3/6, any
diastolic murmur, Sa
Auscultatory Basal systolic
murmur grade
1/6 or 2/6
present in 80% of
pregnant
women, 53
evaluation with a cardiologist, a maternal fetal medicine spe-
cialist, and an obstetric anesthesiologist to determine the risks
of pregnancy and develop a management plan for labor and
the postpartum period. Disease specific risk should be
assessed using the modified World Health Organization preg
nancy risk classification (https:ilacademic.oup.com/view-
largel186437995). which is currently the most accurate system
of risk assessment.
Women wilh certain cardiovascular disorders, including
uncomplicated small patent ductus arteriosus. mild pulmo-
nary stenosis, and mitral valve prolapse, have no increased
morbidity or mortality. Similarly. pregnant patients with suc
cessfully repaired simple lesions (atrial or ventricular septal
defect, patent ductus arteriosus, or anomalous pulmonary
venous drainage) or isolated atrial or ventricular ectopic beats
do weil. These patients can be cared lbr and deliver in a local
hospital.
Small increased risk of maternal mortality or moderate
increase in morbidity is associated with an unrepaired atrial or
ventricular septal defect, repaired tetralogy of Fallot, most
arrhythmias (supraventricular arrhythmias), and Turner syn
drome without aortic dilatation. Patients with these conditions
also can be cared for and deliver in a local hospital setting.
Cardiovascular conditions that confer an extremely high
risk for nraternal mortality or severe morbidity include
Pregnancy and Cardiovascular Disease
pulmonary hypertension, previous peripartum cardiomyopa
thy with residual left'uentricular dysfunction, severe left ven
tricular dysfunction (ejection lraction <30'X, or New York Hearl
Association functional class III IV sy,mptoms), severe mitral
stenosis, symptomatic severe aortic stenosis, and marked
ascending aorta dilatation. Care and delivery should be per
formed at an expert center for pregnancy and cardiac disease.
XEY POITTS
. Women with cardiovascular disease who are anticipat-
ing pregnancy should undergo multidisciplinary
prepregnancy evaluat ion.
o Cardiovascular conditions that confer an extremely
high risk for maternal mortality or severe morbidilz
include pulmonary hypertension, previous peripartum
cardiomyopathy with residual left ventricular dysfunc
tion, severe left ventricular dysfunction (ejection frac
tion <30% or New York Heart Association functional
class III IV symptoms), severe mitral stenosis, sympto-
matic severe aortic stenosis, and marked ascending
aorta dilatation.
Management of Cardiovascular
Disease During Pregnancy
Women with obstructive valvular lesions ntay experience
symptoms during pregnancy because of the resultant increases
in blood volume and cardiac output. These patients should be
evaluated to determine whether cardiac intenrention should be
considered before pregnancy. Women with regurgitant valve
lesions usually tolerate pregnancy well. Women with sympto-
matic obstructive hypertrophic cardiomyopathy should be
treated with nonvasodilating p blockers, with monitoring of
fetal growth.
Vaginal delivery is generally preferred fbr patients with
cardiovascular disease because it results in less blood loss.
quicker recovery and lower risk for thrombosis than does
cesarean delivery. Cesarean delivery is recommended for
obstetric reasons in women with severe decompensated car
diovascular disease and in some patients with a n-rarkedly
dilated aorta. In women receiving warf'arin therapy, cesarean
delivery is indicated to reduce the risk for f'etal intracranial
hemorrhage because the t'etus is fully anticoagulated.
Peripartum Cardiomyopathy
Peripartum cardiomyopathy is left ventricular systolic dys
lunction recognized towarcl the end of pregnancy or in the
months following delivery in the absence of another identifi
able cause. Risk factors fbr peripartum cardiomyopathy
include multiparity, age older than 30 years, Black race, mul
tiletal pregnancy, gestational hypertension, preeclampsia, a
previous episode of peripartun.r cardiomyopathy, and tocolytic
therapy (e.g., terbutaline). Patients with peripartum cardio
myopathy should be co managed by a cardiologist.
Type of Sign or
Symptom
Pulmonary
Cardiac
Heart rhythm
Heart rate
Normal
Mild dyspnea,
dyspnea with
exertion
No symptoms
Mild peripheral
edema
Atrial and
ventricu la r
premature beats
Heart rate
increased by
20%-30%
Blood pressure
typically is
modestly
decreased
(- 10 mm Hg)
Pathologic
Orthopnea,
paroxysmal
nocturnal dyspnea,
cough, pulmonary
edema
Chest pressure,
heaviness, or pain
More than mild
edema
Atrialfibrillation or
flutter, ventricular
tachycard ia
Heart rate >100/min
Ed ame
L
I
I
I
I
I
,
115

128. Pregnancy and Cardiovascular Disease
Death in women with peripartum cardiomyopathy is
caused by heart failure, thromboembolic events, or arrhyth-
mias. Most women who develop peripartum cardiomyopathy
recover fully, as measured by improvement in ejection frac-
tion; however, in a prospective study, 13%, had major cardiovas-
cular events or persistent severe cardiomyopathy. Studies
suggest that the time frame for recovery is 6 months. Prognostic
factors that portend a worse outcome include severe Ieft ven-
tricular dysfunctionidilatation at presentation, Black race,
older maternal age, and multiparity.
Women with peripartum cardiomyopathy should be
promptly treated with medical therapy that may include
B-blockers, digoxin, hydralazine, nitrates, and diuretics. ACE
inhibitors, angiotensin receptor blockers, and aldosterone
antagonists are teratogenic and should be avoided until after
delivery. Owing to the high risk lor thromboembolism associ-
ated with peripartum cardiomyopathy, anticoagulation is rec-
ommended for women with left ventricular ejection fraction
below 35'7,. The choice of anticoagulant (heparin or warfarin)
depends on whether the patient is still pregnant and the time
since delivery. Duration of anticoagulation is at least 8 weeks,
although therapy can be discontinued sooner if the ejection
fraction normalizes.
Women with severe refractory heart failure due to peri-
partum cardiomyopathy should be referred to a specialty
center for advanced treatment, including ventricular assist
device placement, advanced arrhythmia management, and
heart transplantation.
Because subsequent pregnancy is often associated with
recurrent or further reduction of left ventricular function,
potentially resulting in clinical deterioration or death, women
with a previous episode of peripartum cardiomyopathy with
persistent left ventricular dysfunction should be advised to
avoid future pregnancy.
Other Cardiovascular Disorders
Data have linked adverse pregnancy outcomes (hypertensive
disorders of pregnancy, preterm delivery glucose intolerance
during pregnancy, small for gestational-age delivery placental
abruption, and pregnancy loss) with the acquisition of cardio-
vascular risk factors and cardiovascular disease. It is unknown
how this information should be used to reclassiflz cardiovascu-
lar risk in affected women, but a heart healthy diet and exer-
cise are recommended beginning in the postpartum period
and extending throughout the lifespan.
Women with Marfan syndrome and related conditions
have an increased risk for pregnancy related aortic dissection.
Aortic repair is recommended before pregnancy in women with
Marfan syndrome and an aortic diameter of 4.5 cm or greater.
Risk factors for dissection in pregnant patients with Marfan
syndrome and an aortic diameter smaller than 4.5 cm include
rapid dilatation of the aorta or a personal or family history of
aortic dissection. Patients with risk factors and an ascending
aortic diameter greater than 4.0 cm should be counseled to
undergo aortic valve repair or replacement before pregnancy
Spontaneous coronary artery dissection is the leading
cause of pregnancy associated myocardial infarction and
occurs most commonly in the first month postpartum (see
Coronary Artery Disease). Conservative noninterventional
therapy is preferred for most patients.
Cardiovascular Medication Use
During Pregnancy
Guidelines for the use of select cardiolascular drugs during
pregnancy are outlined in Table 46. Most cardiovascular drugs
are not FDA-approved for use during pregnancy because of
limited safety data. Cardiovascular medications should be
used only when needed and at the lowest possible dosage,
with the desired therapeutic effect outweighing the risk.
p-Blocker use during pregnancy or lactation requires peri
odic fetal and newbom heart rate monitoring because p-blockers
cross the placenta and are present in human breast milk.
labetalol is the preferred
B blocker in this setting. Atenolol has
been linked to premature delivery and small-for-gestational-age
babies and thus is usually avoided during pregnancy.
Adenosine is the drug of choice for treatment of acute
symptomatic supraventricular tachycardia during pregnancy.
Recurrent episodes of tachycardia are often treated with
p-blockers; sotalol and flecainide have also been used safely.
Because of toxicity concerns, amiodarone is used rarely.
ACE inhibitors, angiotensin receptor blockers, direct
renin inhibitors, and aldosterone antagonists should be
avoided during pregnancy, although some ACE inhibitors and
spironolactone are safle to use while breastfeeding. There is
inconclusive evidence on the safety of direct renin inhibitors
and angiotensin receptor blockers while breastfeeding; these
drugs are generally avoided during lactation.
A useful source of information on drugs to which breast-
feeding mothers may be exposed, including potential adverse
effects and suggested therapeutic alternatives, is the LactMed
database (https://toxnet.nlm.nih.gov/newtoxnet/lactmed.htm).
Anticoagulation Therapy During Pregnancy
Pregnancy is associated with hypercoagulability. Table 47 lists
the indications for anticoagulation in pregnancy, recom
mended regimens, and monitoring parameters for therapy.
Prepregnancy counseling is recommended for all r.tomen
requiring long-term anticoagulation to enable them to make
informed decisions regarding anticoagulant preference and to
understand the maternal and fetal risks.
Warfarin, unfractionated heparin, and low molecular-
weight heparin can all be used during pregnancy. Careful
monitoring and dosage adjustment to attain the therapeutic
target are indicated for all anticoagulation regimens. Warfarin
use during the first trimester can cause warfarin embryopa
thy; the risk is increased when the daily dose is greater than
5 mg. Warfarin is stopped before delivery owing to the risk for
fetal intracranial hemorrhage ifspontaneous labor and vaginal
delivery occur while the mother (and thus the fetus) is antico
agulated with warfarin.
I
116

129. Pregnancy and Cardiovascular Disease
Use in
Pregnancy
Compatibility with
Breastfeeding
Drug Comments
ACE inhibitors
Captopril, enalapril
Lisinopril
Aliskiren
ARBs
Adenosine
Amiodarone
Antiplatelet and
anticoagulant agents
Dipyridamole,
clopidogrel
Aspirin (<81 mg)
DOACs (apixaban,
dabigatran, rivaroxaban,
edoxaban, betrixaban)
p-Blockers
Atenolol
Esmolol
Labetalol
Metoprolol
Propranolol
Sotalol
Calcium channel blockers
Diltiazem, verapamil
Digoxin
Disopyramide
Diuretics
Flecainide
Hydralazine
Lidocaine
Sodium nitroprusside
Organic nitrates
Phenytoin
Procainamide
Propafenone
Ouinidine
?
?
No
No
No
No
Yes
No
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
No
Yes
No
Yes
Yes
Yes
Yes Teratogenic in firsttrimester; cause fetal/neonatal kidney failure
with second- or third-trimester exposure; scleroderma renal
crisis is only indication
Same as above
Teratogen ic in first trimester; causes feta l/neonata I kid ney fa ilu re
with second- or third-trimester exposure
Teratogenic in first trimester; cause fetal/neonatal kidney failure
with second- or third-trimester exposure
No change in fetal heart rate when used for supraventricular
tachycardia
Fetal hypothyroidism, prematurity
Second-line agent; no evidence of harm in animal clopidogrel
studies; no human data
Pregnancy and lactation should be avoided in DOAC-treated
patients; available data do not suggest a high risk for DOAC
embryopathy or neonatal complications
Second-line agent; low birth weight, intrauterine growth
restriction
Second-line agent; more pronounced bradycardia
Preferred drug in class
Shortened half-life
Second-line agent; intrauterine growth restriction
Second-line agent; insufficient data; reserve use {or arrhythmia
not responding to alternative agent
Second-line agent; maternal hypotension with rapid intravenous
i nfusion; used for feta I su praventricu la r tachyca rd ia
Second-line agent; shortened half-life
Second-line agent; case reports of preterm labor
Second-line agent; use when needed for maternalvolume
overload only
Second-line agent; inadequate data but used for fetal
arrhythmia; case report of fetal hyperbilirubinemia
Vasodilator of choice
Treatment of choice for ventricular arrhythmias
Potentia I feta I th iocya nate toxicity
No apparent increased risk
Known teratogenicity and bleeding risk; last resort for arrhythmia
Used for fetal arrhythmia
Second-line agent; used for fetal arrhythmia
Preferred drug in class; increases digoxin levels
?
?
No
?
Yes
?
?
No
Yes
Yes
No
?
Yes
Yes
?
Yes
?
Yes
Yes
Yes
Yes
Yes
Yes
Yes
?
? = unknown; ARB = angiotensin receptor blocker; DOAC = direct oral anticoagulant.
pp 356 357. @2008 American College of Physicians.
117

130. Venous Thromboembolism
TABLE 47. Anticoagulation Regimens During Pregnancy
Weeks 13 37
Weeks 37 to term
Bibliography
Weeks of Gestation Recommended Regimen
Weeks 6 to term Weight-based LMWH
UFH (lV or SQ; aPTT 2 x control)
Nonvalvular Atrial Fibrillation
Weeks 6-12 Warfarin (if dose to attain
therapeutic INR <5 mg)
UFH (lV or SO; aPTT 2 x control)
Weight-based LMWH
UFH (SO; aPTT 2 x control)
Weight-based LMWH
Warfarin (therapeutic INR)
UFH (lV; aPTT 2 x control)
Mechanical Valve Prosthesis
Weeks 6-12 lf warfarin daily dose <5 mg for
therapeutic INR
Continue warfarin (class 2a
recommendation)
UFH: lV aPTT 2 x control (class 2b
recommendation)
Anti-factor Xa adjusted LMWH
(class 2b recommendation)
lf warfarin daily dose >5 mg for
therapeutic INR
UFH: lV aPTT 2 x control (class 2a
recommendation)
Anti-factor Xa adjusted LMWH
(class 2a recommendation)
Warfarin (therapeutic INR)
Weeks 37 to term UFH (lV; aPTT 2 x control)
aPTT = activated partial thromboplastin time; lV = intravenous; LMWH = low
molecular weight heparin; SO = subcutaneous; UFH = un{ractionated heparrn.
Recommendations from Otto CM, Nishimura RA, Bonow RO, et al.2020 ACC/AHA
guideline for the management of patients with valvular heart disease: a report of
the American College of Cardiology/American Heart Association Joint Committee
on Clinical Practice Guidelines. Circulation. 2021
; 1 43:e7 2-e227. IPMID: 333321 501
doi:10.1 161/C1R.0000000000000923 and Bates St'.,1, RajasekharA, Middetdorp S,
et al. American Society of Hematology 201 8 guidelines for management of venous
thromboembolism: venous thromboembolism in the context of pregnancy. Blood
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Pregnant women with a mechanical valve prosthesis
represent a high risk subset of patients. Concerns include
valve thrombosis with its associated maternal risks, bleeding,
and fetal morbidity and mortality. Warfarin appears to be the
salest agent to prevent maternal prosthetic valve thrombosis;
however, warfarin poses an increased fetal risk, with possible
teratogenicity, miscarriage, and fetal loss. Data suggest that
low-molecular-weight heparin and unfractionated heparin
are safer for the fetus than warfarin, but these therapies
appear to increase the risk for maternal prosthetic valve
thrombosis.
Guidelines from the American College of Cardioloryr
American Heart Association on the management of anticoagu
lation during pregnancy in the setting of mechanical valve
prosthesis recommend warfarin during the first trimester if
the daily dose is 5 mg or less at the time of conception.
lntravenous unfractionated heparin or dose adjusted lo'w
molecular-weight heparin is preferred if the warfarin dose is
more than 5 mg daily. During the second and early third tri
mesters, warfarin therapy is preferred. Intrarenous unfrac
tionated heparin is preferred for anticoagulation around the
time oldelivery in patients with a mechanical valve prosthesis.
At least weekly monitoring of the anticoagulation level is rec
ommended during pregnancy regardless of the regimen used.
with dosage adjustment as indicated.
In patients receiving direct oral anticoagulants, it is rec
ommended that pregnancy and lactation be avoided because
of limited data related to safety. If a woman becomes pregnant
while taking a direct oral anticoagulant. therapy should be
switched to an appropriate alternatir,e anticoagulant (heparin
or warfarin).
TEY POIf,TS
. Women with peripartum cardiomyopathy who present
before delivery should be treated with medical therapy
that may include B blockers, digoxin, hydralazine,
nitrates, or diuretics; ACE inhibitors, angiotensin recep-
tor blockers, and aldosterone antagonists can be used in
patients presenting postpartum but should be avoided
during pregnancy because of teratogenicity.
. In pregnant patients with a mechanical valve prosthesis,
warfarin anticoagulation is preferred during the first tri
mester if the daily dose is 5 mg or less; intravenous
unfractionated heparin or dose-adiusted low molecular-
weight heparin is preferred if the warfarin dose is more
than 5 mg daily.
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53:2231- 47. IPMID: 19520246] doi:10.1016/j.jacc.2009.02.05O
Yu AR Yadav NU, Eaton AA, et al. Continuous trastuzumab therapy in breast
cancer patients with asymptomatic left ventricular dysfunction.
Oncologist. 2015;20;1105 10. IPMID: 262401351 doi:10.1634/theoncologist.
2015 0125
Pregnancy and Cardiovascular Disease
Arany Z, Elkayam U. Peripartum cardiomyopathy. Circulation. 2016;133:1397
409. [PMID: 270451281 doi:10.1161/ClRCULATIONAHA.115.02049l
McNamara DM, Elkayam U, Alharethi R, et al; IPAC Investigators. Clinical
outcomes for peripartum cardiomyopathy in North America: results ofthe
IPAC study (lnvestigations of Pregnancy-Associated Cardiomyopathy). J
Am Coll Cardiol. 2015;661905 - 14. [PMID: 262937 60l doi: 10.1016/j. jacc.
2015.O6.1309
121

134. Bibliography
Ofto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the
management of patients with valvular heart disease: a report of the
American College of Cardiologr/American Heart Association Joint
C,ommittee on Clinical Practice Guidelines. Circulation. 202l)43te72-e227.
IPMID: 333321501 doi:10.1161/CIR.0000000000000923
Parikh NI, C,ot]-u;alez JM, Anderson CAM, et al; American Heart Association
Council on Epidemiologr and Prevention; C-ouncil on Arteriosclerosis,
Thrombosis and Vascular Biologr; Council on Cardiovascular and Stroke
Nursing; and the Stroke Council. Adverse pregnancy outcomes and cardio-
rascular disease risk: unique opportunities for cardiovascular disease pre-
vention in women: a scientific statement from the American Heart
Association. Circulation. 2021;143:e902 e9f6. [PMID:33T192t3ldoi:lO.tt6tt
cIR.0000000000000961
RegiE-Zagrosek Y Roos-Hesselink IW, Bauersachs J, et al; ESC Scientific
Document Group. 2ol8 ESC guidelines for the management of cardiovas-
cular diseases during pregnanry. Eur Heart J. 2018;39:3165-3241. [PMID:
301655441 doi:10.1093/eurheartj /ehy340
Tweet MS, Hayes SN, Codsi E, et al. Spontaneous coronary artery dissection
associated with pregnancy. J Am Coll Cardiol. 2017;70:426-435. [PMID:
287286861 doi: 10.lol6/j.jacc.2017.05.055
van Hagen IM, Roos-Hesselink ,w, Ruys TE et al; ROPAC lnvestigators and the
EURObserrrational Research Programme (EORP) Team'. Pregnancy in
women with a mechanical heart ralve: date of the European Society of
Cardiologr Registry ofPregnanry and Cardiac Disease (ROPAC). Circulation.
2075 ;132:132- 42. [PMID: 26100109] doi:1o.1161 /CIRCULATIONAHA.IIS.
015242
122

135. Cardiovascu lar Medicine
Self-Assessment Test
This self-assessment test contains one best-answer multiple choice questions. Please read these directions carefully
before answering the questions. Answers, critiques, and bibliographies immediately follow these multiple-choice
questions. The American College of Physicians (ACP) is accredited by the Accreditation Council for Continuing
Medical Education (ACCME) to provide continuing medical education for physicians.
The American College of Physicians designates MKSAP 19 Cardiovascular Medicine for a maximum of 30 AMA PRA
Category I CreditsrM. Physicians should claim only the credit commensurate with the extent of their participation
in the activity.
Successful completion of the CME activity, which includes participation in the evaluation component, enables
the participant to earn up to 30 medical knowledge MOC points in the American Board of Internal Medicine's
Maintenance of Certiflcation (MOC) program. It is the CME activity provider's responsibility to submit participant
completion infbrmation to ACCME for the purpose of granting MOC credit.
Earn Credits or MOC Points Online
To earn CME credits or to apply for MOC points, MKSAP users need to answer at least one of two questions correctly
(earning a score of at least 50"1,) and click the Submit CME button. Each single MKSAP 19 self-assessment question
qualifles for one quarter of a CME credit hour or ABIM MOC point.
MKSAP l9 Subscribers can enter their self-assessment question answers and submit for CME/MOC in two ways:
1. Users of MKSAP 19 Complete who prefer to use their print books and a paper answer sheet to study and
record their answers can use the printed answer sheet at the back of this book to record their answers. The
corresponding online answer sheets, which are available on the MKSAP 19 Resource Page, may be used to
transcribe answers onto the online answer sheets. Users may then submit their answers to qualify for CME
credits or MOC points (see below for information on Opting in for MOC). Users who prefer to record their
answers on a paper answer sheet should save their answer sheet for future use. Users who study with MKSAP 19
print can also submit their answers directly within MKSAP 19 Digital by accessing the self-assessment
questions dashboard and selecting the preferred subspecialty section to begin answering questions.
2. Users of MKSAP 19 Digital can enter their answers within the digital program by accessing the self-assessment
questions dashboard and selecting the preferred subspecialty section to begin answering questions and
clicking the Submit CME button once they qualify for CME and are ready to submit. Learners should keep in
mind their yearly CME and MOC deadlines when determining the appropriate time to submit.
Learners' CME/MOC submission progress will be shown on the MKSAP 19 Digital CME/MOC/CPD page.
Opting in for MOC
MKSAP 19 users can opt in for simultaneous submission of CME and MOC points as they answer self-assessment
questions. To opt in, users will be required to complete a fbrm requesting their name, date of birth, and ABIM
number. The MOC Opt-in Form will be presented upon the user's first CME submission and needs to be completed
only once.
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137. Directions
Each of the numbered items is followed by lettered answers. Se/ect th e ONE lettered answer that is BEST in each case.
Item 1
A 67 year-old woman is evaluated during a follow up visit
for heart failure with reduced ejection fraction. She reports
stable dyspnea with exertion when carrying groceries from
her car or walking up a hill. She has no other symptoms.
Medications are carvedilol, spironolactone, maximum dose
lisinopril, dapagliflozin, and furosemide.
On physical examination, blood pressure is 108/72 mm
Hg and pulse rate is 60/min. The remainder of the examina-
tion is unremarkable.
Laboratory studies reveal a serum creatinine level of
1.3 mg/dl (114.9 pmol/L).
A previous echocardiogram showed a left ventricular
ej ection fracti on of 37 "/,'.
Which of the following is the most appropriate
treatment?
(A) Add isosorbide dinitrate hydralazine
(B) Add ivabradine
(C) Increase carvedilol dosage
(D) Switch lisinopril to valsartan sacubitril
Item 2
An 84 year old man is evaluated during a routine phys-
ical examination. His only concern is a gradual loss of
stamina and low energy. He can no longer complete his
routine 2-mile run without stopping to rest with a pro
longed recovery time. He has hypertension, for which he
takes lisinopril.
On physical examination, blood pressure is 125/78 mm
Hg and pulse rate is 52lmin. Heart rate is regular. The remain
der of the examination is unremarkable.
Laboratory studies are within normal limits, including
a recent thyroid stimulating hormone measurement.
ECG is shown. Ambulatory 48-hour ECG monitoring
shows a maximum heart rate of 72lmin during vigorous
aerobic activity. An echocardiogram and exercise stress test
performed l year ago for atypical chest pain revealed normal
left ventricular function and no ischemia, with appropriate
augmentation of heart rate.
Which ofthe following is the most appropriate management?
(A) Hospital admission
(B) Pacemakerimplantation
(C) Switch lisinopril to amlodipine
(D) Reassurance and clinical monitoring
Item 3
A 76 year-old man is evaluated in follow-up for a 4 month
history of intermittent claudication, for which he completed
an exercise rehabilitation program. He continues to experience
left lower extremity discomfort with exertion, which has lim-
ited his walking ability. Medical history is significant for hyper
tension and hlperlipidemia. He quit smoking 2 years ago.
Medications are low dose aspirin, atorvastatin, and enalapril.
Serum total cholesterol level is 150 mg/dL (3.8 mmol/L),
serum LDL cholesterol level is 68 mg/dl (1.7 mmol/L), and
serum HDL cholesterol level is 49 mgldL (1.3 mmol/L).
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Which ofthe following is the most appropriate additional
treatment to improve this patient's leg pain?
(A) Cilostazol
(B) Clopidogrel
(C) Evolocumab
(D) Pentoxi[zlline
(E) Vitamin B complex supplementation
Item 4
A 62 year old woman is er,aluated in the emergency depart
ment for acute chest pain that began 90 minutes ago. The
chest pain lasted fbr 45 minutes and then subsided spon
tancously.
'lhe
pain was located across the front of the
chest, did not radiate, and was described as a dull ache. lt
was associated with nausea, diaphoresis, and shortness of
breath. Medical history is notable only for asthma treated
with inhaled mometasone furoate.
On physical examination, blood pressure is 174184 mm
Hg in both arms. pulse rate is 98,min, respiration rate is
24tmin, and oxygen saturation is 957, with the patient
breathing ambient air. Cardiac examination reveals an S.,
but is otherwise normal. Wheezing is present on pulmonary
auscultation.
An initial high sensitivity cardiac troponin level is
equivocal. D dimer measurement is normal.
ECG shows sinus tachycardia with nonspecific S'l'
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wave changes.
The estimated pretest probability of coronary artery
disease is 9'lu.
Which of the following is the most appropriate diagnostic
test?
(A) Adenosine myocardial perfusion imaging
(B) Cardiac magnetic resonance imaging
(C) Coronary CT angiography
(D)'l'ransthoracicechocardiography
Item 5
An 18 year old man is evaluated for a heart murmur noted
on a preparticipation sports examination. He remembers
being told about a murmur in the past, but he has had no
prior testing or intervention. He is asymptomatic. without
known medical problems, and takes no medications.
On physical examination, vital signs are normal. The
estimated central venous pressure is normal. Apical impulse
is normal, and a thrill is noted along the left sternal border.
A grade 4/6 holosystolic murmur is heard at the left sternal
border, obscuring the Sr. The remainder of the physical
examination is unremarkable.
An ECG and chest radiograph are normal.
Which of the following is the most likely diagnosis?
(A) Atrial septal defect
(B) Coarctation ofthe aorta
(C) Patent ductus arteriosus
(D) Ventricular septal def'ect
Item 6
A 57 year-old man is evaluated lbr a 2 month history of
exertional chest pain.
The physical examination. including vital signs. is
normal.
Baseline ECG is normal. During treadmill stress testing,
he der,elops 2 mm horizontal ST segment depression in
leads V, through Vo associated with chest pain that resolves
u,ith rest. Coronary angiographic flndings include a 60'/,,
distal left main bifurcation stenosis u'ith extension into
the proximal left circumllex artery. There is an additional
80',L stenosis involvir-rg the bifurcation of the left anterior
descending artery and large first diagonal branch. The right
coronary artery is large and without signiflcant stenosis.
Left ventriculogram shows global left ventricular dysfunc-
tion with a left ventricular ejection fraction of 42%.
Which ofthe following is the most appropriate management?
(A) Coronaryarterybypassgrafting
(B) Medicaltherapywithout revascularization
(C) Myocardial viability testing
(D) Percutaneous coronan, intenention
Item 7
A 70 year old man is evaluated tbr fatigue and dyspnea with
exertion that have progressed over 1 year. He reports no chest
pain. Medical history is significant for hypertension, hyper
lipidemia, and bilateral carpal tunnel syndrome. Medications
are losartan, hydrochlorothiazide. and atorvastatin.
On physical examination, vital signs are normal. Cen
tral venous pressure is elel'ated. and crackles are heard at
the lung bases. The remainder of the physical examination
is normal.
Laboratory studies show a B type natriuretic peptide
level of 6,10 pg,,ml (640 ngrL).
An ECG is shown (top of next page). An echocardio
gram shows normal left ventricular (LV) cavity size and
moderate concentric LV hypertrophy. The LV ejection frac
tion is 507,. Valve structure and function are normal. Right
ventricular hypertrophy is present. cavity size is normal.
and function is normal. Estimated right ventricular systolic
pressure is 40 mm Hg.
Which of the following is the most appropriate test?
(A) Cardiac magnetic resonance imaging with gadolinium
contrast
(B) Exerciseechocardiography
(C) Rigtrt and left heart catheterization
(D) Serum o galactosidase level
Item 8
A 78 year old woman with progressive exertional dyspnea
and chest pain is evaluated fbr aortic valve replacement.
Results show severe aortic stenosis in the absence of obstruc
tive coronary artery disease. Medical history is signiflcant
lbr hypertension, chronic kidney disease (stage G3a), hyper
lipidemia, COPD, and atrial fibrillation. Medications are
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amlodipine, atorvastatin, Iisinopril, metoprolol, apixaban,
furosemide, and an albuterol ipratropium inhaler.
Her Society of Thoracic Surgeons adult cardiac surgery
risk score is consistent with high risk.
Which of the following is the most appropriate next step in
treatment?
(A) Balloon aortic valvuloplasty
(B) Continued medical therapy
(C) Surgical aortic valve replacement
(D) Transcatheter aortic valve implantation
Item 9
A 21 year old woman is evaluated in the hospital following
cardiac arrcst that occurrecl cluring a collegiate cross country
race. She received cardiopulmonary resuscitation at the scene
and hirs recovered r.t,hile at the hospital. She has no pertinent
personal medical history. The patient t:rkes no medications.
On physical examination, vital signs are normal. The
remainder of the examination is unremarkable.
Laboratory studies are within normal limits.
Eclrocardiogram shows normal left ventricular func-
tion and right ventricular dilation and dysfunction. Results
fiom cardiac catheterization are normal.
Metoprolol is initiated.
Which of the following is the most appropriate additional
management before discharge?
(A) Amiodarone
(B) Genetic testing
(C) lmplantable cardioverter deflbrillator
(D) Lisinopril
Item 10
A 43-year-old man is evaluated during a follow-up visit
for heart failure with reduced ejection fraction (ejec-
tion fraction, 45%) and type 2 diabetes mellitus. He is
currently asymptomatic. Medications are metformin,
valsartan sacubitril, metoprolol, spironolactone, and
atorvastatin.
On physical examination, blood pressure is 108/72 mm
[{g and pulse rate is 64lmin. There is no S.,, jugular venous
distention, or peripheral edema.
Hemoglobin A," level is 6.9')i,.
Which of the following is the most appropriate additional
treatment?
(A) Digoxin
(B) Empagliflozin
(C) Furosemide
(D) Isosorbidedinitrate hydralazine
Item 11
A 70 year old man is evaluated for recently diagnosed par
oxysmal atrial flbrillation that is mildly symptomatic. Med
ical history is significant for hypertension and previous
stroke. Medications are rivaroxaban and metoprolol. He
has experienced no episodes ofbleeding on anticoagulation
therapy.
127

140. Self-Assessment Test
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On physical examination, blood pressure is 128/74 mm
Hg and pulse rate is 72lmin and regular. The remainder of
the examination is unremarkable.
An echocardiogram reveals an enlarged Ieft atrium and
normal left ventricle. Forty-eight-hour ambulatory ECG
monitoring shows atrial flbrillation prevalence of 10% with
a controlled ventricular rate less than 90i min and no other
abnormalities.
Which of the following is the most appropriate treatment?
(A) Left atrial appendage occlusion
(B) Pacemakerimplantation
(C) Rhythm control
(D) Switch rivaroxaban to warfarin
(E) No additional therapy
Item 12
A S7-year-old man is evaluated for a 6-month history of
stable chest pain. He experiences chest pressure that occurs
after walking 1 mile and resolves promptly with rest. He has
no symptoms at rest. His history includes aspirin allergy
manifesting as hives and difficulty breathing after taking
325 mg of aspirin as a teenager. He is a never smoker. He has
hyperlipidemia. His only medication is atorvastatin.
On physical examination, vital signs are normal. BMI
is 30. the remainder of the examination is normal.
Resting ECG is normal, and an exercise ECG is scheduled.
The patient receives counseling on lifestyle interven-
tions to reduce his risk for disease progression. Antianginal
medications will be initiated.
Which of the following is the most appropriate
cardioprotective treatment?
(A) Clopidogrel
(B) Low-dose aspirin
(C) Prasugrel
(D) Ticagrelor
Item 13
A 22 year old woman is evaluated in the emergency depart
ment tbr orthopnea and paroxysntal nocturnal dyspnea.
She is B days postpartum. Delivery was uncomplicated, and
she was discharged home the following day. She has no
other medical problems and takes no medications.
On physical exanlination, blood pressure is 108/82 nrm
tIg in both arms, pulse rate is 112/min and regular, and respi
ration rate is 261min.
'lhe
central venous pressure is elevated,
and an S., and bilateral pulmonary crackles are present.
A chest radiograph shows pulmonary edema. An ECC
reveals sinus tachycarclia without ischemic changes. Trans
thoracic echocardiogram shows left ventricular dilatation
with global hypokinesis; ejection lraction is 38'7,. Right
heart size and flunction are normal.
Which of the following is the most likely diagnosis?
(A) Acute pulmonary embolism
(B) Asccnding aortic dissection
(C) Peripartum cardiomyopathy
(D) Spontaneous coronary artery dissectior.r
Item 14
A 68-year-old man is evaluated in the hospital for a 1 month
history of nonproductive cough, dyspnea. and constant
chest pressure. He is a never-smoker.
On physical examination. blood pressure is 106,62 mm
Hg with 18 mm FIg pulsus paradoxus. and pulse rate is
l001min. Central venous pressure is elevated, and heart
sounds are distant.
A fbcused echocardiogram short,s a 2 cm circumfer
ential pericardial eflusion with evidence of tamponade.
Pericardiocentesis yields 650 ml- of sanguinous fluid with
marked improvement in symptoms.
A transthoracic echocardiogram (apical four chamber
vierv) after pericardiocentesis is shor,r'n (RA
= right atrium,
RV = right ventricle. LV = left ventricle, EFF = pericardial
effusion). A chest CT scan with contrast after pericardiocen
tesis reveals a 4-mm right middle lobe nodule and a small
pericardial effusion with drain in place. A :l x 3 cm right
atrial mass is present, contiguous u'ith the lateral w'all of'
the right atrium.
Which of the following is the most likely diagrosis?
(A) Atrial myxoma
(B) Bronchogenic carcinoma with cardiac metastasis
(C) Cardiacangiosarcoma
(D) Papillaryfibroelastoma
Item 15
A 72'year-old man is hospitalized with decompensated
heart failure. Initial overnight treatment consisted of intra
venous furosemide equal to his total oral outpatient dose
(40 mg). Overnight urine output was 250 mt-, with nochange
in his symptoms. Medical history is significant for hyperlip-
idemia and hypertension. Outpatient medications are lisin
opril, nretoprolol succinate, furosemide. and aton'astatin.
On physical examination, blood pressure is 122 82 mm
Hg, pulse rate is B8/min. respiration rate is 26, min. and oxy
gen saturation is 95'2, with the patient breathing 2 L/min
ot'oxygen by nasal cannula. I{e is alert, and his skin is
warm and dry. Central venous pressure is elevated. Cardiac
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examination does not revcal an Sr. There is pitting edema to
his knees.
Serunr electrolytes are normal, creatinine levcl is
1.5 mg/dl. (132.6
[mol/1,), and B type natriuretic peptide
level is elevated.
Which of the following is the most appropriate treatment?
(A) Add intravenous milrinone
(B) Add intravenous nitroglycerin
(C) Discontinuemetoprolol
(D) Increaseintravenousfurosemide
Item 16
A 60 year old woman is evalulted fbr severe abdominal
pain. She was admitted to the hospital 2 days ago with a
type B aortic dissection and treated medically. ller admis-
sion CT angiogram revealcd an aortic dissection beginning
just distal to the left subclavian artery and extending to the
distal aorta just below the in{erior mesenteric artery. Her
synlptoms responded to morphine, esmcllol, and nitroprus-
side. Yesterclay, esmolol was replaced with metoprolol. She
is taking no other medications.
On physical examination today, temperature is 37.2"C
(99
'F), blood pressure is 120/80 mm Hg, pulse rate is 64lmin,
and respiration rate is 20/min. Oxygen saturation is 9B'X,
with the patient breathing ambicnt air. The patient is rest
less. Despite significant abdominal pair.r. palpation reveais
mild to moderate tenderness and no guarding. The skin is
cool and mottled fiom the knees to the f'eet.
Which of the following is the most appropriate next step in
management?
(A) Add intravenous enalaprilat
(B) [ncreasenitroprussideinfusion
(C) Repair the descending aorta
(D) Switch esmolol to metoprolol
Item 17
A 72 year-old man is evaluated during a routine physical
examination. He feels well and reports no exertional lim-
itations. He has no pertinent personal or family history. He
takes no medications.
On physical examination, pulse rate is 72lmin with
occasional irregularity; other vital signs are normal. On
cardiac examination, heart sounds are regular, with occa
sional premature beats associated with cannon c waves
on neck examination. The remainder of the examination is
unremarkable.
Laboratory studies, including complete blood count
and thyroid stimulating hormone level, are normal.
ECG shows one premature ventricular contraction and
is otherwise normal.
Which of the following is the most appropriate
management?
(A) Cardiac magnetic resonance imaging
(B) Exercise ECG
(C) Metoprolol
(D) Reassurance
Item 18
A 7S-year old man is evaluated for a 3-week history of
shortness of'breath and intermittent f'evers. l{e underwent
transcatheter aortic valve implantation tbr aortic stenosis
3 years ago. He was admitted to the hospital 1 month agtr
with diverticulitis and was treated with antibiotic therapy:
he developed intermittent fevers 1 week later. His only med
ication is low-dosc aspirin.
On physical examination, temperaturc is 37.6 'C
(99.7'F), blood pressureisl4SlT2 mm tJg, and pulse rate is
90/min. Cardiac examination reveals a normal S, and S, and
no murmurs. There is no evidence olheart failure.
An ECC shows no notable findings. A transthoracic
echocardiogram shows a left ventricular ejection fraction
of 55'1, with normal right ventricular function. A biopros
thetic aortic valve is evident, with fully mobile and nornral-
appearing leaflets.
Three sets ofblood cultures are negative.
Which of the following is the most appropriate diagnostic
test?
(A) Cardiac CT
(B) Cardiac magnetic resonance imaging
(C)'1'ransesophageal echocardiography
(D) No further testing
Item 19
A 42-year-old woman is seen to establish care. She is asymp-
tomatic. Medical history is signiflcant only for hypertension,
for which she takes losartan; she has no history of diabetes
mellitus or smoking. She does not take aspirin. Family his
tory is noncontributory.
On physical examination, blood pressure is 118/74 mm
Hg. The remainder of the examination is unremarkable.
Laboratory studies (nonfasting) :
Total cholesterol 24lmgldL (6.24 mmol/L)
HDL cholesterol 99 mgldL (2.s6 mmol/L)
LDL cholesterol 116 mg/dl (3.00 mmol/L)
Triglycerides 128 mg/dl (1.45 mmoUl)
Which of the following is the most appropriate next step in
management?
(A) Begin high intensity statin therapy
(B) Begin high-intensity statin therapy and ezetimibe
(C) Calculate 10 year risk for atherosclerotic cardiovascular
disease
(D) Repeat lipid proflle while fasting
Item 20
A 59 year old woman is evaluatcd in the emergency depart-
ment fbr two episodes of'crushing chest pair.r occurring
at rest during the past 24 hours. The last episode occurred
2 hours agoi she is currently pain free. She has no history
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A 68 year old man is evaluated for a 6-month history of
intermittent claudication. His symptoms have been slowly
progressive but are not life limiting. Medical history is
signiflcant for hypertension, hyperlipidemia, and carotid
artery stenosis treated with left carotid endarterectomy. He
is a former smoker but quit 10 years ago. Medications are
low-dose aspirin, high-intensity rosuvastatin, and losartan.
On physical examination, vital signs are normal. The dor-
salis pedis and posterior tibialis pulses are diminished bilat
erally. Bilateral femoral bruits and carotid bruits are noted.
Ankle brachial index measurements conflrm the diagnosis of
bilateral lower extremity peripheral artery disease.
Laboratory studies reveal a serum LDL cholesterol level
of 56 mg/dl (r.+s mmol/L).
The patient is Jnrolled in a supervised exercise pro
gram.
Which of the following is the most appropriate treatment
to reduce this patient's cardiovascular risk?
(A) Cilostazol
(B) Ezetimibe
(C) Peripheral artery bypass surgery
(D) Rivaroxaban
(E) Ticagrelor
llem 22
A 56 year-old man is evaluated during a follow up visit
for ischemic cardiomyopathy (ejection fraction. 20'7,). He
has had lwo heart failure hospitalizations in the past year.
He can no longer perform household chores. He also has
hypertension and chronic kidney disease. with a baseline
serum creatinine level of 1.5 mg/dl (132.6
Umol/L).
He is
receiving maximally tolerated heart failure therapy consist
ing of losartan, carvedilol, furosemide, and spironolactone.
Physical examination flndings, including vital signs,
are normal.
Serum creatinine level is 1.9 mg/dl (168 pmol/L),
serum potassium level is 5.3 mEq/L (5.3 mmolil). and
serum sodium level is 129 mEq/L (129 mmol/L).
The patient wishes to pursue care that will restore
functionality.
Which ofthe following is the most appropriate long-term
treatment?
(A) Digoxin
(B) Hearttransplantation
1
130

143. Self-Assessment Test
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(C) Left ventricular assist device placement
(D) Tolvaptan
Item 23
A 48 year old man is evaluated for a 6 month history of
dyspnea and near syncope with exertion. He has no other
medical problems and takes no medication.
On physical examination, vital signs are normal. A grade
3/6 systolic ejection quality murmur is heard along the left
sternal border. The carotid impulse is brisk. The murmur
decreases significantly in intensity with the patient squatting.
There is no radiation of the murmur to the carotid arteries. No
ejection sound or systolic click is heard. There is no variation
with respiration, and no diastolic murmur is present.
Which of the following is the most likely diagnosis?
(A) Bicuspid aorlic slenosis
(B) Hypertrophic cardiomyopath)'
(C) Restrictive membranous ventricular septal defect
(D) Ruptured sinus of Valsalva aneurysm
Item 24
A :l:l ycxr olcl r,r,onrrur is hospitalized firr acute crusl.ring
st-tbsternal chesf pain, which occurreci n,hile jogging irnd
resolvecl r.r.ith rest. The pain wirs associated r,r,ith nausea lrnd
cliaphoresis. Shc hirs hacl several previous cpisodes of'cl.rest
pirin u,ith activitv. rrlthough thc pain r.r'as lcss se.',ere. She is
ll nronths postpilrtum front an uncomplicaterl spontancous
vaginrrl delirrery.
ln the enrergency departnrcnt. bkrod prcssure \/rs
1O.1 76 nrm Hg. an<l pr-rlse rltc urls lll nrin. lhe rentlintlcr
of the erarniniition u,as unrernirrklble .
(lirrcliirc cathete rizrtion ret ells normal coronan' arter
ie-s. Aclnri nistratiolr of i ntriicoroltxr)' n i t roglyc'eri rl shott's nrr
change in corclnary xrtcry diantctcr.
(-irrdiirc nragne tic rcsoltilncc inragir-rg finclings short,
clillusc pcrtusion lbnornralities.
Which of the following is the most likely cause of this
patient's chest pain?
(A) Coronary' \'ilsosl)rlsn1
(B) N{icroi,asculirr il!stirnction
(C) l)eripartum crrclionrl'opathy
([)) Spontaneolls coronary artery clissection
Item 25
A 42 year old wonran is evaluated in the emergency
department for abrupt-onset palpitations. For the
past year, she has been having similar episodes with
increasing frequency. She has been able to stop these
previous episodes by coughing. She reports no syncopal
episodes.
On physical examination, blood pressure is 95/68 mm Hg.
pulse rate is 200/nrin, and respiration rate is l6lmin. AII
other physical exanrination findings are unremarkable.
ECG is shown. A previous ECG showed sinus rhythm
with ir heart rate ot'72lmin without any abnormalities.
Which of the following is the most likely rhythm
diagnosis?
(A) Atrial fibrillation
(B) Atrial tachycardia
(C) Atrioventricularnodalreentranttachycardia
(D) Ventriculartachycardia
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Item 26
A 65-year-old woman is evaluated in follow-up for a recent
diagnosis of nonischemic cardiomyopathy. Her symptoms
included dyspnea with minimal exertion and orthopnea.
The initial evaluation also revealed echocardiographic flnd-
ings compatible with severe secondary mitral regurgitation
(mitral annular dilation without any mitral leaflet abnor-
malities) and a left ventricular ejection fraction of 4O%.
Image quality was excellent. Lisinopril, carvedilol, spirono-
lactone, and furosemide have been titrated to achieve max-
imal therapeutic effectiveness. Her symptoms are now New
York Heart Association functional class I.
On physical examination, blood pressure is 95/62 mm
Hg and pulse rate is 7Olmin; other vital signs are normal. A
holosystolic murmur is loudest at the apex. Central venous
pressure is slightly elevated. Lungs are clear.
Which of the following is the most appropriate additional
management?
(A) Cardiac magnetic resonance imaging
(B) Cardiac resynchronizationtherapy
(C) Surgical mitral valve repair
(D) No additional therapy
Item 27
A 42 year old man is evaluated during a follow-up visit for
a patent ductus arteriosus (PDA) identified early in life. He
had regular follow-up visits through adolescence but has
not had a medical evaluation recently. He is asymptomatic.
On physical examination, blood pressure is 120/70 mm
Hg. Apical impulse is displaced laterally. A continuous mur-
mur that envelops the S, is heard beneath the left clavicle.
The remainder of the cardiac examination is unremarkable.
Echocardiogram demonstrates a PDA with left-to-right
shunt. The left atrium and left ventricle are moderately
enlarged, and the left ventricular ejection fraction is 63%.
The right heart chambers are normal in size. The estimated
right ventricular systolic pressure is 30 mm Hg.
Which of the following is the most appropriate
management?
(A) Cardiac magnetic resonance imaging
(B) Indomethacin
(C) PDA device closure
(D) Serial echocardiographicmonitoring
Item 28
A 74 year-old man is evaluated for a 6-month history of
progressive bilateral calf pain. The discomfort is worse with
walking and improves quickly with rest. Medical history is
signiflcant for hypertension and hyperlipidemia. He has a
S0-pack year history of smoking but quit 5 years ago. Med-
ications are rosuvastatin, quinapril, and metoprolol.
On physical examination, vital signs are normal. A
right femoral bruit is noted. Bilateral femoral pulses and
pedal pulses are faint. Motor and sensory examinations are
normal; reflexes are normal.
The ankle-brachial index is 0.92 on the right and 0.94
on the left.
Which of the following is the most appropriate test to
perform next?
(A) Exercise ankle brachial index testing
(B) Invasiveangiography
(C) Magneticresonanceangiography
(D) Segmental lower extremity blood pressure testing
Item 29
A 57 year-old woman is evaluated in the emergency
department for an acute anterior ST-elevation myocardial
infarction.
Aspirin, ticagrelor, unfractionated heparin. and intra
venous nitroglycerin are initiated. Emergent coronary
angiography reveals an acutely occluded proximal left
anterior descending (LAD) coronary artery and 70'U, stenosis
of the mid right coronary artery. The LAD lesion is treated
with drug eluting stent placement, resulting in resolution
of chest pain and improvement in the ST-segment elevation
seen on ECG.
Following successful percutaneous coronary interven
tion, blood pressure is 138/82 mm Hg, pulse rate is 7Srmin.
respiration rate is l8/min, and oxygen saturation is 94'7,
with the patient breathing ambient air. The remainder of the
examination is unremarkable.
Which of the following is the most appropriate additional
management?
(A) Glycoprotein IIb/llla inhibitor infusion
(B) Predischarge exercise ECG
(C) nignt coronary artery revascularization
(D) Supplementaloxygen
Item 30
A 4l-year-old man is evaluated for a 2 week history of
daily palpitations that last minutes at a time and resolve
spontaneously. The palpitations are not associated with
any specific activity. He is otherwise healthy and takes no
medications.
Physical examination flndings, including vital signs,
are normal.
Which of the following is the most appropriate initial test?
(A) Exercise ECG
(B) 30-Day event monitor
(C) rz Lead resting ECG
(D) 24-Hour ambulatory ECG monitor
Item 31
A 34-year-old woman is evaluated for exertional dyspnea
when walking up stairs or carrying groceries from the
car. Six months ago, she was diagnosed with heart failure
1 month after her last pregnancy. She has no traditional
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132

145. Self-Assessment Test
atherosclerotic risk factors. Medications are valsartan
sacubitril, carvedilol, furosemide, and spironolactone. The
patient is Black.
On physical examination, blood pressure is 138/76 mm
Hg and pulse rate is 64lmin. The remainder of the examina-
tion is unremarkable.
Laboratory studies are within normal limits.
Repeat echocardiogram shows an ejection fraction of
25"/,,.
Which of the following is the most appropriate treatment?
(A) Digoxin
(B) Isosorbide dinitrate-hydralazine
(C) Liraglutide
(D) Lisinopril
Item 32
A 56 year-old man is evaluated in the emergency depart-
ment for acute shortness of breath and the sensation of a
racing heart. His only medical problem is hypertension
treated with chlorthalidone.
On physical examination, blood pressure is 89/52 mm
Hg and pulse rate is 150/min. Cardiac examination reveals a
regular tachycardia. Jugular venous distention and pulmo
nary crackles are present.
ECG is shown. The patient is successfully cardioverted.
Which of the following is the most appropriate additional
treatment?
(A) Amiodarone
(B) Catheter ablation
(C) Flecainide
(D) Metoprolol
(E) No additional treatment
I
Item 33
A 68-year-old man is evaluated I month after atherectomy
and stenting ofthe right superflcial femoral artery for severe
claudication. Since the procedure, he can walk and perform
all of his customary activities without claudication. Medical
history is signiflcant for hypertension, hyperlipidemia, and
coronary artery disease. He exercises 150 minutes weekly
and consumes a heart healthy diet. He quit smoking 15 years
ago. Medications are low dose aspirin, low-dose rivarox-
aban, metoprolol, ramipril, and high intensity rosuvastatin.
On physical examination, blood pressure is 124i70 mm
Hg. The remainder of the physical examination is unre
markable.
Laboratory studies reveal a serum total cholesterol level
of 120 mg/dl (3.1 mmol/L), serum LDL cholesterol level of
50 mg/dl (t.s mmol/L), and serum HDL cholesterol level of
4smgldL (t.zmmol/L).
Which of the following is the most appropriate additional
treatment?
(A) Cilostazol
(B) Evolocumab
(C) Ticagrelor
(D) No additional treatment
Item 34
A 47-year old man is evaluated for a heart murmur. He is
asymptomatic and has no exercise limitations.
On physical examination, vital signs, including blood
pressure, are normal. There is a grade 1/6 decrescendo dia-
stolic murmur heard at the left sternal border. The remain-
der of the examination is normal.
Echocardiogram shows a left ventricular ejection frac-
tion of 557,, a bicuspid aortic valve with mild aortic regurgi
tation, and a normal left ventricular end systolic dimension'
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The ascending aorta is enlarged, with a dimension of 4.2 cm.
Echocardiographic imaging quality of the aortic sinuses,
sinotubularjunction, and ascending aorta is excellent.
Which of the following is the most appropriate
management?
(A) Aortic valve replacement and repair of the ascending
aorta
(B) Cardiac magnetic resonance imaging
(C) Echocardiographicsurveillance
(D) Initiation of losartan
Item 35
A 48 year old man is evaluated for recurrent pericarditis.
Six months ago, he had acute pericarditis treated with ibu
profen and colchicine. His symptoms resolved completely
within 3 weeks of initiation of therapy. Evaluations for an
infectious cause and connective tissue disease were nega
tive. The patient's symptoms recurred after ibuprofen was
tapered over 1 month with continuation of colchicine. Ibu
profen was re initiated at a high dose with resolution of
symptoms and tapered over a 2 month period. His current
symptoms began 24 hours ago. Currently, his only medica
tion is colchicine.
On physical examination, temperature is 38.0 'C
(100.4
"F); other vital signs are normal. Pulsus paradoxus of
10 mm Hg is present. There is no jugular venous distention.
The lungs are clear to auscultation. A friction rub is heard at
the left sternal border and apex.
ECG shows normal sinus rhythm with widespread
ST-segment elevation of 0.5 to 1.0 mm. Echocardiogram
shows a small circumferential pericardial effusion (diastolic
echo-free space, 3 mm) without evidence of tamponade.
Which of the following is the most appropriate treatment?
(A) Anakinra
(B) Colchicine and intravenous immune globulin
(C) Ibuprofen
(D) Ibuprofen, colchicine, and prednisone
Item 36
A 60 year old woman comes to the office for follow up
evaluation 1 year after having a drug-eluting stent placed
in the mid left anterior descending coronary artery to treat
non ST-elevation acute coronary syndrome. She has been
adherent to dual antiplatelet therapy for the past year with
out any bleeding events. The patient's only other medical
problem is diabetes mellitus. Medications are metformin.
liraglutide, atorvastatin, clopidogrel, and aspirin.
Which of the following is the most reasonable
management of the antiplatelet therapy?
(A) Stop aspirin
(B) Stop aspirin and clopidogrel
(C) Stop clopidogrel and add low-dose rivaroxaban
(D) Continue aspirin and clopidogrel
Item 37
A 6l y'ear old man is evalurted in the emergencl, depart
ment fbr palpitations that began this morning. He has a
history of coronary artery disease treated rt'ith coronary'
artery stenting lB months ago and left ventricular d_v''sfunc
tion. Medications are aspirin, metoprolol. spironolactone.
and lisinopril.
On ph1'sical eramin:rtion, blood pressure is 100 65 nrm
IIg and pulse rate is 130'nrin: other vital signs are normal.
Oxygen saturation is 98')1, lvith the patient breathing ambi
ent air. Intermittent cannon o waves are noted on neck
exanrination. Other than tachycardia. cardiac examinati<tn
is normal. Breath sounds are clear.
ECG rvith rhythm strips is shown (top of nert page).
Which of the following is the most likely diagnosis?
(A) Monomorphicventriculartachl'cardia
(B) Preexcitedtachycardia
(C) Suprirventricular tachy'cardia n'ith aberrano'
(D) Torsades de pointes
Item 38
A 47 year old man is evaluated for management of heart
failure with reduced ejection fraction (ejection fraction,
30'7,) diagnosed 3 years ago. He has New York Heart Asso
ciation functional class III symptoms and has been stable
for the past year. His medical history also includes ACE
inhibitor-induced angioedema, spironolactone-induced
gynecomastia, and atrial fibrillation. An implantable
cardioverter defibrillator is in place. Medications are
losartan, carvedilol, furosemide, and apixaban.
On physical examination, blood pressure is 120/68 mm
Hg and pulse rate is 7Srmin and irregular. Other than an
irregularly irregular heart rhythm, the remainder of the
examination is normal.
Serum creatinine and electrolyte levels are normal.
Which of the following is the most appropriate treatment?
(A) Add eplerenone
(B) Add ivabradine
(C) Switch carvedilol to metoprolol succinate
(D) Switch losartan to valsartan-sacubitril
Item 39
A 45 year old man is seen for cardiovascular risk reduction.
Hyperlipidemia was diagnosed 4 years ago and is treated
withatorvastatin. t{ehasa35 pack yearhistoryofcigarette
smoking and is a current smoker. Six months ago, predia
betes was diagnosed. He occasionally has a depressed mood.
He does not take aspirin on a regular basis.
On physical examination, vital signs are normal. BMI
is 29. The remainder of the examination is unremarkable.
Which of the following is the most appmpriate management?
(A) Depression screening and treatment
(B) Low dose aspirin
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147. Self-Assessment Test
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(C) Smoking cessation counseling and varenicline
(D) Weight loss
Item 40
A 56 year-old woman is evaluated before starting treatment
with trastuzumab fbrearly stage HER2 positive breast cancer.
She has no cardiovascular symptoms and has no exercise
related limitations. She has hypertension and hyperlipidemia.
Medications are losartan and atorvastatin.
On physical examination, vital signs and other flndings
are normal. The breast surgery site has healed.
Results of routine laboratory studies are normal.
Findings on echocardiogram are normal. Left ventri
cular ejection fraction is 55',1,.
Which of the following is the most appropriate cardiac
surveillance for this patient?
(A) Cardiac magnetic resonance imaging
(B) Echocardiography
(C) Multigated acquisition (MUGA) scan
(D) No surveillance
Item 41
A 69 year-old man is evaluated for persistent angina
despite maximally tolerated antianginal therapy. Symp
toms appear alter walking less than one half mile and
interfere with his quality of life and occupation as a mail
carrier. He has no pain at rest or heart lailure symptoms.
He f requently experiences light headedness when arising
from a seated position. He has a 20 pack year history of
smoking but stopped 25 years ago. Medications are aspirin,
metoprolol, sublingual nitroglycerin, and rosuvastatin.
On physical examination, blood pressure is 108/72 mm
Hg, pulse rate is 54/min, and respiration rate is 20i min. The
remainder of the physical examination is normal.
An ECG shows sinus rhythm. A chest radiograph is
normal.
Which of the following is the most appropriate
management?
(A) Addition of clopidogrel
(B) Addition of isosorbide mononitrate
(C) Coronaryangiography
(D) Exercise echocardiography
Item 42
A 70 year old man is referred fbr evaluation after a 6.1 cm
abdominal aortic aneurysm was discoverecl on routine
screening ultrasonography. Medical history is significant fbr
hypertension and hyperlipidemia. lle has a 50'pack-year
history of cigarette smoking, stopping 6 years ago. Medic:r
tions are rosuvastatin and chlorthalidone.
On physical examination, vital signs are normal. BMI
is 28. A bruit is heard over the abdomen, and a pulsatile
abdominal mass is present to the left of the midline.
Which of the following is the most appropriate next step in
management?
(A) Abdominalaortography
(B) ACE inhibitor therapy
(C) CT angiography
(D) Open abdominal aortic aneurysm repair
(E) Repeat duplex ultrasonography in 6 months
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Item 43
A S5-year old woman is evaluated for a 6-month history
of progressive fatigue and dyspnea while walking on level
ground.
On physical examination, blood pressure is normal and
pulse rate is BO/min. Cardiac examination reveals an open
ing snap and a diastolic rumble heard best at the cardiac
apex. Estimated central venous pressure is normal.
A resting echocardiogram shows a left ventricular
ejection fraction greater than 55% and a normal size right
ventricle with preserved function. The mitral valve is thick
ened and appears rheumatic, with restricted opening of the
leaflet tips. The mitral gradient and calculated valve area are
consistent with moderate mitral stenosis.
Which of the following is the most appropriate
management?
(A) Exerciseechocardiography
(B) Cardiaccatheterization
(C) Cardiac magnetic resonance imaging
(D) Percutaneousballoon mitralcommissurotomy
(E) Transesophagealechocardiography
Item 44
A74 year old man is evaluated in the emergency depart
ment fbr somnolence. He resides in a skilled nursing lacility.
His transfer note indicates that he has moderately severe
Alzheimer disease treated with donepezil.
On physical examination, bktod pressure is 70i 40 mm
tlg and pulse rate is 30/min; other vital signs are normal.
Oxygen saturation with the patient breathing ambient air is
97"/,,.The patient is diflicult to arouse. Cardiac eramination
reveals bradycardia but is otherwise unremarkable.
Complete blood count and electrolyte levels are nor
ma[.
Cardiac telemetry shows sinus bradycardia with heart
rate ol30/min.
Which of the following is the most appropriate treatment?
(A) Amiodarone
(B) Chest compressions
(C) lntravenousatropine
(D) Isoproterenol
Item 45
A 78 year old woman is evaluated for a 3-month history of
heart failure with reduced ejection fraction (ejection frac
tion, 20%). She has stable dyspnea when walking up stairs
but has no other symptoms. Her medical history is other
wise unremarkable. Medications are valsartan-sacubitril,
carvedilol, furosemide, and spironolactone. Carvedilol is at
half maximum dosage; all other medications are at maxi-
mum recommended dosages.
On physical examination, blood pressure is 118/74 mm
Hg and pulse rate is 88/min. BMI is 27, unchanged from her
last visit. Central venous pressure and the remainder of the
examination are normal.
Which of the following is the most appropriate tre"atment?
(A) Add ivabradine
(B) Decreasevalsartan-sacubitril
(C) Increasecarvedilol
(D) Increase furosemide
Item 46
A 75 year old man is evaluated in the emergency depart
ment for a 2 day history of left leg pain. The pain started
abruptly and has been constant. He has diabetes mellitus.
hypertension, peripheral artery disease, and hyperlipid
emia. Medications are metfbrmin. chlorthalidone. atonla
statin. cilostazol. and lor,v dose aspirin.
On physical examination, pulse rate is 108'min and
irrcgular: other vital signs are normal. Cardiac eramina
tion reveals an irregularly irregular rhythm. The left lon'er
extremity is cold and mottled. The lelt popliteal and ankle
pulses are absent. Passive range ol motion is normal in the
left leg. but the patient has significant pain and is unable to
actively flex the left knee lully. Left leg sensation is intact.
The remainder of the physical examination is unremark
able.
ECG shows atrial flbrillation.
Which of the following is the most appropriate next step in
management?
(A) Angiography
(ts) Apixaban
(C) Intravenoustenecteplase
(D) Unlirrt'tionated heparin
(E) Venous duplex ultrasonography
Item 47
A 28 year <,rldwoman is evaluated in the emergency depart
mcnt for substernal chest tightness that started several hours
ago. She is 2 weeks postpartum after an uncomplicated preg
nancy and delivery. She has no history of cardiopuhnonary
disease and has no risk factors for atherosclerotic cardiovas
cular disease.
On physical examination. blood pressure is 122i72 mm
I Ig in both arms. pulse rate is 90i min and regular. and respi
ration rate is 24'min. The estimated central venous pressure
and apical impulse are normal. An S.r is present. The rest of'
thc examination is unremarkable.
Laboratory studies are significant for an elevated
high sensitivity cardiac troponin level (>99th percentile
upper ref'erence limit).
An ECG shou,s ST segment elevation in the anterior
precordial leads. An echocardiogram shows anterior wall
hypokinesis. Estimated ejection fiaction is,10'iI,.
Which of the following is the most likely diagnosis?
(A) Atheroscleroticplaque rupture
(B) Peripartum cardionryopathy
(C) Spontaneous coronary artery dissection
(D) Stress induced (takotsubo) cardiomyopathy
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149. Self-Assessment Test
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Item 48
A 42 year old man is evaluated in tl-re emergency deparl
ment lbr sudden onset right sided weakness. His symp
tonrs resolved fully over the past hour. Ilc has no knowr.r
medical problems ar.rd takes no meclications.
On pl.rysical examination, vital signs are norntal. llis
heart rhythm is regular. Ner-rrologic cxamination is normal.
No carotid bruits <.rr heart murmurs are noted.
Laboratttry stud)/ results are normal.
Magnctic resonance angiogram of the brain demon
strates a snrall left sided ischemic stroke but no other
iesions. ECC, carotid ultrasouncl, telemetry, and lower
extremity ultrasouncl are nrtnnal. A transesophagcal echo
cardiogranr demonstrates a patent fbramen ovale (PFO)
with right to left shunt noted with cough and Valsalva
release. No other abnormalities are identified.
Which of the following is the most appropriate
management?
(A) Aspirin
(B) PIrO device cl<tsure
(C) Warf arin
(D) Observation
Item 49
A 49 year-old woman is evaluated during a new patient
visit. She has no medical problems or concerning symptoms
and takes no medications.
On physical examination, vital signs are normal. The
S, is diminished, and there is an aortic ejection click shortly
after the S,. The S, is normal. There is a grade 216,high-
pitched, decrescendo early diastolic murmur heard best at
the left sternal border. The murmur is accentuated when the
patient leans forward.
Which ofthe following is the most appropriate diagnostic test?
(A) Cardiac catheterization
(B) Cardiac magnetic resonance imaging
(C) Stressechocardiography
(D) Transesophagealechocardiography
(E) Transthoracicechocardiography
Item 50
A 42 year old man is evaluated for dyspnea that occurs
while walking on a flat surface and causes him to stop
when walking uphill. He has hypertrophic cardiomyopa,
thy, which was diagnosed at age 38 years. He has no other
symptoms and has no family history of sudden cardiac
death. Medications are metoprolol and disopyramide.
An echocardiogram shows asymmetric septal hyper
trophy with maximal septal thickness of 24 mm. Systolic
anterior motion of the mitral valve is present with dynamic
left ventricular outflow tract obstruction and peak gradient
of 64 mm Hg at rest.
Six months ago, 24-hour ECG monitoring performed
as part ofsurveillance showed sinus bradycardia and single
premature ventricular contractions averaging 6 per hour.
Which of the following is the most appropriate treatment?
(A) Additionofvalsartan-sacubitril
(B) Implantable cardioverter-deflbrillator therapy
(C) Replacement of metoprolol with carvedilol
(D) Septal reduction therapy
Item 51
A 73 year old m:rn is evaluated in the emergency depart
ment for a 2 hour history ol crushing chest pain and dia
plroresis. An admission EC(l is shown.
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A drug eluting stent is placed in the proximal right cor
onary artery, and he is transf'erred to the coronary care unit.
On physical examination, blood pressure is 80/50 mm
Hg. pulse rate is 120/min, respiration rate is 22lmin, and
oxygen saturatior.r is 94'X, rvith the patient breathing ambient
air. An S., is heard; there is no pericardial rub. Central venous
pressure is clevated.'lhe lungs are clear to auscultation.
Which of the following is the most appropriate treatment?
(A) Intra aortic balloon pump placement
(B) lntravenous dobutanrine
(C) [ntravenous 0.9'], saline
(D) Oral metoprolol
Item 52
An B4-year old man is evaluated before hospital discharge
following an exacerbation of heart failure. Ile has an
implantablc cardioverter deflbrillator. Medications are lis
inopril, lurosemicle, carvedilol, and spironolactone. I)uring
hospitalization, the patient received intravenous Iurose
micle at a dosage twice that of his home oral dosage.
On physical examination. vital signs are normal. BMI
is 28. Centralvenous pressure is not elevated.
'lhere
is no S.,,
and there are no pulmonary crackles.
Serum creatinine level has returnecl to the baseline
level, and clectrolytes are normal.
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Echocardiogram from this hospitalization shows
EJ ejection fraction of 25'1, rn'ith left ventricular end cliastolic
coNi
dimension of 72 mm.
Which of the following is most likely to prevent early
hospital readmission in this patient?
(A) Echocardiograpl.ry in 3 rnor.rths
(B) Follorn, up officc visit in 30 days
(C) Folkrw up telephone call in 2 clays
(D) Decrease in turoscmide to original hclme dosage
Item 53
A 75 year old man is evaluated for dyspnea and an episode
of exertional syncope. He is diagnosed on clinical examina-
tion with aortic stenosis.
An ECG shows normal sinus rhythm and left ventricular
hypertrophy with repolarization abnormalities. The echo
cardiogram reveals a severely thickened, minimally mobile
tricuspid aortic valve compatible with severe aortic stenosis.
However, hemodynamic data from echocardiography show
a mean aortic gradient and aortic valve area consistent with
moderate aortic stenosis. Left ventricular ejection fraction is
greater than 55%, and stroke volume index is normal.
Which of the following is the most appropriate next step in
management?
(A) Cardiac catheterization
(B) CToftheaorticvalve
(C) Exercise stress testing
(D) Surgical aortic valve replacement
(E) Transcatheter aortic valve implantation
Item 54
A 70 year old woman is evaluated in follow up for peripheral
artery disease. She walks 2 miles daily, and her symptoms
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ITEM 55
fbrce her to stop several times. Medical history is otherwise
signiflcant for hypertension and hyperlipidemia. She has a
50 pack year smoking history but quit 10 years ago. Medi
cations are aspirin, cilostazol, lisinopril, and amlodipine. She
was prescribed atorvastatin, but she took the medication for
only 1 week after reading that muscle ache is an adverse effect.
Which of the following is the most appropriate treatment?
(A) Atorvastatin
(B) Colestipol
(C) Ezetimibe
(D) Icosapent ethyl
Item 55
A 45-year old woman is evaluated in the office for palpita
tions. She has no other symptoms at rest or with exertion.
The palpations make her anxious and are intolerable. They
seem to subside with exercise and at night. She does not
smoke cigarettes or drink alcohol or caffeinated beverages,
and she has no other pertinent personal or family history.
She takes no medications.
On physical examination, pulse rate is 65/min and
irregular; other vital signs are normal. Heart sounds are reg
ular, with premature beats associated with cannon o waves
on neck examination. The remainder of the examination is
unremarkable.
Laboratory studies, including complete blood count
and thyroid stimulating hormone level, are normal. Preg
nancy test results are negative.
ECG is shown. Echocardiogram is normal.
Whichofthe following is the most appropriate management?
(A) Amiodarone
(B) Exercise ECG
(C) Propranolol
(D) Reassurance
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151. Self-Assessment Test
Item 55
A 35 year-old man is evaluated for exertional dyspnea. His
history is otherwise unremarkable.
On physical examination, vital signs and oxygen sat-
uration are normal. Central venous pressure is elevated.
A left parasternal impulse is present. A grade 2/6 systolic
murmur is heard at the second left intercostal space, and
a diastolic flow rumble is heard at the left sternal border.
Fixed splitting of the S, is present. The remainder of the
physical examination is normal.
An ECG demonstrates sinus rhythm with right axis
deviation and incomplete right bundle branch block. A
transthoracic echocardiogram demonstrates a 1.5 cm
ostium secundum atrial septal defect, with moderate right
heart enlargement. Left ventricular cavity size and function
are normal. the estimated right ventricular systolic pressure
is 30 mm Hg.
Which of the following is the most appropriate
management?
(A) Atrial septal defect closure
(B) Cardiopulmonaryexercisetesting
(C) Coronaryangiography
(D) Echocardiographicsurveillance
Item 57
A 71-year-old man is evaluated fbr a 6-month history of
exertional chest pain. lhe pain has incre:rsed in {iequency
and now occurs earlier during his exercise regimen. Jhe
pain is relieved by subiingual nitroglycerin. He underwent
coronary artery bypass gralt surgery 4 years ago. History is
also signiflcant fbr hypertension and hyperlipidemia. Medi-
cations are metoprolol, Iisinopril, atorvastatin, and aspirin.
Physical examination findings, ir-rcluding vital signs,
are normal.
ECG shows left bundle branch block.
Which of the following is the most appropriate test?
(A) Dobutamine echocardiography
(B) Exercise ECG
(C) Exercise single photon emission CT
(D) Vasodilator single-photon emission CT
Item 58
A74 year old manisevaluatedinthehospitirl fbra 6 month
history ofprogressive fatigue and exertional dyspnea. along
with increasing peripheral edema and abdominal girth
over the past 3 months. IJe also has corouary artery dis
ease, fbr which he had a coronary artery bypass gral't at age
62 years. Medications are metoprolol, 1ow-dose aspirin. and
atorvastatin.
On physical examination. vital signs are normal. Jugtt
lar venous distention with prominent waveforms is noted'
There is no discernable fall in the central venous pressure
during inspiration. An early diastolic sound is present.
The liver is enlarged and pulsatile. Ascites is present, and
peripheral edema extends to the knees bilaterally.
Ou chesl. raclkrgrullh, stcnrotoltl uilcs :rnri vasci-rhr
clips are seen. irnd snrall bihte rai plcr.rr:li cf lirsious l"c pn se n1"
Which of the following is the most likell, cliagnosisl'
(A) Cardiac tirmponrlclc
(B) Chronic live r diseasc
(C) Constrictive pericartlitis
(D) Itestrictive crrrtlior.nvop;rthv
Item 59
A 65 year olcl ntatr is evlltt;t l er-l li ir rr 2 ti:tr' lr ir t r.rr_l. ol srlcrlr l
episocles of chest clisctltrt[irrt rrntl d1,s1lr-rc';r ()!:ci.lri'in! iti-,lh ;l
rr'st and n'itlt e.rcrtion. I'orlir-r, lrr prtjsr,rrn rr-!lh :i holrrs rrl'
persislerll severt' ccntral ehi:sl prrssurc.
On ph1'sicaIerantirtlIion. Irlood Jr-r-,ssrilr.ii I55 9{.} il}lt
IIg. pttlse rille is 9(-).n1in. r'csllirrrlion nilc is l.l(l rnin.:,urri
oxygen saturdtioll is 9:l'i, \'ilh thc l)rtir,11l brcething ri;thi
ent air. UNll is 29. . S, is prcscnt. llul llrr rrri.rilritr-ll'1lrc
carcliac erarninlliorr is nonn;rl.
liigh scnsitivilr' clrrli.rc l roplnin lt'r'rl ir ,,'lr:r';rtr'el
(>991h percenlile trppe r rclcrcrrce limil).
An L-(l(l is shou'u.
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He is given lspirin. c'L4rickrgrcl. ilrtr.l\j(rlotls illtfi-tL
tionated heparitr. lncl a nitt-ogl_\'ccrir.l iillllsi{rtt, llir t'hi'st
pain initiirlll. inrprovcs hut rt'turns dr:piie tiilrt:dl-r'L:i'ritt
infusion ancl rnorph i ne.
Which of the following is the most appropriate managenrent?
(A) t'.cltoc:trcliographl'
(13) F.plerenone
(C) O.xygen pcr ttrrsal cal11ltll.t
(D) Urgent (orollilr! angir.,grrplr-'"'
Item 60
A 26-year-old woman is evaluated for palpitations that
began 2 months ago. The palpitations begin with no speciflc
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inciting factors, Iast 5 to 15 minutes, and are alleviated by
rest or deep breathing. Ihey occur a few times per week.
Between events, she engages in usual daily activities with
no limitations. She has no other pertinent history and takes
no medications.
Physical examination flndings, including vital signs,
are normal.
Resting l2-lead ECG shows sinus rhythm and no
abnormalities.
Which of the following is the most appropriate diagnostic
testing option?
(A) Exercise ECG
(B) Implantable loop recorder
(C) Mobile cardiac telemetry
(D) 30-Day event monitor
Item 61
A 76 year old woman is evaluated in the en-rergency depart
menl lor acute onset substernal chest discomfort with asso
ciated dyspnea. The symptoms developed tl-ris morning after
her husband's unexpected death. She has hypertension
treated with chlorthalidone.
Or.r physical examination, blood pressure is 140/70 mn.t
llg and pulse rate is 90/min. Oxygen saturation is 9B'1, with
the patient breathing ambicnt air. Other than an S.,, the
carcliopulmonary examination is norma[.
Serum high sensitivity cardiac troponin level is ele
vatecl (>99th percentile upper reference limit).
An ECG demonstrates S'l' segment elevation in the
irnterior precordial leads. ancl echocardiogram shows
preserved basal left ventricular flnction with apical and
rnicl ventricular hypokinesis. The lefl ventricular ejection
fraction is 35',1,. Valve function and the ascending aorta
appear normal. Coronary ar.rgiography shows normal cor
()nrry arteries.
Which of the following is the most likely diagnosis?
(A) Ascending aortic dissection
(B) Coronary artery dissection
(C) Coron:rry artery embolism
(l))'l'akotsubo cardiomyopathy
Item 62
A 5,1 year old man is evaluated in the emergency depart
mcnt 2 hours after the orrset of acute, sharp, central chest
pain that radiates to the back and has intensified over time.
l le has hypertension. lle cluit smoking 2 years ago. Medica
Iions are hydrochlorothiazidc and lisinopril.
On physical examination. blood pressure in the right
rlrnl is 90,,56 mm Hg. blood pressure in the left arm is not
obtainable, and pulse rate is l20r'min. He is confused and
ciiaphoretic. Cardiac examination reveals a summation gal
lop ancl a grade 3i 6 diastolic murmur at the right upper
stenral border. Puln-ronary examination reveals crackles.
Itadial pulses are weakly prcsent on the right and absenl on
the left. l-ower extremity pulses are diminished. Skin is cool
and mottled.
Chest radiograph reveals a widened mediastinum, and
a C'l'angiogram shows a type A aortic dissection.
Which of the following is the most appropriate treatment?
(A) Coronaryangiography
(B) Intravenous nitroprussicle
(C) Open aortic repair
(D) thoracic endovascular aortic repair
Item 63
A 45 year-old woman is evaluated for occasional palpita-
tions. She also has hypertension. Medications are chlortha
lidone and diltiazem.
On physical examination, vital signs are normal. Car
diac examination reveals an irregular rhythm. There is a
midsystolic click and late systolic murmur at the apex, radi
ating to the back. There are no signs ofheart failure.
An ECG shows atrial flbrillation. with a ventricular rate
of 80/min.
A transthoracic echocardiogram shows severe poste-
riorly directed mitral regurgitation, with a left ventricular
ejection fraction of 557, and left ventricular end-systolic
dimension of 60 mm. There is severe anterior mitral valve
prolapse; hemodynamic measurements indicate severe
mitral regurgitation.
Which of the following is the most appropriate next step in
management?
(A) Cardiac magnetic resonance imaging
(B) Surgical mitral valve repair
(C) Transcatheter mitral valve repair
(D) Transesophagealechocardiography
Item 64
A 69 year old man is evaluated fbr a 1 week history of'
redness at the site <,rf his pacenraker. Medical history is sig
nificrnt fbr diabetes mellitus and hypeftension. Medications
are metfbrmin. atorvastatin, and Iisinopril.
On physical examinatiorr, the patient is afebrile:
other vital signs are normal. The pacemaker site is shown
(top of next page). The renrainder of the examination is
unremarkable.
Laboratory studies, including lcukoc-y,te count. are within
normal limits. Three sets of blood cultures are ohtained.
Which of the following is the most appropriate additional
management?
(A) Aspiration ofthedevice pocket
(U) Pacemakerextraction
(C) 1'opical mupirocin
(D) Clinical observation and rcassessment
Item 65
A 78-year old woman is evaluated 4 months after placement
of a drug-eluting stent for treatment of chronic stable angina
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153. Self-Assessment Test
An echocardiogram shows a lelt ventricular ejection
fraction of 5O%, increased left and right ventricular wall
thickness, and abnormal left ventricular diastolic function.
The estimated right ventricular systolic pressure is 64 mm Hg.
ITEM 64
pectoris. She is asymptomatic. She has hypertension, gas-
troesophageal reflux disease, and a history ofseveral colonic
angiodysplasias treated with electrocoagulation 8 months
ago. Medications are pravastatin, aspirin, clopidogrel, metop-
rolol, hydrochlorothiazide, ferrous sulfate, and omeprazole.
On physical examination, blood pressure isl32l72mm
Hg, pulse rate is 78/min, and respiration rate is 2Olmin. BMI
is 17. Scattered ecchymoses are evident over both lower
extremities.
Results of laboratory studies show a hematocrit of 34%.
Which of the following is the most appropriate initial
management?
(A) Assess platelet reactivity
(B) Discontinueaspirin
(C) Discontinue clopidogrel
(D) Discontinueomeprazole
Item 66
A 65-year-old man is evaluated in the hospital for progres
sive dyspnea with light exertion. He was diagnosed with
restrictive cardiomyopathy 2 years ago and atrial flbrillation
18 months ago. Attempts at pharmacologic rhythm control
have been unsuccessful. Medications are apixaban, spirono-
lactone, torsemide, and potassium chloride.
On physical examination, blood pressure is 106/68 mm
Hg, pulse rate is 86/min and irregularly irregular, and oxy-
gen saturation is 94% with the patient breathing ambient
air. Central venous pressure is elevated. Bibasilar crackles
are present. Hepatomegaly is noted. There is bilateral pitting
edema to the knees.
ECG shows atrial fibrillation with a ventricular rate of
86/min, right bundle branch block, and QRS duration of
136 ms.
Which ofthe following is the most appropriate management?
(A) Cardiac resynchronizationtherapy
(ts) Cardiac transplanlation
(C) Implantablecardioverter deflbrillator
(D) Pulmonary vein isolation
Item 67
A 46-year old man is evaluatecl fbr exertional chest pain
that started 2 months ago. His pain is substernal and can
also be provoked by stressful <lr emotional situations. The
pain subsides in 2 to 3 minutes with rest. He has no other
medical problems and takes no medications.
Physical examination findings, including vital signs,
are normal.
ECG shows sinus rhythm with first degree atrioven
tricular block.
Which ofthe following is the most appropriate management?
(A) Coronary artery calcium scoring
(B) Exercise ECG
(C) Exerciseechocardiography
(D) 48 Hour ambulatory ECG
Item 68
A 28-year-old woman is seen for pregnancy planning. She
underwent mitral valve replacement with a mechanical
prosthesis 4 years ago for congenital mitral valve stenosis.
She is asymptomatic. Medications are warfarin, 4 mg/d, and
low-dose aspirin. Her INR measurements have been within
the therapeutic range for the past 18 months, including her
most recent INR measurement of 3.0.
Other than a mechanical-sounding Sr, vital signs and
all physical examination flndings are normal.
Echocardiography shows a normally functioning
mitral valve prosthesis and normal left ventricular function
and estimated pulmonary artery pressure.
The patient would like to attempt pregnancy as soon as
possible. During this time, aspirin will be continued.
Which of the following is the most appropriate
anticoagulation stratery during the first trimester?
(A) Stop warfarin and start bivalirudin
(B) Stop warfarin and start dabigatran
(C) Stop warfarin and start subcutaneous dose-adjusted
unfractionated heparin
(D) Continue warfarin
Item 69
A 72-year-old man is evaluated in the office following a
non-ST-elevation myocardial infarction. He underwent
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percutaneous coronary intervention and is currently
asymptomatic. Medications are low-dose aspirin, ticagrelor,
lisinopril, metoprolol, and atorvastatin.
On physical examination, blood pressure is 140/72 mm
Hg; other vital signs are normal. Femoral bruits are pres
ent bilaterally. Femoral and pedal pulses are diminished
bilaterally.
Ankle-brachial index testing:
Right systolic brachial pressure 140 mm Hg
Left systolic brachial pressure 95 mm Hg
Right dorsalis pedis pressure 112 mm Hg
Left dorsalis pedis pressure 120 mm Hg
Right posterior tibialis pressure 100 mm Hg
Left posterior tibialis pressure 116 mm Hg
Which of the following is the most appropriate test to
perform next?
(A) Exercise ankle brachial index testing
(B) Lower extremity CT angiography
(C) Toe-brachial index testing
(D) No additional testing
Item 70
A 27- year-old woman is hospitalized for a l-day history of
orthopnea and paroxysmal nocturnal dyspnea. She deliv-
ered a healthy baby boy 6 days ago. She is breastfeeding.
On physical examination, blood pressure is 134/78 mm
Hg, pulse rate is 98/min, respiration rate is 26lmin. and
oxygen saturation is 94'7, with the patient breathing ambi
ent air. There is jugular venous distention and an Sr. Crack-
les are heard about halfway up the lungs. There is lower
extremity edema to the knees.
Laboratory studies show an elevated B-type natriuretic
peptide level, a normal high-sensitivity troponin level
(<99th percentile upper reference limit), and a serum cre-
atinine level of 1.2 mg/dl (106.1 pmol/L).
Chest radiograph shows pulmonary edema. Echocar
diogram shows an ejection fraction of20% and di{Iuse hypo
kinesis.
lntravenous furosemide and bilevel positive airway
pressure are initiated.
Which of the following is the most appropriate additional
treatment?
(A) Bisoprolol
(B) Diltiazem
(C) Enalapril
(D) Ivabradine
Item 71
A 25-year old man is evaluated fbr recurrent syncope. The
syncopal episodes are abrupt and without prodrome and
have occurred several times during the past year. He reports
no chest pain or exertional symptoms' He has no other
pertinent personal history. His father died in his sleep at age
45 years. He takes no medications.
On physical examination, vital signs are normal. There
is no heart murmur. The remainder of the examination is
unremarkable.
Laboratory studies, including a comprehensive meta
bolic panel, are within normal limits.
ECG is shown. Echocardiogram is normal.
Which of the following is the most likely diagnosis?
(A) Brugada syndrome
(B) Coronary artery disease
(C) Long QT syndrome
(D) Vasovagal syncope
Item 72
A 30-year-old man is hospitalized for a 3 day history of pro
gressive fatigue, fever, and shortness ofbreath. He under
went surgical aortic valve replacement 3 years ago. He also
has end-stage kidney disease. fbr which he receives hemo
dialysis. Medications are lisinopril, sevelamer, and warf'arin.
On physical examination, blood pressure is 145/34 mm
I'tg and pulse rate is l20i min. Cardiac examination reveals
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bounding pulses and a loud decrescendo diastolic murmur at
the left sternal border. Crackles are heard at the lung bzrses.
An tiCG shows prolonged first degree atrioventricu
lar btock. A chest radiograph reveals pulmonary edema.
A transthoracic echocardiogrant reveals a left ventricular
ejection fiaction of 60'1, with nornral left ventricular dinren
sions and a 1.S-cm vegetation on the aclrtic valve biopros
thesis associated with severe aortic regurgitation.
Multiple blood cultures are obtained, irnd empiric
intravenous antibiotic therapy is initiated.
Which ofthe following is the most appropriate management?
(A) Cardiac cetheterizxtion
(B) Early surgical aortic valve replacement
(C) Placement of a temporary pacemaker
(D) No change in therapy
Item 73
A 44 year-old woman is evaluated for a 6 month history of
progressive exertional dyspnea and fatigue coincident with
the onset of hear,y menstrual periods. Before the onset of
symptoms, her hemoglobin level was 17.9 gldL (179 glL).
Medical history is significant for a large patent ductus arte-
riosus and resultant Eisenmenger syndrome. Her only med-
ication is bosentan for pulmonary arterial hypertension.
On physical examination, blood pressure is 100/65 mm
Hg; the remaining vital signs are normal. Oxygen saturation
with the patient breathing ambient air is 92'2, in the upper
extremities and B2'7, in the lower extremities. There is jugu
lar venous distention with a prontinent o wave, and a prom
inent left parasternal impulse is noted. S, is loud. Clubbing
of the toes and central cyanosis are present.
Self-Assessment Test
Today, the hemoglobin Ievel is 74.8 gldL (148 g/L), and
iron studies are compatible with iron deficiency.
Which of the following is the most appropriate initial
management?
(A) Hysterectomy
(B) Oral iron therapy
(C) Phlebotomy
(D) Supplemental oxygen therapy
Item 74
A 58 year old man is evaluated in the emergency depart-
ment 30 minutes after onset of severe and persistent chest
pain at rest associated with diaphoresis and nausea. His
medical diagnoses include hypertension and hyperlipid
emia treated with amlodipine and atorvastatin. An ECG is
shown.
On physical examination, blclod pressure is 159/84 mm
Hg, pulse rate is 50/min, respiration rate is 181min, and oxy
gen saturation is 94'l, with the patient breathing ambient
air. BMI is 25. All other physical examination findings are
unremarkable.
The nearest percutaneous coronary intervention (PCI)
center is located at least 140 minutes awav.
Which of the following is the most appropriate
management?
(A) CT angiography
(B) Thrombolysis and admission to a telemetry bed
(C) Thrombolysis and transfer to a PCI center
(D) Transfer to a PCI center for primary PCI
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156. Self-Assessment Test
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Item 75
An 18-year old man is evaluated before participating on his
college basketball team. He has no history of hypertension
or other pertinent medical history. He has no history of
palpitations, chest pain, or unusual dyspnea, and there is no
family history of sudden cardiac death or cardiomyopathy.
On physical examination, blood pressure is 110/70 mm
Hg and pulse rate is 52/min. BMI is 22. No murmur is heard.
ECG shows sinus bradycardia, with voltage criteria for
left ventricular (LV) hypertrophy. The corrected QT interval
is 400 ms. Early repolarization is noted.
An echocardiogram shows a mildly dilated LV cavity.
The ejection fraction is greater than 55% without regional
abnormality. Symmetric LV hypertrophy is noted, with LV
wall thickness of 12 mm. LV diastolic fllling, left atrial size,
and valvular structure and function are normal.
Which of the following is the most likely diagnosis?
(A) Athlete heart
(B) Fabrydisease
(C) Hypertensive heart disease
(D) Nonobstructive hypertrophic cardiomyopathy
Item 76
A 42-year old woman is evaluated for a 3-month history of
exertional dyspnea and a burning sensation in her throat.
She had Hodgkin lymphoma at age 22 years treated with
chemotherapy and mantle irradiation. She has no history
of hypertension, hyperlipidemia, or diabetes mellitus and
has never smoked.
On physical examination, vital signs are normal. No
jugular venous distention is seen. Heart sounds are normal.
No murmur or early diastolic sound is heard. There is no
peripheral edema.
A chest radiograph and 12 lead ECG are normal.
Which of the following is the most likely diagnosis?
(A) Aortic stenosis
(B) Constrictivepericarditis
(C) Coronary artery disease
(D) Mitral stenosis
(E) Restrictivecardiomyopathy
Item 77
A 63-year old man is evaluated during a follow up visit for
a 6 month history of heart failure. He currently has New
York Heart Association functional class III symptoms. His
only hospitalization was at the time of diagnosis. Medica-
tions are valsartan sacubitril, carvedilol, furosemide, and
spironolactone.
On physical examination, blood pressure is 110/76 mm
Hg and pulse rate is 64lmin. The remainder of the examina
tion is unremarkable.
Laboratory studies are within normal limits.
ECG demonstrates left bundle branch block with a
QRS duration of 130 ms. Since beginning guideline-directed
medical therapy, his ejection fraction has increased from
15"/,'to25%.
Which of the following is the most appropriate treatment?
(A) Cardiacresynchronizationtherapy defibrillator
(B) Implantable cardioverter-deflbrillator
(C) Implantable pulmonary artery pressure sensor
(D) Wearablecardioverter deflbrillator
Item 78
A 72 year old man is evaluated in the emergency depafi
ment fbr sudden-onset anterior chest pain radiating to his
back. I te has no history of heart murmur or aortic disease.
I{e has hypertension treated with chlorthalidone ancl val
sartan.
On physical examination, bloocl pressure is 182/54 mm
IIg in the right arm and 12.5i 63 mm Hg in the left arm. lhere
is a grade 2/6 decrescendo diastolic murmur heard at the
left sternal border. Central venous pressure is elevated. and
there are crackles at the lung bases.
Chest radiograph shows pulmonary edema and a rvid
ened mediastinum. A CT angiogram short's an ascending
aortic dissection extending into the aortic arch. An echo-
cardiogram sl.rows a left ventricular ejection fraction of 55'X,
and moderate aortic regurgitation.
Which of the following is the most appropriate
management?
(A) Cardiac rnitgnetic res()nunce intlging
(B) Emergent surgery
(C) h-rtra aortic balloon counterpulsation
(D) Transesophageal echocarcliography
Item 79
A 68-year-old man is evaluated during a routine follow up
visit. Medical history is signiflcant for type 2 diabetes melli-
tus, hyperlipidemia, hlpertension, and aortofemoral bypass
surgery 2 years ago. Previous evaluation documented a left
ventricular ejection fraction of50% and stage G3bA2 chronic
kidney disease. Medications are metformin, atenolol, lisin
opril, amlodipine, aspirin, rivaroxaban, and atorvastatin.
On physical examination, vital signs are normal. BMI is
30. An aortofemoral bypass surgical scar is present. Pulses
are present and moderately strong in the lower extremities.
The most recent hemoglobin A,. level is7.l%.
Which of the following is the most appropriate additional
treatment?
(A) Clopidogrel
(B) Liraglutide
(C) Niacin
(D) Pramlintide
Item 8O
A 48-year old woman is evaluated in the hospital for trirn-
sient left facial droop and right sided weakness, which
lasted 15 minutes and completely resolved. She also has a
1-month history of recurrent f'evers and night sweats. She
tx
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144

157. Self-Assessment Test
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reports lack ot appetite and unintentional weight loss of
lll
z s kg (5 lb). She has no orher symptoms or medical prob,
c0Nl 1.-r and has not undergone a rec.ent medical proceclure.
She takes no medicatior.rs.
Orr physical examinaticln. vital signs are nonnal. Car,
diac examination reveals a normal S, and Sr, with a so{t early
diastolic sound heard best at the apex.
ECG dcmonstrates normal sinus rhythm.
CT of'the head with and without contrast is normal.
Echocarcliogram (shown) denlonstrates normal chamber
size and ventricular function (LV
= left ventricler RA = right
irtrium; RV = right ventricle).
Which of the following is the most appropriate
management?
(A) Administer a direct oral anticr,ragulant
(B) Administerintravenousthrombolytic therapy
(C) Obtain blood cultures and begin empiric antibiotics
(D) Urgcnt surgicrl excision
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Item 81
A 53 year old woman is evaluated in the coronary care
unit. She underwent successtirl primary percutaneous
coronary intcrvention with drug-eluting stent placement
in the mid right coronary artery fbr an inferior S'l' elevation
myocardial infarction. ln the catheterization laboratory.
she had several episodes of symptornatic 2:1 atrioventric
ular block with sinus bradycardia. After returning to the
coronary care unit, she has symptomatic intermittent 2:l
atrioventricular block and several episodes of c<lmplete
heart block with a narrou, complex escape rhythrn (heart
rate at 58i min). Meclications are atorvastalin. aspirin. and
clopidogrel.
On physical examination. blood pressure is 1lBiB2
mm FIg. pulse rate is 681min. respiration rate is lB/min.
and oxygen saturation is 96'7, with the patient breath
ing ambient air. Cardiac examination reveals a regularly
irregular rhythm. The remainder ol the examination is
normal.
Which of the following is the most appropriate treatment?
(A) Atropine
(ts) Intravenous unfractionated hcparin
((l) Temporary pacing
(D) Urgentdual chamberpacemakcr
Item 82
A 78 year-old woman is evaluated for 6 weeks of exertional
dyspnea. She has hypertension and paroxysmal atrial flbril
lation. Medications are apixaban, enalapril, and chlortha-
lidone.
On physical examination, blood pressure is 148/90 mm
Hg; other vital signs are normal. BMI is 38. Central venous
pressure is normal, and lungs are clear. An Sr, but no mur
mur, is noted.
B-type natriuretic peptide level is 211 pg/ml (2ttnglL).
An ECG demonstrates sinus rhythm and left ventric
ular hypertrophy. An echocardiogram shows an ejection
fraction of55% and increased left ventricular wall thickness.
The calculated cardiac index is 2.9 L/min/m2. There is no rest
or dynamic outflow tract obstruction. The estimated right
ventricular systolic pressure is 40 mm Hg. The left atrium
is enlarged.
Which of the following is the most likely diagnosis?
(A) Heart failure with preserved ejection fraction
(B) High output heart failure
(C) Hypertrophic obstructive cardiomyopathy
(D) Noncardiac dyspnea
Item 83
A 76 year-old woman is evaluated during a wellness visit.
She reports that she fatigues easily, and her husband notes
that she snores loudly. Medical history is signiflcant for
hypertension. Her only medication is metoprolol.
On physical examination, pulse rate is 75lmin and
irregular; other vital signs are normal. Cardiac examination
reveals irregular heart sounds with no murmurs or gallops.
No signs of heart failure are present.
An ECG shows atrial flbrillation at 75lmin without
ischemic changes.
Which of the following is the most appropriate
management?
(A) Add aspirin
(B) Emergent cardioversion
(C) Increase metoprolol dosage
(D) Initiate oral anticoagulation
(E) Reassurance
Item 84
A 55 year old man is evaluated for a 6 week history of
cough and worsening exertional dyspnea and orthopnea.
He has heart failure, for which he has received guideline-
directed medical therapy for 4 months.
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On physical examination, blood pressure is 130/67 mm
Hg and pulse rate is 90/min and regular. There is an early
systolic click and a holosystolic murmur loudest at the apex
and radiating to the back. The central venous pressure is
elevated, and there are crackles at both lung bases.
An ECG is normal. A chest radiograph reveals pulmo
nary edema. A transthoracic echocardiogram shows a left
ventricular ejection fraction greater than 55%. The echocar
diographic data are consistent with moderate mitral regur-
gitation.
Which of the following is the most appropriate next step in
management?
(A) Cardiac magnetic resonance imaging
(B) Repeat echocardiography in 1 year
(C) Surgical mitral valve repair
(D) Transcatheter mitral valve repair
Item 85
A 67-year-old man is evaluated for a 3 month history of
progressive dyspnea and peripheral edema. He also has a
6-month history of exertional chest "heaviness." Medical
history is otherwise significant for hypertension and type 2
diabetes mellitus. He is a former cigarette smoker. quitting
6 months ago. Medications are hydrochlorothiazide, ator-
vastatin, metlormin, and liraglutide.
On physical examination, blood pressure is 122l86 mm
Hg and pulse rate is 96lmin; other vital signs are normal.
BMI is 27. Jugular venous distention and an S, are present.
Lower extremity edema to the mid thigh is noted.
ECG shows left bundle branch block. Echocardiogram
shows ejection fraction of 25"/,, with anterior hypokinesis
and normal wall thickness.
Which of the following is the most appropriate test?
(A) Cardiac catheterization
(B) Cardiac magnetic resonance imaging
(C) Cardiac PET
(D) Technetium 99m pyrophosphate scintigraphy
Item 86
A 56 year old man is evaluated for a 3 month history of
progressive left calf discomfort that is exacerbated when
walking stairs and hills and is absent at rest. Medical history
is signiflcant for hypertension, hyperlipidemia, and coro
nary artery disease. He also has a 50 pack-year smoking
history but quit smoking 3 years ago. Medications are aspi
rin, rosuvastatin, metoprolol, and amlodipine.
On physical examination, vital signs are normal. BMI is
28. Left femoral, popliteal, and pedal pulses are faint.
The ankle brachial index is 0.68 on the left and 0.98 on
the right.
Which of the following is the most appropriate
management?
(A) CT angiography
(B) Ethylenediaminetetraaceticacid
(C) Pentoxifylline
(D) Revascularization
(E) Supervised exercise training
Item 87
A 28 year old woman r.tith Marfan syndrome is seen fol
lowing recent transthoracic echocardiography obtained as
part of a prepregnancy evaluation. Her mother has l\4arfan
syndrome and had emergency surgery for ascending aortic
dissection B years ago. The patient's only medication is
metoprolol succinate.
On physical examination, blood pressure is 110i 60 mm
Hg and pulse rate is 60imin and regular. The patient has
phenotypical features of Marfan syndrome. The remainder
of the examination is normal.
Transthoracic echocardiogram reveals a dilated proxi
mal ascending aorta with a dimension of 4.3 cm; the
dimension was 3.7 cm 1 year ago. No aortic or mitral valve
regurgitation is present. Left ventricular size and func-
tion are normal. A CT scan confirms the aortic dimension
obtained by echocardiography.
Which of the following is the most appropriate management?
(A) Add atorvastatin
(B) Add losartan
(C) Aortic repair before pregnancy
(D) Proceed with pregnancy
Item 88
A 66 year old woman is evaluated for a 6 month history
of right shoulder pressure that occurs after walking half
a mile and improves with 5 minutes of rest. She has no
dyspnea, nausea, or tatigue. The frequency and duration of
her symptoms have not changed. Her history is otherwise
unremarkable.
On physical examination, vital signs are normal. BMI is
33. Other than a paradoxically split Sr, the cardiac examina
tion is normal. The lungs are clear to auscultation.
A chest radiograph is normal. ECG is shown (top o1'
next page).
Which of the following is the most appropriate test?
(A) Coronaryangiography
(B) Exercise ECG
(C) Pharmacologic myocardial perfusion imaging
(D) Transthoracic echocardiography
(E) No further testing is required
Item 89
A .12
1.'ear old nran is evaluatcd in the eurergcncl, depart
ment tbr pxlpitatiorls. neck pulsations. and light headedness
that bcgarr,15 minlltes irgo. IIe reitorts no chest pain or
breathlessness. He hls been unrler pressure at rtork irnd
l-ras becn anxious and sleepless. llrere is no other relevant
personal or familv history. He cloes not use illicit drugs or
supplements.
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159. Self-Assessment Test
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ITEM 88
m
On physical examination, blood pressure is 90/70 mm
E IIg and pulse rate is 160/min; other vital signs are normal.
c0NL 6rr*., saturation is 9B'X, with the patient breathing ambi
ent air. Intermittent cannon o waves are noted on neck
examination. Other than a rapid regular rhythm, cardiac
examinatior.r is unremarkable. Lungs are c1ear.
ECG reveals ventricular tachycardia. He is successfully
cardioverted.
Complete blood count and metabolic panel, including
electrolytes, are normal.
Echocardiogram and subsequent ECG are both normal.
Which of the following is the most appropriate initial
management?
(A) Cardiac magnetic resonance imaging with stress per
fusion
(B) Electrophysiologystudy
(C) lmplantable cardioverter-defibrillator
(D) Implantable loop recorder
Item 90
A 76-year-old man is evaluated for a 4 week history of
shortness of breath and chest discomfort with minimal
exertion. Medical history is signiflcant for hlpertension,
hyperlipidemia, and coronary artery disease. Medications
are low dose aspirin, amlodipine, atorvastatin, lisinopril,
and metoprolol.
On physical examination, blood pressure is 135/83
mm Hg; other vital signs are normal. Cardiac examination
reveals a late-peaking crescendo-decrescendo systolic mur
mur heard at the right upper sternal border with loss of Sr.
Central venous pressure is elevated. There are crackles at
the lung bases.
Laboratory studies demonstrate a B-type natriuretic
peptide Ievel of 2000 pg/ml (2000 ng/L).
An ECG shows normal sinus rhythm without ST-
T-wave changes. An echocardiogram shows a moder
ately thickened, partially mobile aortic valve, with left
ventricular ejection fraction of 45'/,' and hemodynamic
measurements compatible with Iow flow, low-gradient
severe aortic stenosis.
Which of the following is the most appropriate
management?
(A) Cardiaccatheterization
(B) Dobutaminestressechocardiography
(C) Surgical aortic valve replacement
(D) Transcatheter aortic valve implantation
Item 91
A 62-year-old woman is hospitalized for a non-ST-elevation
myocardial infarction. She has hypertension, type 2 diabe
tes mellitus, newly symptomatic aortic stenosis, and coro-
nary artery disease, for which she underwent percutaneous
coronary intervention 1 year ago. Medications are low -dose
aspirin, ticagrelor. metoprolol, ran.ripril, metformin, and
high intensity atorvastttin.
On physical examinatiort, vital signs are nonnal. A
grade 3/6 harsh midsystolic murnlur is noted at the right
upper sternal border.
An echocardiogram reveals normal left ventricular
ejection fraction, severe aortic stenosis, and an enlarged
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thoracic aortar the maximal diameter of the ascending aorta
lll is 5.6 cm. Coronary angiography reveals diffuse in-stent
c0NT
1g51sn6sis of the proximal left circumflex stent (intarct-
related artery), focal proximal left anterior descending
artery stenosis. and chronic total occlusion of the right
coronary artery.
Coronary bypass graft surgery is planned.
Which of the following is the most appropriate additional
intervention?
(A) Ac.rrtic valve replacement
(B) Aortic valve replacement and aortic repair
(C) Transcatheter aortic valve implantation
(D) No additional intervention
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Item 92
A 38-year-old man undergoes a preoperative evaluation
before repair of a torn anterior cruciate ligament. His car
diovascular history includes repaired tetralogy of Fallot. He
has no symptoms.
On physical examination, vital signs are normal.
Jugular venous distention and a prominent o wave are
noted. A right ventricular heave is present. A single S,
is heard, as is a grade 1/6 early systolic murmur local-
ized to the left second intercostal space and a grade 2/6
diastolic murmur best heard in the left second and third
intercostal spaces. The diastolic murmur increases with
inspiration.
Which of the following is the most likely diagnosis?
(A) Aortic coarctation
(B) Aorticregurgitation
(C) Mitral stenosis
(D) Pulmonaryregurgitation
Item 93
A S9-year old man is evaluated during a routine visit. He
has type 2 diabetes mellitus. One year ago, he had an athero-
sclerotic stroke with no residual neurologic deflcits. Medica-
tions are aspirin, metformin, candesartan, and rosuvastatin.
He remains active and has no symptoms.
On physical examination, blood pressure is 132/80 mm
Hg; other vital signs are normal. BMI is 25. The remainder of
the examination is unremarkable.
Laboratory studies show a serum LDL cholesterol level
of 66 mg/dl (t.Zt mmol/L) and an estimated glomerular
flltration rate of 60 ml/min 11.73 m2. A hemoglobin Ar. level
measured 3 months ago was 6.8%.
Which of the following is the most appropriate
management of this patient's atherosclerotic
cardiovascular disease?
(A) Begin empagliflozin
(B) Begin ezetimibe
(C) Obtain exercise ECG
(D) No changes in management
Item 94
A 35-year-old woman is evaluated in the emergency depart-
ment for a 1 week history of fever and chest pain. The pain
is sharp and midsternal, worse lying down, and improved
leaning forward.
On physical examination, temperature is 38.5 'C
(101.3 'F), blood pressure is 120/70 mm Hg with pulsus para-
doxus of 10 mm Hg, and pulse rate is 92lmin. A three-phase
friction rub is heard along the left sternal border and apex'
ECG shows normal sinus rhythm and normal voltage
with diffuse ST-segment elevation of 1 to 2 mm. An echo-
cardiogram shows a pericardial eflusion without evidence
of tamponade.
Which of the follou'ing is the most appropriate
management?
(A) Discharge on ibuprofen and colchicine
(B) Discharge on prednisone
(C) tlospitalize and begin ibuprofen and colchicine
(D) Hospitalize and begin methylprednisolone
Item 95
A S7-year-old man is evaluated in the emergency depart-
ment af'ter a cardiac arrest. Bystander cardiopulmonary
resuscitation and use of an automated external deflbrillator
resulted in a return of sinus rhythm. An initial ECG revealed
ST-segment depression in leads Vr and Vu. After irritiation of
aspirin, unfractionated heparin, and ticagrelor, angiogra
phy revealed no significant obstructive lesions. He is admit-
ted to a monitored bed, where he develops acute persistent
chest pain, hypotension, and the ECG changes shown.
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Blood pressure is 95/60 mm Hg, pulse rate is 55/min,
respiration rate is 24lmin, and oxygen saturation is 96'2,
with the patient breathing ambient air. Cardiac examina-
tion is normal. tsibasilar pulmonary crackles are present.
The initial high-sensitivity troponin level is elevated
(>99th percentile upper reference limit); urine toxicology
screen is negative for drugs.
148

161. Self-Assessment Test
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Follow-ing intravenous nitrclglycerin, blood pressure
increases and chest pain subsides. Follow-up ECG is shown.
Which of the following is the most likely diagnosis?
(A) Coronary embolism
(B) Coronaryvasospasm
(C) Pulmonary embolism
(D) Stress (takotsubo) cardiomyopathy
Item 96
A 68-year-old man is evaluated for increasingly frequent
angina. One month ago, coronary angiography was per-
formed because of the occurrence of angina at lower levels of
exertion. It showed diffuse coronary disease without lesions
amenable to revascularization and preserved left ventricu-
lar function. Following coronary angiography, the patient
increased his dosage of isosorbide mononitrate to twice
daily; however, his exertional chest discomfort worsened.
He also has hypertension and diabetes mellitus. Previously,
diltiazem was substituted for metoprolol because of intoler-
ance. Medications are aspirin, metformin, liraglutide, ator-
vastatin, lisinopril, diltiazem, and isosorbide mononitrate.
On physical examination, blood pressure is 135/80 mm
Hg, pulse rate is 67lmin, and respiration rate is l8/min. The
remainder of the examination is unremarkable.
ECG shows sinus rhythm and nonspeciflc intraventric-
ular conduction delay, unchanged from 1 month ago.
Which of the following is the most appropriate management?
(A) Add amlodipine
(B) Decrease Iisinopril dosage
(C) Reduce isosorbide mononitrate dosage to once daily
(D) Repeatcoronaryangiography
Item 97
A 70-year-old man is evaluated in the emergency depart
ment for 3 days of palpitations and dyspnea. New-onset
atrial fibrillation is diagnosed. He also has chronic lympho-
cytic leukemia. He began ibrutinib 1 month ago.
On physical examination, pulse rate is 128/min and
irregularly irregular. Oxygen saturation is 95'X, u'ith the
patient breathing ambient air. Other than a rapid, irregular
rhythm, the cardiopulmonary examination is normal. Sple
nomegaly is present. There is no lower extremity edema.
Laboratory studies show a nclrmal D-dimer level. The
initial high sensitivity cardiac troponin level is normal
(<99th percentile upper rel'erence limit). Thyroid-stimulating
hormone and free thyroxine levels are normal.
ECG shows atrial flbrillation with rapid ventricular
response. nonspecific ST-T abnorrnality, and incomplete
right bundle branch block. Echocardiogram shows normal
biventricular size and f'unction. normal valvular structure
and function. and normal atrial size.
Which of the following is the most likely contributor to
the patient's atrial fitrrillation?
(A) Acute myocardial infarction
(B) lbrutinib
(C) Pulmonarythromboembolism
(D) Thyrotoxicosis
ttem 98
A 71'year old woman is hospitalized with cardiogenic
shock.
On physical examination, blocld pressure is B7l51 mm
Hg, pulse rate is 112/min, respiration rate is 22lmin, and
oxygen saturation is 92')(, with the patient breathing 40')(,
Fror. An S.,, jugular venous distention, pulmonary crackles,
ar,d cool extremities are present.
Laboratory studies show an elevated seruln high-
sensitivity cardiac troponin level (>9gth percentile upper
ref'erence limit) and serum creatinine level of t.8 mg/dl
(ls9
Uraoli L).
An ECC demonstrates 2-mm ST-segment depressions
in leads V., through V,,.
Emergent cardiac catheterization shows critical lesions
in the left ilnterior descending and circumflex arteries, and
stents are placed. Following stenting, the patient's clinical
status is unchanged.
Medications are aspirin. prasugrel, f'urosemide, and
norepinephrine.
Which of the following is the most appropriate additional
management?
(A) Digoxin
(B) Intra'aortic balloon pump placement
(C) N4etoprolol
(D) Urgent cardiac transplantation
(Fl) Vasopressin
Item 99
A 66-year-old man is seen in the office after hospitaliza
tion for an embolic stroke 7 days ago. His initial neurologic
flndings were minimal and have since resolved. An embolic
CONI.
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162. Self-Assessment Test
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source has not been identifled. He has no other pertinent
personal or family history. Medications are aspirin and
clopidogrel.
Physical examination, including vital signs and neuro
logic examination, is normal.
Ambulatory 4S-hour ECG monitoring showed no
arrhythmias.
Which of the following is the most reasonable management?
(A) Discontinue aspirin and clopidogrel; begin warfarin
(B) Left atrial appendage occlusion
(C) Loop recorder implantation
(D) Test for thrombophilia
Item 10O
A S5-year-old man is evaluated for easy bruising. The patient
underwent mechanical mitral valve replacement l year ago.
He reports no bleeding. His only medication is warfarin.
On physical examination, vital signs are normal. Car-
diac examination reveals a normal mechanical valve sound
without murmur. The remainder of the cardiopulmonary
examination is normal. Examination of the arms reveals
numerous ecchymoses.
Laboratory studies reveal a normal hemoglobin level;
INR is 3.4 and has ranged between 2.6 and 3.5 over the past
3 months.
Which of the following is the most appropriate treatment?
(A) Continue current warfarin dosage
(B) Decrease warfarin dosage
(C) Discontinue warfarin and start aspirin
(D) Discontinue warfarin and start dabigatran
(E) Discontinue warfarin and start rivaroxaban
Item 101
A SO-year-old man is evaluated for 8 weeks of progressive
exertional dyspnea and a syncopal event. He has been well
otherwise and takes no medications.
On physical examination, vital signs are normal. Car
diac examination reveals a late-peaking systolic crescendo
decrescendo murmur heard at the right upper sternal
border with loss of Sr. The remainder of the examination is
normal.
An ECG shows normal sinus rhythm and left ventricu
Iar hypertrophy with repolarization abnormalities.
An echocardiogram shows a severely thickened, min-
imally mobile bicuspid aortic valve. Hemodynamic mea
surements on echocardiogram are consistent with severe
aortic stenosis and normal left ventricular function. The
ascending aorta could not be adequately assessed.
Which ofthe following is the most appropriate management?
(A) Cardiac catheterization
(B) CT angiography of the thoracic aorta
(C) Exercise treadmill stress testing
(D) Transcatheter aortic valve implantation
Item 102
An 80 year old man is evaluated for a 6-week history of
resting right foot and flrst toe pain. He has type 2 diabe
tes mellitus, hypertension, and hyperlipidemia. He has a
SO-pack-year smoking history but quit 10 years ago. Med-
ications are low-dose aspirin, metformin, lisinopril, and
atorvastatin.
On physical examination, vital signs are normal. The
right foot is pale and mottled. A 4 x 4 cm ulceration is
noted on the lateral aspect ofthe right flfth metatarsal. Pedal
pulses are diminished on the left and absent on the right.
The ankle-brachial index is 0.62 on the left and 0.44 on
the right.
Which ofthe following is the most appropriate next step in
management?
(A) CT angiography
(B) Hyperbaric oxygen treatment
(C) Invasiveangiography
(D) Primarybelow-the-knee amputation
Item 103
A 42 year old woman is evaluated for an episode of syncope
that occurred 2 weeks ago while she was hurrying to catch
a bus. She has hypertrophic cardiomyopathy. Before this
episode, her symptoms had been very well controlled. She
continues to accomplish activities of daily living with only
occasional mild dyspnea. She has no family history of sud-
den cardiac death. Her only medication is metoprolol.
Twenty-four-hour ambulatory ECG monitoring shows
one three beat run of nonsustained ventricular tachycar
dia. Echocardiogram shows maximum left ventricular wall
thickness of 30 mm, asymmetric septal hypertrophy, and
systolic anterior motion of the mitral valve. Resting lelt
ventricular outflow tract gradient is 24 mm Hg, increasing
to 36 mm Hg during Valsalva maneuver.
Which of the following is the most appropriate treatment?
(A) Disopyramide
(B) Implantable cardioverter-deflbrillator
(C) Septal reduction therapy
(D) Verapamil
Item 104
477 year old ra,,oman is hospitalized for intennittent recur
rent chest pain. Her ECG is shon'n (top of next page).
The patient had a moderately severe ischemic stroke
2 years ago and has oxygen dependent COPD. Nledications
are aspirin, atorvastatin, and inhaled albuterol and tiotro
pium.
On physical examination. blood pressure is 145 85 mm
Hg, pulse rate is 82'min, and respiration rate is 18 n1in.
Oxygen saturation is 909i on 2 L ofoxl,gen by nasal cannula.
BMI is 18. An erpiratory wheeze is heard on auscultation
of the lungs. Cardiac examination reveals an Sr. Estimated
central venous pressure is 10 cm HrO. Minimal left-sided
weakness is present.
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initiai high-sensitivity troponin level is elevated
(>99th percentile upper reference limit).
lntravenous unfractionated heparin is initiated, and
coronary angiography is planned fbr the morning.
Which of the following is the most appropriate additional
treatment?
(A) Clopidogrel
(B) Prasugrel
(C) Reteplase
(D) f irofiban
Item 1O5
A 73 year oldwoman is evaluated in the emergencydepart
ment fbr a 1 day history of recurrent ischemic chest pain.
'lhe
last episode began 30 minutes ago and is ongoing despite
escalating doses of sublingual ancl parenteral nitroglycerin.
History is signiflcant for hypertension and dyslipidemia.
Medications are enalapril and atorvastatin.
On physical examination, blood pressure is 119184 mm
Hg, and pulse rate is 104/min. Other than an S.,, the cardio
pulmonary examination is normal.
The flrst set of cardiac biomarker levels is normal.
ECG shows sinus tachycarclia and new 2 mm ST
segnlent depressions in the inf'erior leads.
Which of the following is the most appropriate imagtng
test in this patient?
(A) Coronary artery calcium scoring
(B) CoronaryCTangiography
(C) lnvasive coronary angittgraphy
(D) Transthoracic echocardiography
Self-Assessment Test
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Item 105
A 74-year-old man is evaluated for shortness of breath that
occurs when walking around the house and occasionally
when getting dressed. History is significant for ischemic car
diomyopathy and three heart failure hospitalDations over
the past 6 months. He was treated for colon cancer 2 years
agoi there is no evidence of recurrence. He has an implant
able cardioverter defibrillator. Medications are lisinopril,
high dose furosemide, and spironolactone. He previously
took carvedilol and isosorbide dinitrate hydralazine, which
were discontinued because of symptomatic hypotension.
On physical examination, blood pressure is 94166 mm
Hg and pulse rate is BB/min. Cardiac examination reveals
an S. but no jugular venous distention or dependent edema.
Laboratory studies show a serum creatinine Ievel of
1.7 mgldL (150 pmol/L) and a serum sodium level of
132 mEqrL (132 mmol L).
Echocardiogram performed during his last hospitaliza
tion showed an ejection fraction of l0'7,.
The patient is engaged in a discussion regarding man
agement options.
Which of the following is the most reasonable next step in
management?
(A) Heart transplant
(B) Hospitalization
(C) Left ventricular assist device placement
(D) Low dose P blocker
Item 107
A 69-year old woman is evaluated during a follow up visit
7 months after coronary stent placement for non ST-elevation
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164. Self-Assessment Test
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myocardial infarction. History is also notable for paroxys-
mal atrial flbrillation, hypertension, and gastrointestinal
bleeding due to diverticulosis 5 years ago. Medications are
aspirin, clopidogrel, rivaroxaban, metoprolol, lisinopril,
and rosuvastatin.
On physical examination, vital signs are normal. The
patient weighs B0 kg (u6.4 lb). Bruising is present on the
arms and legs. The remainder of the examination is unre-
markable.
Serum creatinine level is 1.0 mg/dl (88.a pmol/L).
Which ofthe following is the most appropriate
management?
(A) Discontinueaspirin
(B) Discontinuerivaroxaban
(C) Switch rivaroxaban to reduced-dose apixaban
(D) Switch rivaroxaban to warfarin
Item 108
A 35 year-old man is evaluated during a routine offlce visit.
He underwent atrial septal defect closure with a transcath
eter device l year ago. His past medical history is otherwise
unremarkable, and he has no other medical problems. He
takes no medications.
On physical examination, vital signs and the remainder
of the examination are normal.
The patient will have periodontal cleaning next week.
Which ofthe following is the most appropriate endocarditis
prophylaxis?
(A) Amoxicillin
(B) Azithromycin
(C) Ceftriaxone
(D) Clindamycin
(E) No endocarditis prophylaxis
Item 109
A 63 year-old man is evaluated during a follow-up exam-
ination for rheumatic aortic valve disease. He is asymptom
atic and has no exercise limitations. He has no other medical
problems.
On physical examination, blood pressure is 134/32 mm
Hg. A grade 3/6 holodiastolic murmur is heard best at the
left sternal border. Peripheral pulses are bounding. There is
no evidence ofheart failure.
A transthoracic echocardiogram with good image qual-
ity shows a left ventricular ejection fraction greater than
55'1, and a tricuspid aortic valve with severe aortic regur-
gitation. The left ventricular end systolic dimension is ele
vated at 45 mm.
Which of the following is the most appropriate
management?
(A) Repeat evaluation in 6 months
(B) Surgical aortic valve replacement
(C) Transcatheter aortic valve implantation
(D) Transesophageal echocardiography
Item 1 10
A 56 year old man is hospitalized for an ST elevation m1'o
cardial infarction. He is treated with percutaneous coronary
intervention and is now asymptomatic. Medical history is
significant for hypertension and paroxysmal atrial fibrilla
tion. Outpatient n-redications are flecainide. rivaroxaban.
metoprolol. and lisinopril.
A predischarge ECG shows sinus rh1'thm (heart rate.
58 min). a QRS complex duration o1 124 ms, and a right
bundle branch block pattern. An echocardiogram reveals
a mildly reduced left ventricular ejection fraction $'ith an
inferior u'al1 motion abnormalitv.
Which of the following is the most appropriate
management?
(A) Ambulatory ECG monitoring
(B) Discontinueflecainide
(C) Discontinuemetoprolol
(D) Exercise stress testing
Item 111
A 78 year old woman is evaluated during follow up of
nonischemic heart failure with reduced ejection fraction
diagnosed 6 months ago. She has New York Heart Associa
tion functional class III symptoms. She is receiving optimal
guideline-directed medical therapy consisting of valsartan-
sacubitril, carvedilol, spironolactone, and furosemide.
On physical examination, blood pressure is lo4l62
mm Hg and pulse rate is 58/min. A grade 2/6 holosystolic
murmur is heard at the apex, and a grade 1/6 crescendo
decrescendo systolic murrnur is heard at the base. There is
no jugular venous distention or peripheral edema.
ECG shows sinus rhythm and left bundle branch block
with QRS duration of 155 ms. Echocardiogram shows an
ejection fraction of 307,, left ventricular end systolic dimen-
sion of 53 mm, mild to moderate mitral regurgitation, and
mild aortic stenosis.
Which of the following is the most appropriate treatment?
(A) Cardiacresynchronizationtherapy
(B) Ivabradine
(C) Mitral valve clip placement
(D) Transcatheter aortic valve implantation
Item 112
A S8-year-old man with stable exertional angina returns
for follow-up evaluation. He has normal left ventricular
function and no high-risk features on exercise ECG. Angina
symptoms include chest pressure that routinely occurs
while walking 25 yards despite maximally tolerated medi-
cal therapy. Medications are aspirin, metoprolol, isosorbide
mononitrate, and atorvastatin.
On physical examination, blood pressure is 110/70 mm
Hg, pulse rate is 54/min, and respiration rate is 16/min. BMI
is 25.
A coronary angiogram shows a 70% proximal right cor-
onary artery stenosis and an 80'/n second obtuse marginal
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165. Self-Assessment Test
artery stenosis. The left anterior descending artery has non-
obstructive lesions in the proximal segment.
Which of the following beneflts can the patient expect
following coronary artery bypass grafting?
(A) Discontinuation of cardioprotective medications
(B) Improvement in symptoms
(C) Increased survival
(D) Reduced risk for myocardial infarction
Item 1 13
A 30 year-old man is seen in the offlce for slowly pro-
gressive dyspnea that flrst appeared when walking rap-
idly or uphill. He denies orthopnea, palpitations, or chest
pain. He has no other medical problems and takes no
medications.
On physical examination, vital signs are normal.
Jugular venous distention is present. There is a left para-
sternal impulse. A grade 2/6 systolic murmur is heard
at the second left intercostal space, and a diastolic flow
rumble is heard over the tricuspid valve. Fixed splitting
of the S, is present. The remainder of the examination is
normal.
An ECG is shown. Chest radiograph reveals right heart
enlargement, a prominent pulmonary artery, and increased
pulmonary vascularity.
Which of the following is the most likely diagnosis?
(A) Atrial septal defect
(B) Bicuspid aortic valve with aortic regurgitation
(C) Mitral stenosis
(D) Ventricular septal defect
Item 1 14
A 65-year-old woman is evaluated in the oIflce after several
visits to the emergency department for paroxysmal atrial
flbrillation and acute heart failure. Medications are apix-
aban, metoprolol, furosemide, and losartan.
Vital signs are normal. Cardiopulmonary examination
and the remainder of the physical examination are normal.
Results of laboratory studies show a normal serum
thyroid-stimulating hormone level.
An ECG shows sinus rhythm with a heart rate of
58/min. An echocardiogram reveals a left ventricular ejec-
tion fraction of 45%.
Which of the following is the most appropriate
treatment?
(A) Atrioventricular node ablation with permanent pace-
maker implantation
(B) Implantable cardioverter-deflbrillator placement
(C) Left atrial appendage occlusion
(D) Rhythm control
Item 1 15
A 57 year old man is evaluated in the emergency department
tbr sudden onset anterior chest pain. He has hypertension,
hyperlipidemia, and coronary artery disease. Medications
are hydrochlorothiazide. aspirin, amlodipine, olmesartan,
labetalol, and atorvastatin.
On physical examination, blood pressure is 172i 64 mm
IJg in the right arm and 135/63 mm I{g in the left arm, pulse
rate is 110/min, respiration rate is 24lmin, and oxygen sat-
uration with the patient breathing ambient air is 96'X,. Car
diac examination reveals a grade 3/6 decrescendo diastolic
murmur heard at the left sternal border.
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153

166. Self-Assessment Test
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A serum high-sensitivity cardiac troponin measure-
ment is indeterminate.
An ECG shows sinus tachycardia and lateral S'f
segment clepressions. Chest radiograph is normal'
Which of the following is the most appropriate diagnostic
test to perform in this Patient?
(A) Coronary artery catheterization
(B) Invasive aortography
(C) Magnetic resonance angiography
(D) Transthoracic echocardiography and CT angiography
Item 1 16
A76 year old woman is evaluated in the emergency depart
ment lor an episode ofnear syncope. She has also had progres
sive dyspnea and nonproductive cough over a :l week period
and an unintentional weight loss o14.1 kg (9 lb) over the past
3 months. She reports no f'ever. She has a S0-pack-year history
of cigarette smoking, but she stopped smoking 3 weeks ago.
On physical examination, the patient is af'ebrile. Blood
pressure is 132/60 mm Hg, with a fall in systolic pressure ol
24 mm tlg during inspiration. Pulse rate is 110/min and regu
lar. Oxygen saturation is 90'X, with the patient breathing ambi
ent air. BMI is 17. Jugular venous distention is present. I leart
souncls are distant. Crackles are present at the lung bases.
A chest radiograph shows a 6 cm right upper lobe mass
and an enlarged cardiac silhouette.
Which ofthe following is the most appropriate diagnostic test?
(A) Cardiaccatheterization
(B) CT directed needle biopsy
(C) Fiberopticbronchoscopy
(D) PET/CT
(E) Transthoracic echocardiography
Item 117
A 57 year-old woman is seen during a routine follow-up
visit for heart failure. She has a S-year history of ischemic
cardiomyopathy with an ejection fraction of 38'/". She also
has a 15 year history of type 2 diabetes mellitus and diabetic
kidney disease. She has had no hospitalizations. Medica-
tions are aspirin, atorvastatin, valsartan sacubitril, metop
rolol succinate. and metfbrmin.
Physical examination, including vital signs, is unre-
markable.
Laboratory studies show an elevated B,type natriuretic
peptide level, a hemoglobin A,. level of 7.0"/,,, a serum cre,
atinine level of 1.5 mgi dL (132.6 pmol/L), and an estimated
glomerular flltration rate of 50 mL/min/1.73 m2.
Which of the following is the most appropriate additional
treatment?
(A) Dapagliflozin
(B) Glimepiride
(C) Liraglutide
(D) Saxagliptin
Item 118
A 78-year oldwoman is evaluated in follow up for a 3-month
history of intermittent claudication, which is worse in the left
leg than in the right. Her symptoms have become life limiting
de"spite adherenie to a structured exercise program' Medical
history is sigfficant for type 2 diabetes mellitus and hyper
tension. She has a 60-pack-year smoking history and quit
l year ago. Medications are aspirin, metformin, canagliflozin,
cilostazol. atorvastatin, and olmesartan.
On physical examination, vital signs are normal' Dor
salis pedis and posterior tibialis pulses are faint bilaterally'
The ankle brachial index is 0.82 on the right and 0'64
on the left.
CT angiogram reveals dilluse calcified atherosclerotic
plaques in both iliac arteries, with a 30% stenosis on the
right and an 80u1, stenosis in the left iliac artery.
CONT,
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Which of the following is the most appropriate treatment?
(A) Pentoxif,zlline
(B) Revascularization
(C) Stop canagliflozin
(D) Switch olmesartan to ramipril
Item 119
A 50 year-old woman is evaluated tbr a 3 month history
of progressive chest pain. The pain is worse with ph1'sical
activity, radiates to the lett arm, and is associatecl with dia-
phoresis.'lhe pain is relieved by rest.
On physical examination. blood pressure is 167,i98 mm
Hg. and pulse rate is 58rmin. Cardiac examination reveals
an S., and no murmurs.
ECG shows sinus rhythm and left ventricular hyper-
trophy,*,ith a strain pattern.
Which ofthe following is the most appropriate diagnostie test?
(A) Dobutamine echocardiography
(B) Dobutamine myocardial perfusion imaging
(C) Exercise ECG
(D) Exercise myocardial perfusion imaging
Item 120
A 49-year-old woman is evaluated for a 3 month history
of substernal chest heaviness that occurs when walking
up a small hill near her home. The discomfort does not
radiate. Each episode lasts for approximately 5 minutes and
improves as she continues walking.
On physical examination, vital signs are normal. BMI
is 37. The remainder of the examination is unremarkable.
Resting ECG shows normal sinus rhythm without
ischemic changes.
Which of the following is the most likely diagnosis?
(A) Atypical angina
(B) Nonanginal chest pain
(C) Typical angina
(D) Unstable angina
tr
154

167. Answers and Critiques
Item 1 Answer: D
Educational Objective: Treat stable heart failure with
reduced ejection fraction with valsartan-sacubitril.
The most appropriate treatment is to switch lisinopril to
valsartan-sacubitril (Option D). This patient with hearl fail
ure with reduced ejection fraction (HFTEF) and New York
Heart Association (NYHA) functional class II symptoms, as
evidenced by dyspnea with moderate exerlion, is stable and
has no evidence of volume overload on examination. In the
PARADIGM-HF trial of patients with symptomatic heart
failure and Ieft ventricular ejection fraction less than 40'1,,
valsartan sacubitril reduced mortality and heart failure hospi
talization by 20'2, compared with enalapril. Based on this study.
the American College of Cardiologr/American Heart Associ
ation heart failure guidelines recommend replacing an ACE
inhibitor or angiotensin receptor blocker (ARB) with valsartan
sacubitril in patients with chronic symptomatic HFrEFl, In
addition, for patients with new-onset heart failure, directly ini
tiating valsartan-sacubitril, rather than a pretreatment period
with an ACE inhibitor or ARB, is a safe and effective strateg/
in patients with HFrEFl, Because of the risk for angioedema,
ACE inhibitors (but not ARBs) should be discontinued at least
36 hours before starting valsafian sacubitril.
lsosorbide dinitrate hydraluine (Option A), when used
in combination with an ACE inhibitor, p-blocker, and aldoste
rone antagonist, reduces mortality compared with placebo in
Black patients with N1TIA functional class III to IV slmptoms.
Guidelines recommend adding this drug combination in Black
patients who remain rynnptomatic on maximal doses of an ACE
inhibitor, ARB, or angiotensin receptor'-neprilysin inhibitor;
B
blocker; and aldosterone antagonist. lf this patient were White,
isosorbide dinitrate hydralazine would not be indicated, and if
this patient were Black, it would be initiated if lnHA class III
slmptoms persisted after initiation of valsartan-sacubitril.
ln patients with NYHA functional class II to IV heart
failure symptoms, ivabradine (Option B) has been shown to
reduce heart failure hospitalizations when added to standard
heart failure therapy. Ivabradine is approved for patients
with symptomatic HFrEF (ejection lraction <357,) who are
in sinus rhythm with a heart rate of 7Olmin or higher and
taking a maximally tolerated B blocker. This patient has a
heart rate of 60/min and is therefore not a candidate.
This patient has a heart rate of 60/min and does not
require p-blocker dosage escalation (Option C) at this time.
I(EY POITI
o Valsartan-sacubitril signifi cantly reduces heart failure
hospitalizations and mortality in patients with symp-
tomatic heart failure with reduced ejection fraction
and is recommended in preference to an ACE inhibitor
or angiotensin receptor blocker.
Bibliography
Maddox l M, Januzzi )L lr. Allen t.A. et al; Writing Committee. 2O2l Update
to the 2017 ACC expert consensus decision pathway for optimization of
heart failure treatment: ansu,ers to l0 pivotal issues about heart failure
with reduced ejection fiaction: a report of the American College of
Cardiologr Solution Set Oversight Commifiee. J Am Coll Cardiol. 2O2t;
77 :77'2 81O. LPMID: 334.16.1i01 doi:l0.l0l6ij.jacc.2O2O.ll.O22
Item 2 Answer: B
Educational Objective: Treat a patient with symptom-
atic bradycardia.
Pacemaker implantation (Option B) is the most appropri-
ate next step in management for this patient who presents
with signs and symptoms of sinus node dysfunction. Com-
mon indications for permanent pacemaker implantation
include symptomatic bradycardia without reversible cause;
permanent atrial flbrillation with symptomatic bradycar
dia; alternating bundle branch block; and complete heart
block, high degree atrioventricular (AV) block, or Mobitz
type 2 second degree AV block, irrespective of symptoms.
This patient is bradycardic at baseline, does not mount a
tachycardic response to activity on ambulatory ECG moni
toring, and has low energr. There is no threshold that deflnes
an inadequate heart rate response; it is determined by
symptoms suggesting that the heart rate is not meeting the
patient's physiologic demands. A common challenge among
these patients, many of whom are older, is differentiating
between age-related decline in physical activity and patho
logic, symptomatic sinus node dysfunction. The former is
less likely to improve with cardiac pacing, and the latter
often improves dramatically. In this case, the patient is very
active, with distinct loss of energr and ECG flndings of sinus
bradycardia. Recent stress test results conflrm normal left
ventricular function and no ischemia; thus, cardiac pacing
is the next appropriate step.
Patients with sinus node dysfunction are rarely unsta-
ble, but if there is evidence of hemodynamic instability,
hospitalization (Option A) is indicated. Warning signs and
symptoms of hemodynamic instability warranting hospital-
ization include hypotension, altered mental status, ischemic
chest pain, and acute heart failure. This patient's condition
is not acute or dangerous, and thus inpatient care is not
warranted.
Lisinopril is unlikely to be contributing to this patient's
bradycardia, although his hypertension is likely a risk factor
fbr sinus node dysfunction. Although amlodipine is a dihy
dropyridine calcium channel blocker, use of this drug may
actually worsen bradycardia; therefbre, switching lisinopril
to amlodipine (Option C) is not indicated.
Reassurance with ongoing monitoring (Option D) is
not appropriate, because it is likely that this patient's loss of
energz is pathologic and related to symptomatic bradycardia.
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168. (a
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Answers and Critiques
XEY POIXI
. Pacemaker implantation is indicated in patients with
symptomatic bradycardia in the absence of a reversi
ble cause.
Bibliography
Kusumoto FM. Schoenfeld MH, Barrett C, et al. 2018 ACC/AHA/HRS guide
line on the evaluation and management ofpatients with bradycardia and
cardiac conduction delayr a report ofthe American College ofCardiologl/
American Heart Association Task Force on Clinical Practice Guidelines
and the Heart Rhythm Sociery Circulation. 2019;140:e382-e482. [PMID:
3058677 2l doi:10.1161/CIR.0000000000000628
Item 3 Answer: A
Ed ucation a I Obj ective : Treat intermittent claudication
with cilostazol.
The most appropriate additional treatment is cilostazol
(Option A). Cilostazol, a phosphodiesterase inhibitor with
antiplatelet and vasodilator activity, increases pain-free
walking distance and overall walking distance in patients
with claudication. This patient with established peripheral
artery disease (PAD) is receiving guideline-directed medical
therapy and has completed a supervised exercise program,
but he has persistent symptoms. The most appropriate treat-
ment is to add cilostazol to his medication regimen. As with
other oral phosphodiesterase inhibitors (e.9., milrinone), the
FDA has placed a black box warning on use of cilostazol in
patients with heart failure. Side effects of cilostazol include
headache, diarrhea, dizziness, and palpitations. Up to 20%
of patients discontinue cilostazol within 3 months due to
side effects.
Guidelines from the American College of Cardiologz/
American Heart Association (ACC/AHA) recommend anti-
platelet monotherapy for patients with PAD to reduce the
risk for myocardial infarction, stroke, or vascular death. This
patient does not have an indication for dual antiplatelet
therapy (e.g., recent acute coronary syndrome or percuta-
neous coronary intervention), and there is no evidence to
support the use of dual antiplatelet therapy with aspirin
and clopidogrel (Option B) over antiplatelet monotherapy in
patients with PAD.
PAD is considered a coronary heart disease risk equiva-
lent, and the ACC/AHA guideline recommends that patients
with PAD be treated with a high intensity statin to prevent
cardiovascular events. Although there is emerging evidence
from subgroup analyses that patients with lower extrem-
ity PAD benefit from the proprotein convertase subtilisin/
kexin type 9 (PCSK9) inhibitors evolocumab (Option C)
and alirocumab, there is no evidence that the addition of a
PCSK9 inhibitor in a patient with an LDL cholesterol level
less than 70 mg/dl (1.8 mmol/L) is associated with improved
outcomes.
Pentoxifylline (Option D) has not been associated
with an improvement in symptoms in patients with
intermittent claudication and PAD, and the ACC/AHA
guideline does not recommend pentoxifylline for treat
ment of claudication.
Elevated homocysteine levels are associated with an
increased risk for cerebrovascular and cardiovascular dis-
ease. Patients with PAD have higher plasma homocysteine
levels than those without PAD. However, there is no evi
dence that B-complex vitamin supplementation (Option E)
to lower homocysteine levels improves clinical outcomes,
including cardiovascular death, myocardial infarction, or
stroke. Vitamin B complex vitamins are not recommended.
IEY POITIT
. In patients with peripheral artery disease and inter-
mittent claudication, cilostazol is recommended to
improve limb symptoms in addition to smoking ces-
sation, aspirin, statin therapy, and supervised exercise.
. Cilostazol is contraindicated in patients with heart
failure.
Bibliography
Gerhard-Herman MD, Gornik HL, Barrett C, et al. 2016 AHA/ACC guideline
on the management of patients with lo €r extremity peripheral artery
disease: a report of the American College of Cardiolos//American Heart
Association Task Force on Clinical Practice Guidelines. I Am Coll Cardiol.
2077 ;69 :e71-e126. IPMID: 27851992] doi:10.1016/j.jacc.2016.11.007
Item 4 Answer: C
Educational Objective: Evaluate acute ehest pain using
coronary CT angiography.
Coronary CT angiography (Option C). by allor,ving visualiza
tion of coronary ancl other thoracic patholoS,. is the' most
appropriate cliagnostic test to perfbrm next in this patieltt
lvith acnte chest pain. Coronary,' CT angiographl' plays an
irnportant role in the evaluation of acute chest pain in the
emergency departlnent. This patient presents \:ith signs and
symptonls that could ir.rdicate an acute coronary syndronte.
aortic syndrorle. pulnronary embolism, clr other acute chest
pain etiolo6Xr. I;or patients with chest pain, coronirry C1'
angiography also mav be useful in patients w'ith a possible
diagnosis of non ST elevation acllte coronary syndrorne
u'ho hare equirocal initial tropor.rin levels or a single tropt-r
nin eler:ation r.tithor-rt lurther symptoms of acute coronary
syndrome, or in patients lr,ho have iscl.remic symptorns that
rcsolved hours belore undergoing testing.
Cardiac stress testing is a reasonable approach fbr this
patient: hortever. adenosine mlrlcardial perfusion irnaging
(Option A) is not an appropriate option fbr this patient
bccause she has reactive ainval,s disease. Broncl.rospastic
reactive airw'ays disease is a contraindication to adenosine
based vasodilatory agents for stress testing.
Cardiac nlagnetic resonance in.r:rging (Option B) may,be
uscd rvith dotrutamine to assess ltall motion abnornralities
or with vasodilators to assess pertusion. [t is commonly lter-
tbrmed to e\"luate the degree of infarctiolr. \,'iability can be
determined by evaluating the extent of myocardial fibrosis
within the left ventricle. Cardiac magnetic resonarrce imag
ing is not a helpfirl test in assessing acLtte chest pain ir.r the
emergency department because of the relatively longer time
fbr image acquisition compared'"r,ith other modalities.
tr
156

169. Answers and Critiques
Itr
'lhis
patient's presentation does not indicate a specific
carcliac structural or functionirl c;tuse that rvould make
transthoracic echocardiography (Option D) usefirl at this
point.
'l'ransthor:rcic
echocardiography is the mainstay <tf
noni nvasive clrdior,ascular imagi ng for structural abnonnal
ities. The patient's presclttation potentiirlly indicates other
causes of the chest pain that are l)etter evaluated u,ith coro
nary C'f angiography'.
IEY POIilT
. Coronary CT angiography, by allowing visualization
ofcoronary and other thoracic pathologr, plays an
important role in the evaluation of acute chest pain
in the emergency department.
Bibliography
Rybicki FJ, Udelson JE, Peacock WF, et al. 20ts ACR/ACC/AHA/AATS/ACEp/
ASNC/NASCI/SAEM/SCCT/SCMR/SCPC/SNMMI/STR/STS appropriate
utilization of cardiovascular imaging in emergency department patients
with chest pain: a joint document of the American College of Radiolog,
Appropriateness Criteria Committee and the American College of
Cardiologz Appropriate Use Criteria Task Force. J Am Coll Cardiol. 20161
67 :853 79. IPMID: 26809772] doi:10.1016/j.jacc.2015.09.011
Item 5 Answer: D
Educational Objective: Diagnose ventricular septal
defect.
The most likely diagnosis in this patient is a small (restrictive)
ventricular septal defect (VSD) (Option D). The presentation
of an isolated VSD depends on the VSD size and pulmonary
vascular resistance. Small VSDs are usually asymptomatic. A
palpable systolic murmur (thrill) is often noted at the left ster
nal border, accompanied by a loud holosystolic murmur that
obliterates the Sr, as observed in this patient. Small VSDs do
not cause left heart enlargement or pulmonary hlpertension,
and the ECG and chest radiograph reveal norrnal flndings.
VSD closure is not indicated for patients with a small left-to
right shunt and no chamber enlargement or valve disease, but
periodic clinical evaluation and imaging are recommended.
Patients with small VSDs do not require activity restrictions.
An adult with an atrial septal defect (ASD) (Option
A) most often presents with dyspnea, atrial arrhythmias,
or right heart enlargement. Physical examination findings
include elevation in venous pressure, a right ventricular
Iift, flxed splitting of the Sr, a pulmonary midsystolic flow
murmur, and, when there is a large shunt, a tricuspid dia
stolic flow rumble. The ECG demonstrates right axis devi
ation and incomplete right bundle branch block, and the
chest radiograph generally demonstrates features of right
heart enlargement and enlarged pulmonary arteries with
increased pulmonary blood flow.
Typical physical examination flndings in aortic coarcta
tion (Option B) include upper extremity hypertension, radial
artery-to-femoral artery pulse and blood pressure differ
entials, and a systolic murmur over the left chest related to
obstruction from the coarctation. In addition, findings of
bicuspid aortic valve, including ejection click and systolic
ejection murmur, are commonly present, as more than 50'/.
of patients with coarctation also have a bicuspid aortic valve.
The ECG demonstrates left ventricular hypertrophy, and a
typical chest radiograph shows abnormal aortic contour and
rib notching.
A small patent ductus arteriosus (PDA) (Option C) gen-
erally causes no cardiovascular symptoms. A continuous
murmur heard beneath the left clavicle that envelops the
S, is typical. Occasionally, no murmur is heard, and the
diagnosis is made by echocardiography. The ECG and chest
radiograph are normal in a patient with a small PDA.
XEY POITIS
e A small ventricular septal defect presents with a loud
(often palpable) holosystolic murmur located at the
left sternal border that obliterates the Sr.
o Ventricular septal defect closure is not indicated for
patients with a small left-to-right shunt and no cham
ber enlargement or valve disease, but periodic clinical
evaluation and imaging are recommended.
Bibliography
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the
management of adults with congenital heart disease: a report of the
American College of Cardiolos,/American Heart Association Task Force
on Clinical Practice Guidelines. J Am Coll Cardiol. 2Ol9:73:e9l-e192.
IPMID' 301212391 doi:10.1016/j.jacc.2018.08.1029
Item 6 Answer: h
Ed ucationa I Objective: Treat complex multivessel
coronary artery disease.
The most appropriate management is coronary artery bypass
grafting (CABG) (Option A). This patient has complex mul
tivessel coronary artery disease (CAD) involving the left
main coronary artery distal bifurcation, proximal left cir
cumflex artery and proximal left anterior descending and
flrst diagonal artery bifurcation. CABG, especially with use
of arterial (internal mammary artery) conduits, is indicated
in patients with multivessel CAD and no contraindications,
because it is associated with enhanced survival compared
with medical therapy alone. CABG also results in decreased
recurrence of angina, lower rates of myocardial infarction,
and fewer repeat revascularization procedures compared
with percutaneous coronary intervention or medical ther
apy alone in these patients.
Guideline-directed medical therapy (Option B) is essen
tial for improved prognosis before and after surgical or per
cutaneous revascularization. Aspirin, B blockade, and statin
therapy, along with ACE inhibition in patients with systolic
left ventricular (LV) dysfunction, such as this patient, are
indicated for secondary prevention of major adverse vas-
cular events. However, anti ischemic and cardioprotective
medical therapy would be inferior to revascularization in
improving survival in this patient with complex CAD involv
ing the left main coronary artery and a large area of poten-
tially ischemic myocardium.
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Answers and Critiques
Myocardial viability testing (Option C) uses imaging
to detect potentially reversible states of ischemic ventric
ular dysfunction. Myocardial viability is associated with
improved survival and ventricular recovery following revas
cularization in patients with LV dysfunction; horvever, per
fbrming viability testing befbre revascularization has not
been shown to predict or enhance outcomes and is not
indicated in this patient.
Drug eluting stent based percutaneous coronary
intervention (Option D) is a reasonable option in patients
with symptomatic CAD. including those with unprotected
left main CAD (without bypass to the left coronary circu
lation). However, in patients with complex CAD including
multiple bifurcations and/or long lesions who are not at
increased surgical risk, CABG is the preferred revascular
ization strateg/.
I(EY POIl{I
. In patients with multivessel coronary artery disease,
coronary artery bypass grafting is associated with
enhanced survival compared with medical therapy
alone.
Bibliography
RltCI MR. CAIhOOTT JII. DChNTCT GJ, Ct AI. ACCIA,\TS/AIIA/ASEIASNC/SCAI
SCCT'STS 2017 appropriate Llse critcria for coronan rerascullriz:tion in
patients s,ith stablc ischemic hearl disease: a report of the Americar-r College
of Cardiokrgr Appr)priate Use Criteril'lask Force, Americf,n Association fi)r
'lhoracic
Surgery American Heart Associittion, Anlerican Societ-n.' ot
I:chocardiographl: Americirn Societ) of Nuclear Cardiokrs: Societ)' ti)r
Cardiovlscular Angiography and Interventions. Society of Cardiovascullr
Computed Tomogrrrphy: and Societ-v ol Thoracic Surget)ns. J Am Coll Cardi(r.
')017
$9)212 22.11. IPMtD' 2829166i]l doi:10.1016 j.jacc.20l7.02.0Ol
Item 7 Answer: A
Educational Objective
disease.
Diagnose infi ltrative cardiac
Cardiac magnetic resonance (CMR) imaging with gadolin
ium contrast (Option A) is the most appropriate test to
perform next. The patient's history and physical exam
ination are consistent with heart failure, and the elevated
B type natriuretic peptide level supports this. Signilicantly
increased wall thickness is present on the echocardiogram.
with discordantly low voltage on the ECG. These findings
suggest an inflltrative cardiomyopathy and, in combination
with the patient's history of carpal tunnel syndrome, suggest
possible transthyretin amyloid (ATTR) amyloidosis. ATTR
amyloidosis is much more common in men. and the patho
logic V1221 mutation in the TTR gene is present in 3'l, to 3.5',1,
of Black persons. If cardiac amyloidosis is present in this
patient, CIvIR imaging with gadolinium contrast would likely
show diffuse late mid myocardial gadolinium enhancement
in a noncoronary distribution. CMR imaging with gadolin
ium is highly sensitive and speciflc fbr cardiac amyloidosis,
but it does not distinguish between AL (immunoglobulin
light chain) amyloidosis and ATTR amyloidosis. An abnor
mal 99m technetium pyrophosphate scan would conflrm
ATTR amyloidosis without the need fbr a biopsy.
Exercise echocardiography (Option B) is useful fbr diag
nosing coronary artery disease and evaluating valvular heart
disease. tt is not indicated for this patient with no anginal
symptoms and near normal left ventricular (LV) systolic
function.
Right and left heart catheterization (Option C) may
demonstrate hemodynamic features consistent with restric
tive physiologr but would not diagnose the underlying cause
of this patient's symptoms without concomitant endomyo
cardial biopsy. Endomyocardial biopsy may be uselul if the
diagnosis of an infiltrative cardiomyopathy is unclear, but
given the risks of this inrasive test, it would not be indicated
as the initial test ol choice.
Fabry disease is a lysosomal storage disorder that aflects
the heart, with marked diltuse LV hypertrophy. The ECG
usually demonstrates marked LV hypertrophy with prom
inent ST T abnormalities, rather than the low voltage seen
in this patient. Neuropathy in Fabry disease often presents
as burning dysesthesia. not carpal tunnel syndrome. Onset
of Fabry disease occurs in childhood, and the diagnosis is
established through genetic testing or an abnormal serum
o. galactosidase level (Option D).
f,tY POTilTS
o Cardiac magnetic resonance imaging with gadolin
ium is highly sensitive and specific for cardiac amy
loidosis, but it does not distinguish between AL
(immunoglobulin light-chain) amyloidosis and
transthyretin amyloidosis.
r In patients with cardiac magnetic resonance imaging
findings consistent with cardiac amyloidosis, an
abnormal 99m-technetium pyrophosphate scan
would confirm transthyretin amyloidosis without the
need fbr a biopsy.
Bibliography
Ruberg l;l-. Grogrn M. Hanna \{. et rl.'lransth}retin iim}'loid cardiomy)p,
thy: J;\CC state of the trt revieu: J Am Coll Cardiol. 2019:73:2872 9l
IPMID: :]l l7l094l doi:10. l0l6/j.jacc.2019.01.003
Item 8 Answer: D
Educational Objective: Treat a patient with symptom-
atic severe aortic stenosis with transcatheter aortic valve
implantation.
The most appropriate next step in treatment is transcath
eter aortic valve implantation (TAVI) (Option D). Aortic
valve replacement is a life prolonging procedure in patients
with severe aortic stenosis. The indications for aortic valve
replacement in severe aortic stenosis are (1) the presence of
symptoms, (2) left ventricular systolic dysfunction (ejection
fiaction <50'X,) in an asymptomatic patient. or (3) a con
comitant cardiac surgical procedure for other indications.
Flxertional dyspnea. syncope, and angina are the most com
mon presenting symptoms that result in aortic valve inter-
vention. Aortic valve replacement can be performed with
surgical aortic valve replacement (SAVR) or via transcatheter
158

171. tr
approach (TAVI). TAVI is reconrmended in preference to
SAVR for symptomatic patients with severe aortic stenosis
who are older than 80 years or fbr younger patients with a
life expectancy less than l0 years. 'IAVI
is also recommended
lbr symptomatic patients ol any age with severe aortic ste-
nosis and a high or prohibitive surgical risk if predicted
postprocedure survival is more thar.r 12 months with an
acceptable quality of iife. For symptomatic patients aged
65 to B0 years, either SAVR or TAVI is appropriate following
shared decision making.
Balloon aortic valvuloplasty (Option A) was previously
used to treat symptomatic severe aortic stenosis by tempo
rarily increasing the aortic valve area in patients who were
not surgical candidates. With the advent of the less invasive
TAVI, balloon valvuloplasty is now rarely used.
Although the patient is at prohibitive surgical risk and
would not likely be approved for SAVR, she would likely be a
candidate for TAVI. Thus, simply continuing medical therapy
(Option B) would be inappropriate, given the mortality and
quality of-life benefit associated with TAVI.
The patient's comorbid conditions*atrial fibrillation,
stage G3a chronic kidney disease, COPD, and hypertension
make her a poor candidate lor SAVR (Option C). Her high
surgical risk is further quantified by her Society ofThoracic
Surgeons adult cardiac surgery risk score.
IEY POtXTS
o Transcatheter aortic valve implantation is recom-
mended in preference to surgical aortic valve replace
ment for symptomatic patients with severe aortic ste-
nosis who are older than 80 years or younger patients
with a life expectancy less than 10 years.
. Transcatheter aortic valve implantation is recom-
mended for symptomatic patients of any age with
severe aortic stenosis and a high or prohibitive surgi
cal risk if predicted postprocedure survival is more
than 12 months with an acceptable quality of life.
Bibliography
Otto CM. Nishimura RA. Bonon' RO. et rl. 2020 ACC AI IA guideline frrr thc
manirgement of patients with \'rlvular heart disease: a report of the
American College of Cardiolog Anlerican Heart Association Joint
(i)mmittee on Clinical Practice Guidelines. Circulation. 2o21:111]:e72
e227. IPMID: 33332150] doi:10.1161 (11R.0000000000000923
Item 9 Answer: C
Educational Objective: Provide secondary prevention of
sudden cardiac death using an implantable cardioverter-
defibrillator.
Jhc rnost appropriate aclclitional nranagenrent before dis
cl.rarge is implantable cardiovefter defibrillator (lCD) place
n.rcr-rt (Option C) in this athletc r,r'ho presented lvith rescuecl
sudden cardiac death. P:rtients $'ith sustained r,entricular
arrhythmitrs (>30 secor-rcls) <tr carcliac arrest ithollt a revers
ible cause have a class I recommendation for secondary
prevention with ICD placement. Despite her nornral left
Answers and Critiques
ventricular frlnction, an ICD is 'uvarranted before dischargc.
barring a clear. acute contrailldication (e.g., active bacterc
mia). [CDs are ir-rdicated and eflective in the sefting of second
lry prevention of sudden cardiac death, even if the causc is
not confirmed (unless it is very clear that the cause is acutely
reversibie and correctable). l{owever, her presentation is not
unusual fbr arrlrythmogenic right ver-rtricular cardion.ryopathy
(AltVC). an inherited "r,tear and tear" disorder that primarily
itfibcts the right ventricle but nta1, be seen in the left. Ventric
ular arrhythmias are often tl.re initial presentation. and somc
rlre very clrarnatic (as in tl-ris case); they are also lery likely to
recur. Cardiac magnetic resoltzlnce imaging lr,ould be helplul
to ,ssess m),,ocardial inliltration, ideally before ICD placement.
A nrajor facet of her treatnlent will be exertional limitation,
of ten a major challenge lor patients who are athletes.
Arniodarone (Option A) is a multichannel antiarrhythmic
agent that ma1, be necessary in patiellts with ARVC and re{rac'
tory ventricular arrhl.thrnias. [-lower,er, this patient has 1at
tcr
htle a trial ol exerlional limitation andior a p blocker (e.g.,
r.netoprolol). and she is quite
)our1g to commit to amiodarone
as Iirst line therapy Furthennorc. amiodarone is not nearly as
e {lective as is :rn ICD ir, preventing sudden cardiac death.
Genetic testing (Option B) n.ray be helpful to under
star.rcl this patient's disease risl< and severity, and results muy
have implications fbr ITer future children and other family
nrcnrbers. Ift.lwever, genetic testing is not required for diag
r.rosis or ICD placement, irnd it should not be undertaken
i,r,,ithout prior genetic counseling.
'lherefore.
it is not nec
essary befbre discharge, ls it is unlikely to affect immediatc
decision rn:rking regarding ICI) placement.
Lisinopril (Option D) may be helpful fbr patients n'ith
ne'ut lcfl rrentricular d1'sfurT ction and has been recommended
in patients r,r,ith ARVC n,ith right ventricular dysfunctior.rr
holtever. it has no current role in this patient. Furthermore,
it is not irs imperative bcfbre discharge as is an lCD.
XEY POIilI
. Patients with zustained ventricular arrhy'thmias
(>30 seconds) or cardiac arrest without a revenible cause
have a class 1 recommendation for secondary prevention
with implantable cardioverter-defibrillator placement.
Bibliography
Al Khatib SM. Stevenson WG. Ackernrrn l\4J. et al. 2017 AHAIACCIHRS
guicleline for management of patients with ventricular affhythmias and
the prevention ofsudden cardiirc deilth: r report ofthe American College
of Cirrdiologr/American Hean Association Trsk Force on Clinical Practice
Cuidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2018r72:
c9l e220. [PN4ID: 290972961 doi:10.1016ij.jacc.2017.10.054
Item 10 Answer: B
Educational Objective: Treat a patient with diabetes
mellitus and heart failure with a sodium-glucose cotrans-
porter 2 inhibitor.
The most appropriate additional treatment is empagliflozin
(Option B). Sodium-glucose cotransporter 2 (SGLI2) inhibi
tors have been studied in patients with diabetes mellitus and
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159

172. Answers and Critiques
in patients with heart failure with or without diabetes. For
patients with diabetes, studies have shown a reduction in
cardiovascular events, including heart lailure-related mor
tality and hospitalizations, with use of these agents. SGLI2
inhibitors (empagliflozin, canagliflozin, and dapagliflozin)
seem to reduce the risk for heart failure hospitalization and
cardiovascular death by 1.9"/,' and death from any cause by
2.3'7,, independent of glucose control. The mechanisms of
this reduction are unclear but are thought to be independent
of increased diuresis due to glucose excretion. The American
Diabetes Association Standards of Medical Care in Diabetes.
endorsed by the American College of Cardiologr, recom-
mends initiating an SGLT2 inhibitor with proven cardiovas-
cular beneflt to reduce the risk for worsening heart failure
and cardiovascular death in patients with type 2 diabetes
and established heart failure with reduced ejection fraction.
Digoxin (Option A) reduces the risk for heart failure
hospitalization but does not reduce mortality. It is occasion
ally used in patients r.r,ith heart failure symptoms refractory
to optimal therapy, but because of its associated toxiciry
digoxin is not recommended as routine therapy in most
patients with heart failure.
Loop diuretics, such as furosemide (Option C), are the
primary diuretic therapy for volume overload in heart failure
because of increased potency compared with other diuretics.
However, this patient has no clinical evidence of volume
overload. and furosemide is not indicated.
Guidelines recommend the addition of isosorbide
dinitrate-hydralazine (Option D) in Black patients who
remain s),,rnptomatic on maximal doses of a p blocker; ACE
inhibitor, angiotensin receptor blocker (ARB), or valsartan-
sacubitril; and aldosterone antagonist. In patients who are
intolerant of ACE inhibitor or ARB therapy, especially those
with chronic kidney disease, isosorbide dinitrate hydralazine
may be considered as a therapeutic option.
I(EY POIl{T
o A sodium-glucose cotransporter 2 inhibitor is recom
mended to reduce risk for worsening heart failure and
cardiovascular death in patients with type 2 diabetes
mellitus and established heart failure with reduced
ejection fraction.
Bibliography
Dunlay SM, Givertz MM, Aguilar D, et al; American Heart Association Heart
Failure and Transplantation Committee of the Council on Clinical
Cardiologz. Type 2 diabetes mellitus and heart failure, a scientific state-
ment from the American Heart Association and Heart Failure Society of
America. I Card Fail. 2019;25:584 619. IPMID: 31174952] doi:10.1016/j.
cardfai1.2019.05.007
Item 11 Answer: C
Educational Objective: Manage atrial fibrillation with
early rhythm control.
Rhythm control (Option C) is the most appropriate treat
ment for this patient who presents with paroxysmal atrial
flbrillation. This patient is reflective of those included in the
EAST AFNET 4 randomized clinical trial, which evaluated a
rhythm control strates/ versus usual care (typically includ
ing rate control) in patients with a recent diagnosis (within
12 months) of atrial flbrillation and coexisting cardiovas-
cular conditions. The inclusion criteria were age older than
75 years or previous transient ischemic attack or stroke, or
two of the following: age older than 65 years, female sex,
heart failure, hypertension, diabetes mellitus, severe coro
nary artery disease, chronic kidney disease, and left ventric
ular hypertrophy. The trial demonstrated improved clinical
outcomes. including a reduction in the primary composite
end point of cardiovascular death, stroke, or hospitaliza
tion for heart failure or acute coronary syndrome, among
patients randomly assigned to an early rhythm control
strates/, including asymptomatic patients. The intervention
included either antiarrhy'thmic drugs or catheter ablation,
but importantly, it included aggressive concomitant medical
therapy (e.g., oral anticoagulation when indicated, hyper-
tension treatment) in both the intervention and the control
groups. Based on the trial results, this patient is mostly likely
to beneflt from early rhythm control for atrial flbrillation.
This patient is appropriately receiving stroke prevention
therapy with a direct oral anticoagulant (DOAC), and he has
had no recurrent stroke or signiflcant bleeding episodes on
the current therapy. Therefore, left atrial appendage occlu-
sion (Option A) is not indicated.
Among the common indications for permanent pace-
maker implantation (Option B) are symptomatic bradycar
dia without reversible cause; permanent atrial flbrillation
with symptomatic bradycardia; alternating bundle branch
block; and complete heart block, high-degree atrioventric
ular (AV) block, or Mobitz type 2 second-degree AV block,
irrespective of symptoms. This patient has no indications for
pacemaker implantation.
Oral anticoagulation in patients with atrial flbrillation
can be accomplished with a vitamin K antagonist (warfarin)
or DOAC, such as rivaroxaban. Rivaroxaban is noninferior to
warfarin in the prevention of stroke or systemic embolism
and is associated with less intracranial and fatal bleeding.
The 2019 American College of Cardiologz/American Heart
Association atrial flbrillation guideline recommends DOACs
in preference to warfarin in DOAC eligible patients. Thus,
there is no suggestion that switching to warlarin (Option D)
would improve outcomes in this patient.
Offering no additional therapy (Option E) would be
inappropriate because early rhythm control is associated
with improved clinical outcomes in patients such as this one.
XEY POIilI
. In patients with recently diagnosed atrial fibrillation
and concomitant cardiovascular conditions, early
rhl.thm control (antiarrhyhmic drugs or ablation)
reduces the primary composite end point of cardio-
vascular death, stroke, or hospitalization for heart
failure or acute coronary syndrome compared with
usual care.
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173. Answers and Critiques
Bibliography
Kirchhof B Camm AJ, Goette A, et al; EAST AFNET ,t Trial Investigators.
Early rhythm-control therapy in patients with atrial fibrillation. N Engl J
Med. 2020;383:1305 1316. [PMID: 328653751 doi:10.los6/NEJMoa20l9422
Item 12 Ansurer: A
Educational Objective: Treat stable angina pectoris in
an aspirin-intolerant patient.
Clopidogrel (Option A) is the most appropriate cardiopro
tective medication for this patient. The patient's chest pain
symptoms, which occur with exertion and subside with rest,
are consistent with typical angina. Therefore, he has a high
pretest likelihood ofcoronary artery disease and is a candi-
date for secondary preventive therapy. All patients with sta-
ble ischemic heart disease should receive guideline directed
therapies consisting of risk factor modiflcation with regular
physical exercise, attention to diet, weight loss, and smoking
cessation, as well as cardioprotective therapy to improve
prognosis and antianginal medication as needed to improve
functional capacity. The Antithrombotic Trialists' Collabora
tion collaborative meta analysis of nearly 3000 patients with
stable ischemic heart disease found an association between
aspirin use and reduced risk for serious vascular events,
including a 46% decrease in the risk for unstable angina and
a 53% risk reduction in the need for coronary angioplasty.
Therefore, in the absence of contraindications, antiplatelet
therapy in the form of low dose aspirin (75 162 mg/d) is
indicated indeflnitely for secondary prevention to reduce
the risk for myocardial infarction and cardiovascular death.
In patients who have an intolerance or allergz to aspirin,
clopidogrel therapy is considered an acceptable alternative.
Aspirin desensitization is another approach.
This patient has experienced an adverse reaction to
aspirin. Aspirin desensitization can be performed safely
under medical supervision in patients who require initiation
of aspirin (for example, those who require dual antiplatelet
therapy after percutaneous coronary intervention). How
ever, in a patient with aspirin allergr or sensitivity, lowering
the aspirin dose (Option B) will not improve tolerance.
Prasugrel (Option C) and ticagrelor (Option D) are newer,
more potent, and more costly antiplatelet agents with potential
for increased bleeding events, and their use as monotherapy
has not been studied in the context of secondary prevention
fbr patients with stable ischemic heart disease; they are not
suggested for this indication in current guidelines.
XEY POIl{TS
. All patients with stable ischemic heart disease should
receive guideline directed therapies consisting of life-
style modification, cardioprotective therapy, and
antianginal medication.
o Patients with stable ischemic heart disease should
receive antiplatelet therapy (low-dose aspirin or clo-
pidogrel in aspirin-intolerant patients) for secondary
prevention of cardiovascular events.
Bibliography
Katz D, Gavin MC. Stable ischemic heart disease. Ann Intern Med. 2019;171:
ITC17 ITC32. IPMtD: 31382288] doi:10.7326lAITC2019O806O
Item 13 Answer: C
Educational Objective: Diagnose peripartum
cardiomyopathy.
'lhe
most likely diagnosis is lteripartunt cardionryoprrtltl,
(Option C). Peripartum clrdiomyoprrthl, is newly notecl
lcft vcntricular s1'stolic dysfunction n'ith onset in the.
trot.tths after delivery or toward thc end of pregnancf in
the absence of another identifiable ciruse. Paticnts usuirlly
present u'ith feltures of heart failure. Trcltnrent u'itl.r
stndard medical therap),'. including B blockers. digoxin.
hyclralazine, nitrates, ancl tliuretics, is appropriate. AOIi
inhibitors. xngiotelrsin receptor blockers. anrl alclostenrnc
rntagor.lists sl-toulcl be i.trtrided until ittter delive rt'becuusc
of teratogenicity.
Pulrnonarr enrbolisnr (PIr) (Option A) nr:r1, occur post
prrtunr. particularly in patierlts requiring prokrnged becl
rest. Patierlts with l)1,) tiequently present with dysprrea. ilow
cver, this patient's elevatect enous prcssure. pnlmonary
c<rngestion. and global recluctior-r in lelt ver-rtricular function
suggest heart tailurc; in aclclition, thc nornral right herrt
size ancl {irnctiorr noted by echocarcliography lrgue :rgainst
henroclvnirmicalll significlnt PIJ.
Thc patient's presentirtioll does trot sup;urrt a clilg
rrosis of irscending irortic clissection (Option B). I'he risk
firr aortic clissection is incrcased in tl.re peri itnd p<tst
partur-lr pcriods. [)atients usttally ]r:tve sudclerr orlset chcst
irncl bacl< plin and often clescribe a tcuring cluality. Ph_vsi
cill exalrinirtion of'ten denlorlstrates right Ief t dift'erentirtl
pulse ancl blood pressure. I'.cl.tocarcliography iu ascetrtlitrg
aortic clissection usually dcnronstrates aortic tiilatiltiott.
rrnd thc clissectior-t tlap rnrrv be visualized. Lctl ventric
tular funclior.r is usualll' trrlnnal in petienls witlr aortic
clissect iou.
Spontirneous coronar)' rrtery clissection (Option D) is
the most comnlon cause ot'pregnarlcy associlted tnYoctlr
dial inlrrrction irncl riccurs ntost contrnol.rly during the filst
nrontl-r postpartnrn. Patients usually ltresent
r,r'ith symptotrrs
of chesl piiin. and nryocarcliitl ischentic changes ltre expectcci
orr the [i(](1. Regionirl wall nrotion abnormirlitics are iclen
tified by echocirrctiography in patients lvitlt sllotttanctltts
coronary ilrtery dissectioll.
I(EY POIl{T
. Peripartum cardiomyopathy is left ventricular systolic
dysfunction with onset toward the end of pregnancy
or in the months after delivery in the absence of
another identifiable cause; patients often present with
features of heart failure.
Bibliography
Douglass EJ, Blauwet LA. Peripartum cardiomyopathy. Cardiol Clin.2o21;
39:ll9 42. IPMID: 332228081 doi:10.1016/j.cc1.2020.09.008
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Answers and Critiques
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Item 14 Answer: C
Educational Objective: Diagnose cardiac angiosarcoma.
The most likel_v diagnosis is cardiac angiosarconta (Option C).
lhis rare rnalignant canliac tuntor often presellts u'ith symp
toms o{ clyspnca and chest pair.r. A cardiac angiosarcomrr
generally arises in the right hcirrt. most commonll'the right
atrium. and tl-ris paticnt's finclings of a right atrial mass
in'r'olving the lateral atrial uall u'ith tamponade and bloody
pcricardial fluid are fypical. Metastasis to the iungs, lir,er,
l1-rnphatic system. bone. and adrenal glands is cotnmotrll'
present at the time of diagnosis. Pcricardiocentesis urith
cytologic examination o{'tl-re fluid is ollen negative ltrr malig
nanc\,. and tissuc biopsy is frequer.rtly required tbr diaguosis.
Surgical resection and adjuvant chemotheralry or rircliother
apy should be considered. but sun'ival rates are lort even filr
patients in whom surgery is t'easib1e.
Atrial myxomas (Option A) occur most commonly'
u'ithin the iell atriurn and u'ith association to the intcr
atrial fossa, although they may present in ttther locations.
including the right atrillm. Thel' t1'picalll' cituse collstitu
tional synlptonrs of fever, rvcight loss, and fatigue; embolic
phenomena; or synlptoms related to obstructiot-t. Pericardiitl
e{fusion with tamponade r'r,ould be au exceptiouirlly rare
prc'sentltion of atrial myxoma.
The CT scan short's only a single, srnail pulmonarl
nodule. All pulmor.rary rtodules har,e potential for associatecl
malignant risk. but giver.r this patient's nonstnoking status
and the nodulc's small size, it is unlikely trt be a prim;rry
malignanc-v car,rsir-rg prolbund cardilc metastasis (Option B).
Papillnry flbroelastorna (Option D) usually occurs on
the surfhce ol the aortic and rnitral val'nes atld is cotntnol1ly
discor,ered in the eighth decade of life. Papillary fibroelas
toma typically does not cause symptoms but may be asso
ciated r,vith strtlke, trirnsient ischen.ric attack. al1d. rarel):
coronary embol iz.ation with inf arction. Ott cchocarcliogram.
these turnors oflen have a heterogencous globular shape or
a mobile liorrd like appearance. This patient's presentation
is not characteristic of fibroelastoma.
XEY POIXI
o Cardiac angiosarcomas are rare malignant cardiac
tumors that typically arise within the right atrium
and are commonly associated with sanguinous peri-
cardial effusion.
Bibliography
Look Hong NJ, Pandalai PK, Hornick lL, et al. Cardiac angiosarcoma manage
ment and outcomes: 20 year single institution experience. Ann Surg
Oncol. 2012:19 :2707 I 5. [PM ID: 224767521 doi : 10. 12.15 I sl} 134 O12 - 233 1 2
Item 15 Answer: D
Ed ucationa I Objective : Treat acute decompensated
heart failure with optimal diuretic dosing.
-lhe
most appn)priate treatment is to increase the dose of intrl
venous (lV) lurosemide (Option D). This patient pr-esentcd
u,ith acute heart failurc in the setting of volume overload,
and he h.rs several signs of an increascd intrirvascular volume.
ir.rcluding eler,ated central vetrous pressure and peripheral
eclema. h.r this setting, the initial IV dose of a diuretic should
be 1 to 2.5 times the patient's total daily oral dosei if the patient
is unresponsir,e to the initial IV dose. it should be increased by
50')1, to 100')i,. Adding a thiazicle diuretic may also be consid
ered, but these agents are usually associated rvith an increased
incidence of l.rl ponatremia.
lnotropes, such as tnilrinone (OptionA), do not improve
outcolnes in pirtients admitted r,r,'ith acute decompensatcd
heart tailure or incrcase urine outpttt. Inotropes may be
considered in patients with low-output heart failure (lolt'
pulse pressure. cool extremities. presence ol S,) to improve
cardiac output but should n<tt be usecl in the routine therapy
of patients adrnitted with heart failure.
IV vasodilator therapy: such as nitroglycerin (Option B)'
is occasionally addecl in patients hospitalized w'ith acute
l.reart Iailure ancl might be considered for this patient. With
reduction of systenric rascular resistance. stroke volume
can oftcr.r be in.rproveci, $'ith resulting patient improve
ment. l{owever, diuretic therapy is the principal treatment
fbr patients rvith dccompensated l.reart failure and {luid
overloaci.
According to the 2019 American College of Cardiology
expert consensus decision pathrt'a1,' otr risk assessment.
lxanagement, and clir-rical trajectory of patients hospital-
ized u'ith heart tailure. patients r,r'ith herrt failure with a
wet antl r,,,,ann profile who are receiving p blocker ther
irpy on admission should cttntintte the p blocker unless
blood pressure is lo',v. If hcart failure rernains refractory
to diuretics, the dose should be halved. Discontinuit.tg
metoprolol (Option C) rright be appropriate if this patient
is resistant to high closes of lV diuretics: regardless. the
best initial step for this patient nould be to increase the IV
furosenride dose.
XIY POIilT
. For hospitalized patients with acute decompensated
heart failure, the initial dose of an intravenous (lV)
diuretic should be 1 to 2.5 times the patient's total
daily oral dose; if the patient is unresponsive, the IV
dose should be increased by 50% to 100%.
Bibliography
Felker GM. Ellison DH, Mullens W, et al. Diuretic therapy for patients with
heart tailure: JACC state of the art revier.n J Am Coll Cardiol. 2020r
75:1178-1195. IPMID: 32164892] doi:10.1016ij.jacc.2019.12.059
Item 16 Answer: C
Educational Objective: Treat complicated type B aortic
dissection.
The most appropriate management fbr this patient is elner
gency endovascular repair of the descending aorta (Option C).
'11'pe
B aortic clissection involves the descendir.rg thoracic
aorta. typically just distal to the left subclavian artery, and
tr
tr
162

175. tr
may extend into the abdominal aorta and lower extremity
arteries; it does not involve the ascending aorta. Patients
with uncomplicatcd type B aortic syndromes nray be treated
u,ith medical therapy initiallyr hortever, aortic repair is
indicated when type B aortic dissection is complicated by
limb or end organ ischemia. persistent severe hypertension,
persistent pain,
irropagation of the dissection, enlargement
of the descending aorta, and/'or rupture of the aorta. This
patient has propagation ol a type B aortic dissection, as
evidenced by new onset abdominal pain (representing mes
enteric ischemia) ar.rd the developmer.rt of limb ischemia.
Patients with type B aortic dissection sl.rould be preferen
tially managed u,ith thoracic endovascular aortic repair.
lt,ith the primary goal ol sealing both proximal and distal
segments of dissection. Surgicai repair of the descending
aorta is associated with high morbidity and mortality.
Enalaprilat (Option A) is an intravenous ACE inhibitor
that is useful mainly in hyperlensi'"e emergencies associated
with high plasma renin activity, such as sclenrdernta renal cri
sis. It is a difficult drug to titrate, with slor,v onset of action and
unpredictable blood pressure response.
'lhis
patient needs
urgent repair of the aorta. not the additior.r oi enalaprilat.
The acute management of aortic dissection focuses
on reducing blood pressure and shear stress. Rapid,acting
p blockers, such as esmolol. are initiatec'l kr reduce heafi rate
to less than 60,rrnin; alter the acute phase of treatment, an
oral p blocker such as rneloprolol mzry replace intravenous
esmolol (Option D). If blood pressure rc'mains elevated,
intravenous nitroprusside (Option B) is usccl to lower the
blood pressure to thc lonrest tolerable limit lvithout impair
ing kidney firnction or cerebral perf'usion, typically between
100 to 120 nrm lJg. However, this patient has clear evidence
ol'complicated type B aortic dissection, ancl urgenl repair of
the aorta is indicated.
TEV POIilIS
o Complicated type B aortic dissection is characterized
by limb or end-organ ischemia, persistent severe
hypertension, persistent pain, propagation ofthe dis-
section, enlargement ofthe descending aorta, and/or
rupture of the aorta.
. Complicated type B aortic dissection is an indication
for immediate vascular intervention.
Bibliography
tsouli M, Patterson BO, Loundou AD, et al. Endovascular versus open repair
for chronic type B dissection treatment: a meta analysis. Ann Thorac
Surg. 2019;107:1.559 1 570. [PMID: 30481.51 6l doi : 10. i016/j.athoracsur.
201 8_lo.o45
Item 17 Answer: D
Educational Objective: Manage asymptomatic prema-
ture ventricular contractions.
Reassurance (Option D) is the most appropriate management
for this patient with asymptomatic premature ventricular
contractions (PVCs). He has no signs or symptoms of heart
Answers and Critiques
failure or exertional limitations. No additional evaluation is
necessary because this patient's PVCs are asymptomatic and
not accompanied by other signs or symptoms of cardiopul-
monary disease. Ambulatory ECG monitoring lor assess
ment of PVC burden may be performed, but in an asymp-
tomatic patient, results are unlikely to affect management.
Asymptomatic PVCs, even at higher burden, are common in
the general population and may vary dramatically through
out the day and/or from day to day They may be influenced
by stress, alcohol or cafleine intake, sleep disturbances, and
comorbid conditions, such as thyroid disorders or anemia.
ln the absence of a significant comorbid condition and/or
symptoms, reassurance is appropriate. Among patients with
a consistently very high burden of PVCs (>15'2, 20'X,) that are
asymptomatic, periodic echocardiographic monitoring for
PVC induced cardiomyopathy may be considered, but there
is no consensus on this surveillance.
Cardiac magnetic resonance imaging (Option A) of the
chest is recommended for cardiac sarcoidosis, which may
present with ventricular arrhythmias. Howevet this patient
has no flndings that suggest sarcoidosis (such as pulmonary
disease or ECG flndings demonstrating conduction disease
[abnormal PR, QRS, QT intervals]). Thus, it would be prema
ture and of low yield to screen for cardiac sarcoidosis in this
asymptomatic patient.
This patient has no exertional symptoms or signs con
sistent with coronary ischemia. Exercise ECG (Option B) is
not needed, and intervention on any positive findings is of
debatable net clinical beneflt.
In the absence of high-risk features (syncope, family
history of premature sudden cardiac death, structural heart
disease), medical therapy is often unnecessary. However,
PVCs require treatment when symptoms are bothersome or
frequent (>10% of all beats or 10,000 PVCs per day). First
line therapy for these patients is a B-blocker, such as metop
rolol (Option C), or calcium channel blocker.
f,EY POIXT
. For patients with asymptomatic premature ventricu-
lar contractions, reassurance is appropriate; medical
therapy is unnecessary in the absence of high-risk
features (syncope, family history of premature sudden
cardiac death, structural heart disease).
Bibliography
Al Khatib SM, Stevenson WG, Ackerman MJ. et al. 2017 AIIA/ACC/HRS
guideline for management of patients with ventricular rrrhythmias and
the prevention ofsudden cardiac death: a report of the American College
ofCardiologr/American I Ieart Association Task Force on Clinical Practice
Cuidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2018;72:e91
e220. f PMID: 290972961 doi: 1 0. 1 01 6 /j.iacc.2017.10.054
Item 18 Answer: C
Educational Objective: Evaluate possible infective
endocarditis with transesophageal echocardiography.
The most appropriate management is tr:rnsesophageal
echocardiography (TEE) (Option C). The patient's history
CONT.
a
(l,
Et-
!,
E
IE
vt
(l,
=
vl
163

176. Answers and Critiques
vl
=
.D
vt
o,
CL
a.|
lt
(D
tl
tr
CONT.
of intermittent f'evers and dyspnea is concerning lbr infec
tive endocarditis (lE). possibly due to dir,erticulitis relatcd
episodes of transient bacterernia. Ol note. an1-onc with
a bioprosthetic valve is at high risk fbr endocarditis with
any significant episode of bacteremia. This patient has a
bioprosthetic valve. symptoms of inf'ection, and a possible
source of bacteremia. According to current guidelines. in
patients'uvith suspected IE. transthoracic echocardiographl'
('l"lE) is recommended to idcntify vegetations. character
ize the hemodynarnic scverity ol valvular lesior.rs, assess
ventricular function and pulnronary pressures, and cletect
complications. In all patients witl-r knor,r,n or suspected IE
and nondiagnostic TTE results, or il complications have
developed or are clinically suspected or if intracardiac
device leads are present, TFIFI is recommended.
Cardiac CT (Option A) is less:rccurate than TTE ancl TEE
fbr iclentifying t alvular vegetation iind valvular perfbrations.
However, in patients in rt'hom the anatomy cannot be clearll'
clelineated by echocardiographl, in the setting of'suspected
parirvalvular ir-rfections. PE'l Cf imaging is reasonable and
superior to cardiac CT alone. This patient has no indicatlon
fbr cardiac CT.
Although cardiac magnctic resonance imaging (Option B)
is useful in the diagnosis of'many conditions, it is not the
appropriate or gold standarcl test lbr diagnosing tlJ.
ln patients with suspectecl IE. TTE is recornrnended
to identify vegetations, charlcterize the hernocll namic
severity of valvular lcsions, assess ventricular function ancl
puln.ronary pressures. and cletect complications. Given the
suspicion fbr prosthetic virlve endocarditis. a potentially
letl.ral condition, not pursuing firrther testing (Option D) is
inappropriate.
KEY POIXTS
. In patients with suspected infective endocarditis,
transthoracic echocardiography is recommended as
the initial imaging choice in most clinical situations.
o In all patients with known or suspected infective
endocarditis and nondiagnostic transthoracic echo-
cardiographic results, or if complications have devel-
oped or are clinically suspected or if intracardiac
device leads are present, transesophageal echocardi-
ography is recommended.
Bibliography
Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the
management of patients with valvular heart disease: a report of the
American College of Cardiologz/American Heart Association Joint
Committee on Clinical Practice Guidelines. Circulation. 2O21;143:e72
e227. [PMID: 333321501 doi:10.1161/CIR.0000000000000923
Item 19 Answer: C
Ed u cati ona I O bjective : Evaluate lo-year atherosclerotic
cardiovascular disease risk.
The most appropriate next step in management is to calculate
this patient's 1O-year risk for atherosclerotic cardiovascular
disease (ASCVD) (Option C). Cardiovascular risk scores can
be used to assess a patient's future risk for major cardiovascu-
lar events and to identi$r preventive interventions. The Amer-
ican College of Cardiologr (ACC)/American Heart Association
(AHA) Pooled Cohort Equations can be used to calculate risk.
The ACC/AHA guidelines recommend that adults aged 40 to
75 years without diabetes mellitus and with an LDL cho-
lesterol level of 70 to 189 mg/dl (1.81 4.89 mmol/L) should
undergo risk assessment with the Pooled Cohort Equations
to classiff 1o-year risk as low (<5%), borderline (5'2, to <7.5'1,),
intermediate (>7.5'7, to <2o'/,), or high (>20'1,). All current
ASCVD primary prevention guidelines recommend calcula
tion ofthe 1o-year ASCVD risk.
According to the ACC/AHA guidelines, high intensity
statin therapy (Option A) is indicated for primary preven-
tion in patients with an LDL cholesterol level of 190 mg/dl
(4.92 mmollL) or higher and those with diabetes and
multiple ASCVD risk factors. This patient meets neither
criterion. High-intensity statin therapy would also be
indicated for patients with an LDL cholesterol level of
7O mgldL to 189 mg/dl (1.81-4.89 mmol/L) and a 10-year
risk of20% or greater. This patient's calculated 10-year risk
is 0.4%. All current ASCVD primary prevention guidelines
agree that this patient does not require high intensity
statin therapy.
For primary prevention of ASCVD, the ACC/AHA
cholesterol guideline recommends ezetimibe (Option B) in
patients with an initial LDL cholesterol level of 190 mg/dl
(+.gz mmol/L) or higher who do not achieve a 50'2, reduction
in LDL cholesterol while taking maximally tolerated statin
therapy or who have an LDL cholesterol level of 100 mg/dl
(z.sq mmol/L) or higher. All guidelines are in consensus
that this patient would not require combination cholesterol
lowering therapy.
Fasting and nonfasting total cholesterol and HDL
cholesterol levels have fairly similar prognostic value and
association with cardiovascular outcomes. Fasting samples
are preferred in adults with an initial nonfasting triglyceride
level of 400 mgldL (4.s2 mmol/L) or higher or with a fam
ily history of premature ASCVD or genetic hyperlipidemia.
A fasting lipid profile (Option D) is unnecessary for this
patient.
XEY POIXIS
o Cardiovascular risk scores can be used to assess a
patient's future risk for major cardiovascular events
and to identify preventive interventions.
. The American College of Cardiologr/American Heart
Association Pooled Cohort Equations are commonly
used to calculate the 10-year risk for atherosclerotic
cardiovascular disease.
Bibliography
Amett DK, Blumenthal RS, Alberr MA, er al. 2019 ACClAHA guideline on the
primary prevention of cardiovascular disease: executive summary: a
report of the American College of Cardiologz/American [{eart Association
Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019:
74:7376-1414. IPMID: 30894319J doi:10.1016/j.jacc.2019.03.009
164

177. Answers and Critiques
tr
Item 20 Answer: A
Educational Objective: Treat non ST-elevation acute
coronary syndrome in a low-risk patient.
Ckrpidogrcl (Option A) is the most appropriate additionat
nlxnagement titr this patieltt with proltable unstable angina.
a lirrnr clf non ST-elevati<;n acutc coroltary s_vnclrome
(NS'lll ACS). F.:rrl1, clopidogrel loacling has been recom
mencled in lrirtients r,l,ith ACS regarclless of reperlr-rsion or
revascularization strategy. In patients prcsenting with signs
and symptor.ns suggestive <tf NS'I'E ACS, risk assesstnent is
indicated to select the trcatment stratct+,. Severirl risk cri
tcria and scorcs are available.
'lhe 'l'lN4l
risk score includes
scvcn indepencler.rt predictors fbr cieath, nonfatal ntyocar
clial infarction. or ischenria requiring revascularization at
1.1 ctays. r,r,ith I poir.rt assigned fbr each ol these preclictors:
agc 6.5 years or older, three rlr lnore coronaly artery disease
(CAI)) risk facturrs (cholestcrol, lamily hiskiry hypcrtcnsion,
cliabctes mellilus. smoking). pr-eviously documented CAI)
(irrrgiographic stenosis >50'l;,). aspiriu use in the past 7 days.
tu() or lrorc ischemic episocles in thc ptst 2.1 l.rours. S'i' seg,
nrent deviation on ECC. and elevatccl cardiac biomarkers
(crcltine kinase MB or tropollin).'lhis pirtient presellts rith
lbatures corlsisterlt u,ith possible ACS but has a'l'lMl sc<lre ol
I uncl is clinicalll stable. [.ow risk p:rticnts undergo noninr,a
sivc stress testing with lcf t vcntricular tirnction ilssesslrent
belirre hospitll clischarge. Clrdiac cathctcrization is reserved
firr patier.rts with active or iutermittent ischemia, including
those lr,ith arrgina despite nredical therirpl, or evi(lence ot'
ischernia on stress testing, and patients iit very higl.r clinical
risk based on risk score.
'll.rc
ischernia guicled approach is
rtppropriate lirr lur,r risk patients (TIMI score <2). pirrticu
hrll lou' risk u,ornen. r,rlto mlry have r,r,orse outconles with
an carly invasive appro:rch.
Nitrates are used prinraril-y to lrrnage angina symptorns
in ACS. Sublingual nitrates should be adlllinistere(l at pre
sentation to relieve chest pailt. Fbr pltients u,-ith persistent
chesl pain despite p blocl<ade, intravcnous nitroglycerin
(Option B) can irlleviate symptoms, plrticularly in those
\'ith hvpertcnsion.
'lhis
prtient does not have chest pain,
and nitroglycerin infusion is not neecled.
If r-rsecl, intravenous gl_Vcoprotein Ilbillla inhibitors.
such as tirolibln (Option C). are gencrirlly reservccl fbr use
during percutancous intcrventior-r: they' should be aclntinis
terccl only in thc catheterizirtion l:rboratorv becausc o[ the
lack olbenefit iurcl increase in bleeding with upfiont aclmin
istrultion in the ernergency clcpartment.
Urgent inr.'asive treatnlent (rvithin 2 hours) (Option D) is
reconrmenclecl ftrr patients rt'ith NSTE r\CS rd-ro hlve herno
dynamic instabilityl ref'ractoly chest pirin, heart f:rilure, or
vcnlricular arrhythmias.
'lhis
patient cloes not have an ir,di
cation Itrr Llrgent coronilrl, angiographl,.
I(EY POIilIS
. In patients with low risk non-ST-elevation acute
coronary syndrome, an ischemia guided strates/ is
appropriate. (Continued)
l(tV P0lXTl (antinued)
. Early loading with either clopidogrel or ticagrelor in
addition to aspirin is recommended in patients with
acute coronary syndrome regardless ofeither an early
invasive strates/ or ischemia-guided strategr.
Bibliography
Anderson JL, Morrow DA. Acute myocardial infarction. N Engl J Med. 2017
May 25;376:2053 64. IPMID: 28538121] doi: 10.1056/NEJMral606915
Item 21 Answer: D
Educational Objective: Reduce cardiovascular risk in a
patient with peripheral artery disease with very low-dose
rivaroxaban therapy.
The most appropriate treatment to reduce cardiovascular
risk in this patient is to initiate very low-dose rivaroxaban
(Option D). In a randomized trial, the addition of very low-
dose rivaroxaban (2.5 mg twice daily) to aspirin (81 mg daily)
was shown to reduce the occurrence ofcardiovascular death.
myocardial infarction, or stroke in patients with peripheral
artery disease (PAD). The absolute risk reduction, when com-
pared with aspirin alone, was 2"1, in 7470 patients enrolled
with either lower extremity PAD or carotid stenosis. Major
bleeding, primarily gastrointestinal bleeding, was increased
by 1% in patients assigned to aspirin plus rivaroxaban. Thus,
low dose rivaroxaban should be avoided in patients with PAD
who have a higher risk for bleeding. It should be noted that
use of rivaroxaban in patients with PAD is at odds with the
2016 American Heart Association/American College of Cardi-
ologz (AHA/ACC) PAD guideline, which recommends against
anticoagulation to reduce the risk for cardiovascular events
in patients with PAD due to lack of beneflt and increased risk
for harm from major bleeding events, including intracranial
bleeding. The studies that informed the AHA/ACC recom-
mendation used warfarin as the anticoagulant.
Cilostazol (Option A), a phosphodiesterase inhibitor
with antiplatelet and vasodilator activity, has been asso
ciated with improvements in pain-free walking distance
and overall walking distance in patients with claudication.
Cilostazol should be considered in this patient in addition to
supervised exercise therapy; however, it will not reduce this
patient's cardiovascular risk.
No study has demonstrated the beneflt of intensi$zing
lipid management, such as with the initiation of ezetimibe
(Option B), when the LDL cholesterol level is below 70 mg/dl
(r.sr mmol/L).
Revascularization, such as with peripheral artery
bypass surgery (Option C), improves symptoms, increases
functional capacity, and improves wound healing (when
applicable) in patients with intermittent claudication or
critical limb ischemia when standard measures, such as
exercise training, cilostazol, and/or wound treatment, are
inadequate. Peripheral artery bypass surgery is not appro
priate for this patient without life limiting symptoms and
would not reduce his cardiovascular risk.
UI
et
ET
lr,
.E,
.E
vt
{,
=
t
165

178. UI
E
.D
UI
q,
EL
n
It
E
.D
t^
Answers and Critiques
In the EUCLID study of patients with symptomatic PAD,
ticagrelor (Option E) was not associated with improved
prevention of cardio'vascular death, myocardial infarction,
or ischemic stroke when compared with clopidogrel; it does
not have a role in the management of patients with PAD for
cardiovascular risk reduction.
TEY POITIS
. In patients with peripheral artery disease, antithrom-
botic therapy with very low-dose rivaroxaban plus
aspirin reduces the occurrence of cardiovascular
death, myocardial infarction, or stroke by 2'7, and
increases the risk for major bleeding by 1'7,.
o No study has demonstrated the benefit of intensifliing
lipid management when the LDL cholesterol level is
below 70 mg/dl (1.81 mmol/L).
Bibliography
Anand SS. Bosch J, Eikelboom lW, et alr COMPASS lnvestigators. Riraroxaban
with or without aspirin in patients s,ith stable peripheral or carotid
artery disease: an intemational. randomised. double blind. placebo
controlled trial. Lancet. 2018r391 :219 229. [PMID: 291328801 doi:10.1016
s0140 6736(17)32,109 i
Item 22 Answer: B
Educational Objective: Evaluate a patient with
advanced heart failure for heart transplantation.
Heart transplantation (Option B) is appropriate for this
patient with advanced heart failure who has signs and symp
toms of a poor prognosis over the next year. He has hypona-
tremia and worsening kidney function, has had recurrent
hospitalizations for heart failure, and has a reduction in
his functional capacity. All of these signs point to a poor
survival over the next year. Patients with persistent severe
heart failure symptoms despite maximal medical therapy
are candidates for advanced treatment. Clinical outcomes
in patients with advanced heart failure undergoing heart
transplantation are excellent. The 1 year survival is around
907,, and median survival is almost 12 years. Acceptable
candidates for transplantation are generally younger than
65 to 70 years with no medical contraindications (e.g., dia
betes mellitus with end organ complications, malignan
cies within 5 years, kidney dysfunction, or other chronic
illnesses that will decrease survival) and have good social
support and adherence.
Digoxin (Option A) has been shown to reduce readmis-
sions in patients with heart failure; however. this drug has
a very small therapeutic window. In this patient with poor
kidney function and older age. digoxin would be a risky drug
because of the potential for an increase in adverse events.
If this patient were older or had comorbid conditions
that would not make him a transplant candidate, he might
be a candidate for left ventricular assist device (I.VAD) place
ment (Option C). With newer continuous-flow devices,
patients have l-year survival approximating that of car-
diac transplant recipients and substantial improvements in
functional capacity and qualily of life. LVAD placement is
currently indicated in patients with ejection lraction less
than 25'., and poor exercise tolerance (Ne'o' York Heart
Association functional class IV) despite maximally toler-
ated therapy, with either a high predicted 1 or 2-year
mortality or inotrope dependency, who still want aggressive
restorative care. Because this patient qualifles for trans
plantation. which is currently associated with better long-
term survival, that would be a better choice. Some patients
receive an LVAD before transplantation if they have acute
decompensation.
Tolvaptan (Option D) is a vasopressin receptor blocker
that is currently approved for the treatment of hypervolemic
and euvolemic hyponatremia with a serum sodium level
less than 125 mEq/L (125 mmol/L). It must be initiated in the
hospital setting and cannot be used for more than 30 days.
Although this patient has hyponatremia, his serum sodium
level is greater than 125 mEq/L (12s mmol/L) and he is not
hospitalized; thus, tolvaptan is not indicated. There is no
evidence that tolvaptan improves chronic heart failure or
clinical outcomes.
XEY POI ]TT
. Patients with persistent severe heart failure symptoms
despite maximal medical therapy are candidates for
advanced treatment; acceptable candidates for cardiac
transplantation are generally younger than 65 to 70 years
with no medical contraindications and have good
social support and adherence.
Bibliography
Guglin M. Zucker MJ. Borlaug BA. et al: ACC Heart Failure and Transplant
l\,,lember Section and I-eadership Council. Ilvalultion fbr heart transplanta
tion and L\AD implantation: JACC Council Perspectives. I Am Coll (lardiol.
'2020
:7 5 :1171 1.187. I PM I I): 32216916] doi : 10. I 0l 6 i.jacc.2020.01.031
Item 23 Answer: B
Educational Objective: Diagnose hypertrophic cardio-
myopathy on physical examination.
The most likely diagnosis is hypertrophic cardiomyopathy
(HCM) (Option B). The murmur of HCM is typically a rap-
idly peaking crescendo decrescendo murmur heard best
along the left lower sternal border. Dynamic maneuvers,
such as Valsalva maneuver or squatting and standing. may
be useful in making the diagnosis. During the strain phase
of Valsalva maneuver, decreased ventricular preload wors
ens the degree of left ventricular outflow tract obstruction,
increasing the intensity of the murmur (positive likelihood
ratio, 14). Squatting increases both preload and afterload,
resulting in a decrease in dynamic left ventricular outflow
tract obstruction and in the intensify of the murmur (pos-
itive likelihood ratio, 7.6). The murmur of HCM typically
does not radiate to the carotid arteries. as it does in aortic
stenosis, and the carotid upstroke is more commonly brisk
and two phased (bifid), reflecting ejection, obstruction, and
a later phase of ejection.
166

179. tr
Bicuspid aortic stenosis (Option A) may be associated
with an early systolic ejection sound (click) that heralds
the murmur. This is heard more commonly while the valve
leaflets remain pliable, before valvular calciflcation pro-
gresses. The murmur radiates to the carotid arteries, and
when signiflcant stenosis is present, the carotid upstroke is
low amplitude and delayed (parvus et tardus). The murmur's
intensity decreases during Valsalva maneuver, with little
change during squatting.
A restrictive membranous ventricular septal defect
(Option C) is not typically associated with symptoms of'
dyspnea. In this condition, a harsh pansystolic murmur is
present at the left lower sternal border. lt does not markedly
change with dynamic maneuvers.
Sinus of Valsalva aneurysm (Option D) of the right or
noncoronary cusp may rupture into the right heart and
is associated with acute dyspnea and decompensation.
Because pressure within the aorta is always higher than in
the right heart, the loud murmur is heard in both systole and
diastole (continuous murmur).
XEY POIXIS
. The murmur of hlpertrophic cardiomyopathy is typi-
cally a rapidly peaking crescendo-decrescendo murmur
heard best along the Ieft lower sternal border.
. Dynamic maneuvers, such as Valsalva maneuver or
squatting and standing, may be useful in diagnosing
hypertrophic cardiomyopathy.
Bibliography
Ommen SR. Mital S. Burke MA. et al. 2020 AIiA,'ACC guideline for the diag
nosis and treatment of patients with hypertrophic cardiomyopathy: a
report ofthe American College ol Cardi0krgl/American Ileart Association
Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2020;
76:e159-e240. IPMID: 33229116] doi: I0.1016ij.jacc.2020.08.045
Item24 Answer: B
Ed u cational Objective: Diagnose microvascular dys-
function as the cause of chest pain in a young woman.
'l
he r.nost likely cause oi'this patient's chest pirin is microvirs
cular cl1'slunction (Option B). In rvorlen r,r'ith typical angina
s!'1r1pton1s. nonobstructi'n'e cororlrir].' stenoses are present
on col-onarl angiography in more than 50'll, of cases. lir-rd
rnicrovascLrlar clysiunction (cr.rdothcliunr dependent or
enclothelium independent) is thought to be a predominant
cluse of symptclms in these patients. Although the epicarclial
coronirry :rrteries are easily visualizecl via coronary angi
ography, the coronary rnicrocirculltion (r,essels <0.5 mm
in cliameter) is not e*sil1,' visualizccl yet accounts lor approx
inrltely 70')1, of the coronary rcsistance ir.r the absence ot
obstructive coronary artery discasc. Inrpaired microcircu
lirtion can be determined b1' several provoc:rtive tests in the
carcl iac catheterization laboratory Clinical disorders of coro
narl microvascular dl,sliurctior.r have largely ireen describecl
in pirtiellts presenting'"vith stablc angin:t.
(irronary lasospasnl (Option A) is sudden coronilry
artery constriction occurring spontancously or fbllowing
Answers and Critiques
use ol illicit substances (rnctharrphetamines. cocaine) or
prescription drugs (5 lluorourircil, bromocriptine). EC(l
abnormalities may be nonspecific or mimic ST elevation
myocardial inlarction patterns. It is a diagnosis ol'exclu
sion and oflen involves coronary ar.rgiography to exclucle
fixed disease. Administratit)n ol nitrates or calcium channel
blocl<ers during cardiac catheterization may show coronary
dilatation, r,vhich may inclicate a vasospastic vessel.
'lhis
patient is in the r,r,ir.rdolv of time fbr an initial pr-e
sentatiur of periparturr cardiomyopathl' (Option C). r,vhich
is charircterized by' increasing dyspnea and heart failure
sylxptoms as opposed to chest pain. the absence ot phy'sical
Iindir.rgs of volume overload mal<es peripaftum cardiomy<-rp
athy unlikely.
Spontaneous coronary artery dissection (SCAI))
(Option D) is a common cause of cl.resl pain among youniler
\{omcn during the peripartum period. SCAD involves devel
oplnent of a nontraumatic ancl noniatrogenic intramtiral
hernatoma. u'ith or rvithout ir.rtinral clissection rith lunti
nal communication. lhe enlarging hematoma ir-r the fhlse
Iumen compresses the true lunren ol the coronary artcr)'
ar.rd il con.rbined u'ith obstrr.rcting dissection, leads to chest
pain, ischemia. and,or inlarction. Indicative findings on
coronary angiography including rnultiple radiolucent
lunrens and perilurninal contrast staining, are absent in
this patient.
t(EY POtllr
o In women with typical angina symptoms, nonob-
structive coronary stenoses are present on coronary
angiography in more than 50% of cases, and micro-
vascular dysfunction is thought to be a predominant
cause of symptoms in these patients.
Bibliography
'l'irmis
Holland JE, Jneid FI, Reynolds llR, et al; American Heart Association
Interventional Cardiovascular Care Committee of the Council on Clinical
Cardiologr; Council on Cardiovascular and Stroke Nursing; Council on
Fipidemiolory and Preventir)n; lnd (iruncil on Quality of Care and
C)utcomes Research. Contenrporary diagnosis and management 01'
patients with myocardial inlarction in the absence ofobstructive coronary
artery disease: a scientific statement fnrm the American Heart Association.
Circulation. 2019;139:e891 e908. IPMID: 309138931 doi:10.1161rCIR.
0000000000000670
Item 25 Answer: C
Educational Objective: Diagnose atrioventricular nodal
reentrant tachycardia.
This patient's presentation is typical for atrioventricu
lar nodal reentrant tachycardia (AVNRT) (Option C), the
most common form of supraventricular tachycardia (SVT).
SVTs are rapid heart rhythms that arise from the atrium
or require conduction through the atrioventricular node.
SVTs usually occur in the absence of structural heart dis
ease, although echocardiography should be performed to
exclude underlying cardiac dysfunction or structural defects.
Patients often have repeated episodes of tachycardia and
may report palpitations, a sensation of pounding in the
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180. Answers and Critiques
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neck, fatigue, light headedness, chest discomfort, dyspnea,
presyncope, and, Iess commonly, syncope. AVNRT accounts
for approximately two thirds of all cases of SW. It frequently
occurs in women and is often terminated by performing
vagal maneuvers, as with this patient. The ECG typically
demonstrates a narrow QRS complex. AVNRT is character
ized by a short RP interval with a retrograde P wave (orrouus)
inscribed very close to the QRS complex, which is best seen
in lead V, (appearing as a pseudo r'wave). In the emergency
department, adenosine is commonly used for acute termi
nation. Cardioversion is rarely required. Ultimately, catheter
ablation may be curative and is often flrst-line therapy, but
AVNRT also can be managed conservatively at the patient's
discretion.
Item 26 Answer: D
Educational Objective: Treat severe secondary mitral
regurgitation with guideline-directed medical therapy.
No change in therapy (Option D) is the most appropriate
management. This patient's echocardiogram is consistent
with severe secondary mitral regurgitation (MR). Left ven
tricular systolic dysfunction is the most common cause of
chronic secondary MR. Consequently, guideline-directed
medical therapy (GDMT) is loundational in improving
symptoms in patients such as this one. GDMT includes a
p-blocker, an ACE inhibitor or angiotensin receptor blocker,
and an aldosterone antagonist. Loop diuretics are used as
needed. GDMT improves symptoms and prolongs life in
patients with heart failure and probably does so when heart
failure is complicated by chronic secondary MR. Current
guidelines recommend GDMT as flrst line therapy (class
1 recommendation) because it can reduce left ventricular
volumes (reverse remodeling) in many patients and, in so
doing, reduces severity ofsecondary MR.
Cardiac magnetic resonance imaging (Option A) or
transesophageal echocardiography may be pursued if trans-
thoracic echocardiography is insufficient to determine either
the exact severity or the mechanism of mitral regurgitation.
Howevel in this case, the severity and mechanism of MR are
not in question.
GDMT also includes consideration of cardiac reqmchroni-
zation therapy (CRI) (Option B) in appropriate patients. Among
patients with a left ventricular ejection fraction (LVEF) of 35'U,
or less and sinus rhythm, CRT is indicated in those with left
bundle branch block, New York Heart Association functional
class II to ambulatory class lV heart failure ry.rnptoms, and a
QRS duration of 150 ms or longer despite GDMT. In addition, in
patients with an L\EF of 35'7, or less and a QRS duration of 120
to 149 ms, CRI may be useful. This patient now has New York
Heart Association functional class I symptoms with an LVEF
greater than 35% and therefore does not qualifiz for CRL
In patients with chronic severe secondary MR related
to left ventricular systolic dysfunction who have persistent
symptoms while receiving optimal GDMT, transcatheter edge
to edge repair (TEER) is a reasonable option (class 2a recom
mendation), according to current guidelines. Surgical mitral
valve repair (Option C) is also reasonable if the patient is
undergoing concomitant coronary artery bypass graft surgery.
Surgery also may be considered if the patient has unfavorable
anatomy for TEER. However, this patient should undergo a
trial of GDMT before mitral valve repair is considered.
r(Ev P0lrTs
. Left ventricular systolic dysfunction is the most com-
mon cause of chronic secondary mitral regurgitation.
. Guideline-directed medical therapy is recommended as
first-line therapy for patients with heart failure and sec-
ondary mitral regurgitation because it can reduce left
ventricular volumes in many patients and, in so doing,
reduces severity of secondary mitral regurgitation.
Atrial flbrillation (Option A) is a narrow-complex
tachycardia primarily deflned by the absence of regular P
waves and presence of an irregular ventricular response.
The patient's ECG has a regular tachycardia with evidence
of retrograde P waves, which is not consistent with atrial
flbrillation.
Atrial tachycardia (Option B) is an automatic SVT that
is less common than AVNRT, is less likely to start abruptly,
and is not usually terminated with vagal maneuvers. The
ECG more commonly shows discrete atrial activity, with an
abnormal P wave axis, and the rate is usually slower than
that seen in this patient.
Ventricular tachycardia (Option D) is a wide-
complex tachycardia. In contrast, this patient presents
with a narrow complex tachycardia. Among patients
with a structurally normal heart and no cardiac history,
ventricular tachycardia would be much less common
than AVNRT.
t(EY P0t 1{r5
. Atrioventricular nodal reentrant tachycardia, the most
common form of supraventricular tachycardia, Bpi
cally has a narrow
QRS complex; it often can be ter-
minated by performing vagal maneuvers.
o Atrioventricular nodal reentrant tachycardia is char-
acterized electrocardiographically by a short RP inter-
val with a retrograde P wave inscribed very close to
the QRS complex, which is best seen in lead V,,
appearing as a pseudo r'wave.
Bibliography
Kotadia ID, Williams SE, O'Neill M. Supraventricular tachycardia: an over
view of diagnosis and management. Clin Med (Lond). 2020;20:43 7.
IPM I D: 319417311 doi:10.7861/clinmed.cme.20.1.3
168

181. Answers and Critiques
Bibliography
tsonow RO, O'Gara PT, Adams DH. et al. 2020 Focused update ofthe 20U
ACC expert consensus decision pathway on the management of mitral
regurgitation: a report ofthe American &rllege ofCardiolory Solution Set
Oversight Committee. J Am Coll (lardiol. 2020;75:2236-2270. IPMID:
320680841 doi:10.1016/i.jacc.202O.02.0Os
Item 27 Answer: C
Educational Objective: Treat patent ductus arteriosus.
The most appropriate management is patent ductus arteri-
osus (PDA) device closure (Option C). A PDA is a persistent
f'etal connection between the aorta and the left pulmo
nary artery that leads to volume overload of the left-sided
chambers, manifested by left atrial and left ventricular
enlargement. The typical murmur of a PDA is a continuous
"machinery" murmur that envelops the Sr, making it inau
dible; the murmur is heard beneath the left clavicle. A tiny
PDA is generally asymptomatic and inaudible. Closure of the
PDA is indicated in patients with left sided cardiac chamber
enlargement as long as pulmonary artery systolic pressure
is less than 50% systemic, even in the absence of symptoms.
Closure may be considered in patients with some degree
of pulmonary hypertension in selected cases (pulmonary
artery pressure 50"/,'-66"1' systemic). Percutaneous closure
is usually performed; referral to a congenital cardiac center
fbr consideration of closure options is recommended. PDA
closure should be avoided in patients with irreversible pul-
monary vascular disease. PDA closure in patients with severe
pulmonary hypertension (pulmonary artery systolic pres
sure >66'l,, systemic) is associated with greater risk compared
with PDA closure in those without pulmonary hypertension
and is not associated with improved survival. In patients
with pulmonary hypertension, the existence of right-to-left
ductal shunting may be necessary to maintain cardiac out-
put, and closure may result in clinical worsening.
Cardiac magnetic resonance imaging (Option A) will
further delineate cardiac chamber size and function and
demonstrate the dimension and length of the PDA. However,
adequate data are available from the echocardiogram to con-
firm that PDA closure is indicated.
lndomethacin (Option B), an inhibitor of prostaglandin
synthesis, is used to promote closure of a PDA in preterm
infants. However, indomethacin has no impact on closure
rates in full-term infants and older patients with a PDA and
is not indicated in this patient.
Serial echocardiographic monitoring (Option D) is not
recommended in patients with a PDA and left to right shunt
large enough to have caused left heart enlargement. Device
closure should be pursued in this patient before irreversible
pulmonary hypertension develops.
t( EY P0 r 1{TS
. The typical murmur of a patent ductus arteriosus is a
continuous "machinery" murmur that envelops the
52, making it inaudible; the murmur is heard beneath
the left clavicle.
(Continued)
xEY P0lxTS (oilinued)
. Patent ductus arteriosus closure is indicated in
patients with left-sided cardiac chamber enlargement
even in the absence of symptoms, as long as pulmonary
artery systolic pressure is less than 50% systemic.
Bibliography
Stout KK, Daniels Cl, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the
management of adults with congenital heart disease: a report of the
American College of Cardiolory/American Heart Association Task Force
on Clinical Practice Guidelines. J Am Coll Cardiol. 2Ol9;73:e81 e192.
IPMID: 30121239] doi:10.1016/j.jacc.2018.08.1029
Item 28 Answer: A
Educational Objective: Diagnose peripheral artery dis-
ease in a patient with normal ankle-brachial index values.
The most appropriate next step is exercise ankle brachial
index (ABI) testing (Option A). This patient has limb symp-
toms consistent with peripheral artery disease (PAD). Gen
erally, patients with claudication have an ABI of 0.40 to 0.90,
whereas patients with ischemic rest pain, ulceration, or
gangrene have an ABI less than 0.40. A resting ABI greater
than 1.40 indicates the presence of noncompressible, cal
cifled arteries in the lower extremities and is considered
uninterpretable. Between 79"1' and 317, of patients with typi
cal claudication symptoms have a normal or borderline ABI.
Because this patient with classic claudication symptoms
and faint pulses has a nondiagnostic ABI, further testing is
indicated. Exercise ABI testing is useful in patients with ABI
values between 0.91 and 1.40 and high pretest probability of
PAD. It requires ABI measurements at rest and after treadmill
walking or plantar flexion exercises. A post-exercise ankle
pressure drop of 30 mm Hg or more or signiflcant decline in
the ABI suggests PAD.
Invasive angiography (Option B) is often reserved for
patients with an indication for revascularization, usually
either intermittent claudication or chronic limb-threatening
ischemia. Likewise, noninvasive anatomic imaging studies,
including arterial duplex ultrasonography, CT angiography,
and magnetic resonance angiography (Option C), are used
to plan for endovascular or surgical revascularization. This
patient with typical limb symptoms, normal resting ABI
values, and no contraindication to exercise should undergo
noninvasive physiologic testing, such as exercise ABI, to
conflrm the diagnosis of PAD before anatomic assessment
is considered.
Segmental blood pressure measurements (Option D)
of the lower extremities are typically performed in patients
with abnormal resting ABI values to localize diseased ves
sels or segments. This procedure involves pulse volume
recordings (measurement of the magnitude and contour
of blood pulse volume in the lower extremities) and blood
pressure measurements at several locations in the lower
extremities. In this patient, the resting ABI was normal;
thus, the indication for segmental blood pressure mea
surement is unclear.
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Answers and Critiques
KEY POII{It
. Betvveen 19'l. and 31'l. of patients with typical claudi
cation symptoms have a normal or borderline ankle-
brachial index.
. Exercise ankle brachial index (ABI) testing is useful to
evaluate for peripheral artery disease in patients with
normal ABI values (>0.90 and <1.40) and high pretest
probability.
Bibliography
Mehta A, Sperling LS, Wells BJ. Postexercise ankle brachial inde-x testing.
JAMA. 2020;324:796 7. IPMID: lZtZtOOt] doi:10.1001/jama.2020.1016.1
Item 29 Answer: C
Educational Objective: Treat multivessel coronary
artery disease in a patient with an ST-elevation myocardial
infarction.
The most appropriate management is right coronary artery
revascularization (Option C). This patient presents with an
anterior ST-elevation myocardial infarction (STEMI). Prompt
reperfusion with primary percutaneous coronary interven-
tion (PCI) is indicated. Because rates of achieving vessel
patency are higher and more reliable with primary PCI than
with thrombolysis, primary PCI is the pref'erred method of
treating STEMI. In addition to the left anterior descending
artery culprit lesion, the patient has obstruction to the mid
right coronary artery. Approximately 50'/o of patients with
STEMI have other obstructive lesions remote from the area of
infarction ("nonculprit" lesions). Several studies have shown
the beneflt of nonculprit lesion revascularization within 4
to 6 weeks following STEMI. Although optimal timing of
nonculprit PCI has not been Iirmly established, prospective
studies have shown the safety and beneflt of immediate mul
tivessel revascularization.
There is no indication for initiation ofpotent antiplatelet
therapy, such as intravenous glycoprotein IIb/llla inhibition
(Option A), following successful primary PCI in the absence
of thrombotic complications, because the hemorrhagic risk
outweighs potential reduction in thrombosis in patients
treated appropriately with oral dual antiplatelet therapy.
Low risk patients with non-ST elevation acute coro
nary syndrome can be managed with an ischemia guided
strategy. These patients will undergo noninvasive stress
testing before hospital discharge. Cardiac catheterization is
reserved for patients rvith active or intermittent ischemia,
including those with angina despite medical therapy or evi
dence of ischemia on stress testing. This patient with STEMI
has undergone revascularization of a culprit lesion and will
undergo revascularization of a nonculprit lesion; predis
charge exercise ECG (Option B) will serve no useful purpose.
Supplemental oxygen (Option D) in the setting of nor-
mal oxygen saturation is associated with increased mortality
in patients with acute coronary syndrome. The American
Heart Association recommends oxygen therapy for oxygen
saturation less than 90'l. or in the presence ofheart failure or
dyspnea. This patient has no need for supplemental oxygen
therapy.
l( EY PO I t{rS
. The preferred method of treating ST elevation myo
cardial infarction is primary percutaneous coronary
intervention.
. Approximately 50% of patients with ST elevation
myocardial infarction (STEMI) have other obstructive
lesions remote from the area of infarction ("noncul-
prit" lesions); studies have shown the benefit of non
culprit lesion revascularization within 4 to 6 weeks
following STEMI.
Bibtiography
Mehta SR. Wbod DA. Storey RFL er al; COMPLETE Trial Sreering Commitree
irnd lnvestigators. Complete revascularization with multivessel PCI fbr
m1'ocardial infarction. N Engl J Med. 2019:381:1.111 21. [PN{ID,
yqlSlSSl
doi:10.'l 0561 NEIM oa1917775
Item 30 Answer: C
Educational Objective: Evaluate palpitations with a
l2-lead resting ECG.
The most appropriate initial test for this patient with pal
pitations is l2-lead resting ECG (Option C). The initial test
in patients with a history of palpitations, presyncope, or
syncope when an arrhythmia is suspected should be 12 lead
resting ECG. The ECG may show evidence of preexcitation,
ectopic rhythms, atrioventricular block, or intraventricular
conduction delay, which can provide insight into the cause
of the symptoms. Other conditions that might be suspected
from the 12 lead resting ECG include hypertrophic cardio
myopathy (marked left ventricular hypertrophy and deep
septal Q waves in leads I, aVL, and V, through Vu) or previous
myocardial infarction (Q waves), each suggesting the possi
bility of ventricular tachycardia. Ventricular tachycardia also
might be suspected if the ECG demonstrated a prolonged
QT interval. The need for further testing is determined by
the ECG findings and suspicion of structural or functional
heart disease.
Findings on the 12-lead resting ECG that indicate pos
sible ischemia as the cause of the symptoms might lead
to exercise ECG (Option A) as the next step. This patient's
differential diagnosis is still broad, as he has no history or
physical examination findings that suggest a heightened risk
for premature coronary artery disease; exercise ECG is not
indicated as the initial diagnostic test.
A 30 day event monitor (Option B) is useful for infre
quent symptoms that are thought to be related to an arrhyth
mia. Use of an event monitor is ideal when symptoms last
longer than 1 to 2 minutes so that patients can activate the
monitor in time for the symptoms to be recorded. These
monitors can be carried around and then held to the chest
when symptoms occur or attached to the chest via elec-
trodes. However, even if the 12-lead resting ECG were nor
mal, this patient has frequent symptoms, occurring daily. In
170

183. this situation, a 24-hour ambulatory ECG monitor would be
a more suitable diagnostic test than an event monitor.
A 24 hour ambulatory IjCG monitor (Option D) is
indicated for patients with daily symptoms after a resting
12 lead ECG is obtained. This continuous monitor captures
every heartbeat for 24 hours (or up to 30 days) and allows
the patient to log symptoms during the course of the study.
It may be that this patient will require 24 hour ambula
tory ECG monitoring, but the initial test remains 12 lead
resting ECG.
I(EY POITT
. The initial test in patients with a history of palpitations,
presyncope, or syncope is l2-lead resting ECG.
Bibliography
Weinstock C, Wagner II. Snuckel M, et al. Ilvidence based approach to pal
pitations. Med Clin North Am. 2021r105:93 106. IPMID: 3324652.51
doi: 10. 101 6rj.mcna.2020.09.00.1
Item 31 Answer: B
Educational Objective: Treat heart failure with reduced
ejection fraction in a Black patient.
The most appropriate treatment is isosorbide dinitrate
hydralazine (Option B). This young Black woman has peri
partum cardiomyopathy, New York Heart Association func-
tional class III symptoms, and heart failure. At baseline, she
is receiving appropriate guideline directed medical ther-
apy (GDMT). Despite this, she has elevated blood pressure
and heart failure symptoms. In Black patients receiving
GDMT, isosorbide dinitrate hydralazine has been shown
to improve survival by 43"1,, reduce flrst hospitalizations by
3ll'/,, and improve quality of litb.
Digoxin (Option A) has been shown to reduce hospital
admissions in patients with heart failure, with no effect on
mortality. Compared with isosorbide dinitrate-hydralazine,
which shows a mortality benefit, digoxin would not be the
best next agent to add to this patient's regimen.
In patients with type 2 diabetes mellitus and established
heart tailure with reduced ejection fraction, a sodium
glucose cotransporter 2 (SGLI2) inhibitor with proven ben
efit in this patient population is recommended to reduce
risk for worsening heart failure and cardiovascular death.
ln patients with diabetes and established atherosclerotic
cardiovascular disease (ASCVD) or several ASCVD risk fac-
tors, either an SGLT2 inhibitor or a glucagon like peptide
I receptor agonist, such as liraglutide (Option C), is recom
mended to reduce both cardiovascular and all cause mor
tatity. Liraglutide is not indicated tbr this patient who has
heart failure in the absence ofASCVD risk factors and does
not have diabetes.
Adding an ACE inhibitor, such as lisinopril (Option D), to
this patient's medication regimen is not appropriate because
she is already taking valsartan-sacubitril, which in random
ized trials was shown to improve survival compared with the
ACE inhibitor enalapril. In addition, using valsartan sacubitril
Answers and Critiques
with an ACE inhibitor increases the risk for angioedema;
therefore, these agents should not be used together. In fact,
ACE inhibitors must be discontinued at least 36 hours before
staft ing valsartan sacubitril.
I(EY POIXI
. In Black patients with New York Heart Association func
tional class III to IV slmptoms, isosorbide dinitrate
hydralazine used in combination with an ACE inhibitor,
B-blockea and aldosterone antagonist has been shov,rn
to reduce mortality compared with placebo.
Bibliography
(larnethon
MR, Pu l, Howard G, et ll; American Head Association Council
on Iipiclemiologr and Prevention; Council on Cardiovascular Disease in
the Young; Council on Cardiovascular and Stroke Nursing; Council on
Clinical Cardiolo$/; Council on Functional Genomics and Translational
Biolog,: and Stroke Council. Cardiovlscuhr health in African Americans:
a scientific statement fiom the American Heart Association. Circulation.
2017:136:e393 e,l23. IPMII): 29061565] doi:10.1161/CIR.0000000
00000053.1
Item 32 Answer: B
Educational Objective: Treat typical atrial flutter with
catheter ablation.
Catheter ablation (Option B) is the most appropriate addi
tional treatment for this patient with typical atrial flutter.
Atrial flutter is an organized macro reentrant tachycardia
with discrete regular atrial activity on ECG, usually with an
atrial rate of 250/min to 300/min. This patient has an atrial
rate of 300/min with 2:1 block, resulting in a ventricular rate
of 150/min. Typical atrial flutter is characterized electrocar
diographically by a sawtooth pattern with inverted flutter
waves in leads II, III, and aVF and positive flutter waves in
lead V,. Tachycardia induced heart failure (HF) may be the
initial presentation of poorly controlled atrial flutter among
patients who are otherwise asymptomatic, and unrecog
nized, prolonged tachycardia may lead to HFl, This form of
cardiomyopathy is often reversible with control of heart rate
and/or rhythm. In typical atrial flutter, catheter ablation may
be curative for most patients and is appropriate first line
therapy for this patient. Acute cardioversion was a reasonable
choice for this patient to achieve acute improvement in clini
cal status. However, it is extremely likely that atrial flutter will
recur, which makes this patient at very high risk for recurrent
HF and underscores the need for definitive treatment.
A rhythm control strate$/ is favored in atrial flutter
because rate control may be difficult to achieve and often
requires high doses of more than one drug. However, drug
therapy (Options A, C, D) may be complicated, toxic, and
often unsuccessful. p Blockers are unlikely to prevent or
treat a recurrence. Catheter ablation is the deflnitive treat
ment for typical atrial flutter, owing to a very high success
rate (>95'/.) and low complication rate.
Because the risk for recurrence is high and this patient
poorly tolerates the rapid ventricular rate, observation with
out additional treatment (Option E) is insufficient for this
patient. Catheter ablation is the most appropriate treatment.
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Answers and Critiques
I(EY PO I ilI
. Catheter ablation is an appropriate treatment for
patients with atrial flutter.
Bibliography
Page RL, loglar JA, Caldwell MA, et al. 2015 ACC/AHA/HRS guideline for the
management of adult patients with supraventricular tachycardia: execu
tive summary: a report of the American College of Cardiolory/American
Heart Association Task Force on Clinical Practice Guidelines and the
Heart Rhythm Societll Heart Rhythm. 2016r13:e92-135. IPMID:
264090971 doi:10.1016,i. hrrhm.2O15. 09.018
Item 33 Answer: D
Educational Objective: Treat peripheral artery disease
with guideline-directed medical therapy.
This patient requires no additional treatment (Option D).
Patients with peripheral artery disease (PAD) are less Iikely
to receive guideline-directed medical therapy than are
patients with other forms of cardiovascular disease, includ-
ing coronary artery disease. Antiplatelet therapy with aspi-
rin alone (75 325 mg/d) or clopidogrel alone (75 mg/d) is
recommended to reduce myocardial infarction (MI), stroke,
and vascular death in patients with symptomatic PAD. Treat
ment with a statin is indicated for all patients with PAD.
Antihypertensive therapy should be administered to patients
with hypertension and PAD to reduce the risk for MI, stroke,
heart failure, and cardiovascular death. Patients with PAD
who smoke cigarettes or use other forms of tobacco should
be advised at every visit to quit. Although not guideline rec-
ommended, the combination of low-dose rivaroxaban plus
low-dose aspirin in patients with PAD may reduce cardio
vascular death, MI, or stroke compared with aspirin alone.
This patient is receiving appropriate management for PAD,
and no additional treatment is recommended at this time.
Cilostazol (Option A) is recommended for patients
with PAD and intermittent claudication to improve symp-
toms and maximal walking distance; however, cilostazol
has no impact on mortaliry MI, or stroke. Furthermore, this
patient is symptom-free and therefore has no indication for
cilostazol.
Evolocumab (Option B) is a proprotein convertase
subtilisin/kexin type 9 (PCSK9) inhibitor. Treatment with
PCSK9 inhibitors produces a 50'2, to 60'2, reduction in LDL
cholesterol. The American College of Cardiologr and Ameri
can Heart Association guidelines suggest an LDL cholesterol
target of less than 70 mg/dl (1.8 mmol/L) for patients at very
high risk for future atherosclerotic cardiovascular events.
However, this patient has an LDL cholesterol level of 50 mg/dl
(t.3 mmol/L) while being treated with rosuvastatin; treat-
ment with evolocumab is not indicated.
Ticagrelor (Option C) currently does not have an indi-
cation for use in patients with PAD. In the EUCLID study of
13,885 patients with lower extremity PAD, monotherapy with
ticagrelor had very similar effectiveness and safety outcomes
to clopidogrel. The use of ticagrelor in this setting, when
added to aspirin, has not been shown to decrease cardiovas-
cular risk.
I(EY POITT
. Guideline-directed medical therapy for peripheral
artery disease includes exercise; an antiplatelet agent;
and aggressive management of atherosclerotic risk
factors, including smoking, hypertension, diabetes
mellitus, and dyslipidemia.
Bibliography
Gerhard-Herman MD. Gornik HL, Barrett C, et dl. 2016 AHATACC guideline
on the management of patients with lower extremity peripheral artery
disease: executive summary: a report of the American College of
Cardiolory/American Heart Association Task Force on Clinical Practice
Cuidelines. J Am Coll Cardiol. 2017;69:1.165-1508. [PMID: 27851991]
doi:10.1016/j.jacc.2016.11.008
Item 34 Answer: C
Educational Objective: Monitor a patient with bicuspid
aortic valve and aortopathy with surveillance echocardi-
ography.
The most appropriate management is serial echocardio
graphic imaging (Option C). Bicuspid valvulopathy is often
accompanied by aortic abnormalities, including aneurysm,
dissection, or coarctation. In patients with a bicuspid aortic
valve, the ascending aorta and aortic arch should be exam
ined for aortopathy with cardiac magnetic resonance (CMR)
imaging, echocardiography, or cardiac CT. In patients with
a bicuspid aortic valve and aortic sinuses or an ascending
aorta 4.0 cm or Iarger in diameter, lifelong serial evalua
tion of the size and morphologr of the aortic sinuses and
ascending aorta by echocardiography, CMR imaging, or CT
angiography is reasonable. This otherwise healthy patient
with mild aortic regurgitation and an ascending aortic root
diameter below the threshold for repair should undergo
repeat echocardiography for surveillance.
Management of bicuspid aortic valve disease is deter
mined by the predominant lesion type (stenosis or regur-
gitation) and its severity. In patients with a bicuspid valve
undergoing surgery for severe aortic stenosis or regurgi
tation, surgical repair of the ascending aorta (Option A) is
advised when the aortic diameter is greater than 4.5 cm. In
the absence of surgical indications for a stenotic or regur
gitant aortic valve, surgical repair of the ascending aorta or
aortic sinuses is advised when the aortic diameter is greater
than 5.5 cm or when the diameter is greater than 5.0 cm in a
patient with additional risk factors for dissection (family his-
tory rate of progression >0.5 cm/year). This asymptomatic
patient does not have an indication for surgical intervention.
In patients with a bicuspid aortic valve, CMR imaging
(Option B), angiography, or CT angiography is indicated if
morphologz of the aortic sinuses, sinotubular junction, or
ascending aorta cannot be assessed accurately or fully by
echocardiography. This patient has no indication for CMR
imaging.
No medical therapies slow aortic dilatation in patients
with bicuspid aortopathy. Blood pressure should be con-
trolled in patients with concomitant hypertension by using
172

185. standard drug therapy, including thiazide diuretics, calcium
channel blockers, ACE inhibitors, or angiotensin receptor
blockers. Angiotensin receptor blockers, such as losartan
(Option D), slow the rate of progressive aortic root dilation
in patients with Marfan aortopathy, even in the absence
of hypertension, but are not eflective in bicuspid aortopa-
thy and are not indicated in this patient in the absence of
hypertension.
TEY POIilIS
. Management of bicuspid aortic valve disease follows
the recommendations for the predominant valve
lesion type (aortic stenosis or regurgitation) and its
severity.
. In patients with a bicuspid aortic valve and aortic
sinuses or an ascending aorta 4.0 cm or larger in
diameter, lifelong serial imaging is reasonable.
Bibliography
Otto CM, Nishimura RA. Bonow RO, et al. 2020 ACC/AHA guideline for the
management of patients with valvular heart disease: a report of the
American College of CardiolorylAmerican Heart Association loint
Committee on Clinical Practice Cuidelines. Circulation. 2o2l:143:e72
e227. IPMID: 33332150] doi:10.1161/CIR.0000000000000923
Item 35 Answer: D
Educational Obiective: Treat recurrent pericarditis.
The most appropriate treatment is triple therapy with an
NSAID, such as ibuprofen; colchicine; and a glucocorti-
coid, such as prednisone (Option D). This patient meets
the deflnition of recurrent idiopathic pericarditis, having a
documented flrst episode of acute pericarditis flollowed by
a recurrent episode after a symptom-free interval of 4 to
6 weeks. This patient had a favorable response to initial treat
ment with an NSAID and colchicine, followed by recurrent
symptoms after tapering standard therapy on two difl'erent
occasions. Recurrence occurs in 15'2, to 30'/. of pericarditis
cases, and the recurrence rate is higher when initial therapy
does not include colchicine. In patients initially treated only
with NSAIDs, it would be reasonable to use the combi
nation of NSAIDs and colchicine to treat a recurrence. In
patients initially treated with both colchicine and NSAIDs,
the addition of a low to moderate dose of prednisone is
reasonable to achieve better control of symptoms. When
patients re-present with pain but without other evidence
of pericarditis, cardiac CT or MRI and measurement of
C-reactive protein level may infbrm the decision of whether
to add glucocorticoids. Infectious diseases, including tuber
culosis, must be excluded before starting a glucocorticoid.
Glucocorticoids are not recommended as flrst line therapy
fbr acute pericarditis.
The interleukin 1 receptor antagonist anakinra
(Option A) has shown benefit in small trials for treatment of
colchicine-resistant, glucocorticoid dependent recurrent
pericarditis, but it is not, at present, FDA approved for this
use. Consideration should be given to initiating rilonacept,
an FDA approved interleukin 1 trap that demonstrated
Answers and Critiques
early and sustained relief of signs and symptoms of inflam
mation and signiflcant reduction in the risk for recurrent
pericarditis.
Intravenous immune globulin (Option B) has been used
with some success to treat pericarditis that recurs despite
combination therapy including prednisone. For this patient,
glucocorticoid treatment should precede consideration of
intravenous immune globulin.
Ibuprofen monotherapy without colchicine (Option C)
may be considered to treat an initial episode of acute peri-
carditis, but it is unlikely to be eflective in patients with
recurrent pericarditis.
KEY POIIII
. In patients with recurrent pericarditis initially treated
with both colchicine and an NSAID, the addition of a
glucocorticoid should be considered.
Bibliography
Adler Y, Charron R Imazio M, et al; ESC Scientific Document Group. 2015
ESC guidelines for the diagnosis and management ofpericardial diseases:
The Task Force for the Diagnosis and Management ofPericardial Diseases
of the European Society of Cardiologl (ESC) Endorsed by: The European
Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J. 2015;36:
2927-64. IPMID: 26320112] doi:10.1093/eurhearti/ehv318
Item 36 Answer: D
Educational Objective: Treat a patient with extended
dual antiplatelet therapy following percutaneous
intervention.
Dual antiplatelet therapy (DAPT) beyond l year (Option D)
is reasonable for this patient with acute coronary syndrome
(ACS) to reduce the risk for repeat myocardial infarction.
DAPT with aspirin and P2Yr2 inhibition is recommended
for I year after ACS presentation with ST-elevation myo-
cardial infarction (STEMI) or non STEMI. Clopidogrel or
ticagrelor is typically indicated as the P2Y,, inhibitor,
regardless of revascularization status, and prasugrel is
reserved for patients treated with percutaneous coronary
intervention. Whereas lifelong aspirin therapy is recom
mended as secondary prevention after discontinuation of
the P2Y,, inhibitor, guidelines indicate that it is reasonable
to extend DAPT beyond 12 months (up to 36 months) in
patients who have successfully tolerated 12 months of DAPT
and remain at high risk for recurrent vascular events (e.9.,
those with depressed left ventricular systolic function,
saphenous vein graft stenting, or diabetes mellitus), for
whom the beneflt exceeds the increased bleeding risk. A
personalized approach is appropriate when considering
whether to extend DAPT, with readily available risk scores,
such as the DAPT bleeding risk score, which provides com
posite risk beneflt assessment for patients considered for
DAPT extension.
Low-dose aspirin is the preferred single antiplatelet
agent beyond 1 year after ACS because of its low cost, effec
tiveness, and lower risk for bleeding compared with P2Yr2
inhibitors. Clopidogrel is a reasonable substitute for aspirin
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in patients with aspirin sensitivity or intolerance, or those
with a history of upper gastrointestinal bleeding. There is no
indication to stop aspirin (Option A) in this patient.
Stopping both aspirin and clopidogrel (Option B) is not
indicated. This patient needs an antiplatelet agent consisting
of aspirin, clopidogrel, or both to reduce the risk for stent
thrombosis and myocardial infarction, stroke, and other
cardiovascular events.
Evidence from the COMPASS (Rivaroxaban for the
Prevention of Major Cardiovascular Events in Coronary or
Peripheral Artery Disease) trial demonstrated that aspirin
(too mg/d) plus rivaroxaban (2.s mg twice daily), a direct
factor Xa inhibitor, was associated with improved major
adverse cardiovascular and limb end points compared with
aspirin plus placebo in patients with coronary artery dis
ease and peripheral artery disease (PAD). This patient does
not have PAD, and aspirin plus rivaroxaban (Option C) is
not indicated.
XEY POITI
o Extending dual antiplatelet therapy (DA[T) beyond
12 months is reasonable in patients who have success-
fully tolerated 12 months of DAPT and remain at high
risk for recurrent vascular events ifthe benefit exceeds
the risk for increased bleeding.
Bibliography
l.evine GN. Bates ER, Bittl JA, et al. 2016 ACC/AHA guideline focused update
on duration ofdual antiplatelet therapy in patients with coronary artery
disease: a report of the American College of Cardiolosr/American Heart
Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol.
2O16;68:1082-115. IPMID: 27036918] doi:10.1016/j.jacc.2016.03.513
Item 37 Answer: A
Educational Objective: Diagnose ventricular tachycardia.
The most likely diagnosis fbr this wide-complex tachycardia
is ventricular tachycardia (VT) (Option A). A wide-complex
tachycardia is any tachycardia with a QRS complex of 120 ms
or longer. Differential diagnoses include suprar,entricular
tachycardia (SVT) with aberrancy, preexcited tachycardia
(antidromic tachycardia), ventricular paced rhythm, and
VT. In adult patients with structural heart disease, 95'71, of
wide-complex tachycardias are VT. Several important clin
ical and ECG features can distinguish VT from other condi
tions. The presence of irregular jugular venous pulsations
of greater amplitude than normai venous waves (cannon
waves) signal the presence of atrioventricuiar dissociation
IIEM 37
and support the diagnosis of'VT. Wide complex tachycardias
that are positive in lead aVR, have a QRS morpholory that is
concordant (all predominantly positive or negative) in the
precordial leads, have QRS morphologr other than typi'
cal right or left bundle branch block, and exhibit extreme
axis deviation ("northwest" axis) are usually VT. Fusion
beats (suprar,entricular and ventricular impulses coinciding
to produce a hybrid complex) (orrou's in the ECG shown
belou') and capture beats (a sinus conducted beat producing
a normal QRS) are all highly suggestive of VT. Of importance,
the fact that the patient is awake. alert. and interactive and,
or has a measurable bloocl pressure does not exclude VT.
lf the origin of a wide-complex tachycardia cannot be
determined, VT should be assumed until expert consultation
can be obtained. This patient with structural heart disease
(in the form of ischemic hearl disease) has ECG findings
characteristic of VT. It is monomorphic VT because consecu
-
tive beats have a uniform and stable QRS morpholog,l
Antidromic atrioventricular reciprocating tachy'cardia is
a preexcited tachycardia (Option B) that is characterized by
a u,ide. slurred
QRS complex resulting from conduction orer
the bypass tract and activation ol the ventricle r,r.ithout use of
the specialized conduction system. lt may be lery difficult to
distingrish from VT on a single ECG tracing. Houever, this
tachycardia tends to occur in younger people, and it is not
associated with cannon o waves, lusion beats, or capture beats.
Antidromic atrioventricular reciprocating tachycardia is much
less likely than VT in this patient with structural heart disease.
SW associated with a bundle branch block or a nonspe
cific intraventricular conduction delay (Option C) will present
as a n ide-complex tachlrcardia (SVf u,ith aberranry). However,
the same reasons that make antidromic atrioventricular recip-
rocating tachycardia unlikely apply to S\T with aberrancy.
Torsades de pointes (Option D) is a polymorphic VT
with ventricular rates from l60rmin to 250imin. The tor
sades de pointes pattern is associated with QT intervai pro
longation and is characterized by changing ofthe QRS axis
by 180 degrees every few beats. This pattern is not present,
and torsades de pointes is ar.r unlikely diagnosis.
TEY POIXI
. In adult patients with structural heart disease, 95% of
wide-complex tachycardias are ventricular tachycardia.
Bibliography
Kxtritsis DG, Brugada J. Differential diagnosis ol wide QRS tachycardias.
Arrhythm Electrophysiol Rev 2020;9: 155 160. [PMID: 33240511] doi;10.
15420 /aer.2O2O.2O
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187. Answers and
Item 38 Answer: A
Educational Objective: Treat a patient with heart failure
with aldosterone antagonist therapy
The most appropriate treatment is to add eplerenone (Option A)
to this patient's regimen. He is clinically stable with New
York Heart Association functional class III heart failure
symptoms, and the goal of treatment at this point is to opti
mize medical therapy to improve his chances of maintaining
clinical stability and reducing morlality. Aldosterone antag
onist therapy (spironolactone or eplerenone) is inclicated in
all patients with symptomatic hearl failure with reduced
ejection fraction and normal kidney function (estimated glo
merular filtration rate >30 mL/min/1.73 m2), as these agents
have been shown to improve suruival in this population.
Eplerenone is a more selective aldosterone antagonist than
spironolactone; it is associated with fewer endocrine side
effects and reduced incidence of gynecomastia (17, vs. 10,/,,).
Therefore, although this patient had gynecomastia when
taking spironolactone, it is reasonable to try epierenone as
an alternative aldosterone antagonist.
Ivabradine (Option B) has been shown to reduce heart
failure admissions in patients u,'ith symptomatic heart
failure with reduced ejection fraction (ejection fiaction
<35%) who are in sinus rhythm with a heart rate of at least
TOlmin and taking maximally tolerated doses of a p blocker.
Ivabradine works by slowing the sinus node and decreasing
heart rate. For patients with atrial flbrillation, however, the
drug is not helpful.
Guideline directed medical therapy for heart failure
includes B-blocker therapy for al1 patients, regardless of
heart failure stage. The benefits of p blocker therapy do
not seem to be a class effect, and one of the three agents
shown to have a mortality beneflt (bisoprolol, carvedilol, and
metoprolol succinate) should be used. Changing from can/e
dilol to metoprolol succinate (Option C) might be of benefit
if the patient were nonadherent with a twice daily drug, but
other-wise, there is no demonstrated beneflt from switching
from one to another.
Valsartan sacubitril is an angiotensin receptor neprilysin
inhibitor that improves survival and symptoms in patients
with heart failure with reduced ejection fraction when com
pared with ACE inhibitor therapy Guidelines recommend
replacing an ACE inhibitor or angiotensin receptor blocker
(ARB) with valsartan sacubitril in patients who are tolerating
therapy with these agents, or initiating therapy with valsaftan
sacubitril instead of an ACE inhibitor or ARB for patients
with new onset heart failure. However. valsartan-sacubitril is
contraindicated in patients with a history of angioedema with
either ACE inhibitor or ARB therapy Therefore, this patient
should not be switched to valsadan sacubitril (Option D).
K EY PO I]tT
o Aldosterone antagonist therapy (spironolactone,
eplerenone) is indicated in all patients with sympto
matic heart failure with reduced ejection fraction and
normal kidney function to improve survival.
Bibliography
Murphy Sf lbrahim NE, Ja:n:uzzi JL Jr. Heart failure u,ith recluced ejection
fraction:areview.JAN4A.202O;324:,188 S0.1.IPMTD:32749493]doi:10.1001i
jama.2020.10262
Item 39 Answer: C
Educational Objective: Reduce cardiovascular risk with
smoking cessation.
Smoking cessation counseling and varenicline (Option C) is
the most appropriate management for this patient. lbbacco
use is the leading preventable cause ofdisease, disability, and
death in the United States. Almost one third of cardiovascular
disease deaths are attributable to smoking and exposure to
secondhand smoke. Even low Ievels of smoking increase risks
for acute myocardial infarction; thus, reducing the number of
cigarettes per day does not eliminate risk completely. Smok
ing cessation substantially reduces cardiovascular risk within
2 years, with risk returning to the level of a nonsmoker after
approximately 15 years. Smoking status should be assessed
at every visit, and cessation counseling and pharmacologic
therapy should be oflered to active smokers. Of all the rec
ommended pharmacologic agents approved for smoking
cessation, varenicline is the most ellective monotherapy Com-
bination pharmacologic therapy is probably more eflbctive.
Psychosocial factors, including depression, anger, and
anxiety, are associated with worse cardiovascular outcomes.
Depression has been linked to a higher risk for cardiovas
cular events. Psychosocial stressors also aflect the course of
treatment and adherence to healthy lifestyles after a cardio-
vascular event. Although it is impoftant to detect and treat
these disorders ifpresent, there is no evidence that detection
and treatment (Option A) affect cardiovascular risk itself.
The U.S. Preventive Services Task Force recommends
1ow dose aspirin (Option B) for the primary prevention
of atherosclerotic cardiovascular disease (ASCVD) and col
orectal cancer in adults aged 50 to 59 years with a 10 year
ASCVD risk of 10'% or greater who do not have an increased
risk fbr bleeding, have a life expectancy of at least 10 years,
and are willing to take iow dose aspirin daily for at least
10 years. The American College of Cardiologr/American
Heart Association guideline recommends that aspirin be
used infrequently in the routine primary prevention of
ASCVD because of lack of net beneflt.
The National Diabetes Prevention Progranr found that
in persons at high risk for diabetes, interventions such as
changes in diet, exercise, and weight loss (Option D) of 57,
to 7% reduced the risk for developing diabetes by 5B'X, but did
not reduce CVD events.
KIY POIflTS
. Smoking cessation substantially reduces cardiovascular
risk within 2 years, with risk returning to the level of a
nonsmoker after approximately 15 years.
o Smoking status should be assessed at every visit, and
cessation counseling and pharmacologic therapy should
be offered to active smokers.
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Bibliography
Arnett DK. Blumenthal RS, Albert MA. et al. 2019 ACC/AHA guideline on the
primary prevention of cardiovascular disease: a report of the American
College ofCardiologr/American Heart Association Task Force on Clinical
Pract-ice Guidelines. Circulation. 2019 ; 140:e596 e646. IPMID: 30879355]
doi:l o. I 1 61 /CIR.000000000O000678
Item 4O Answer: B
Educational Objective: Screen for myocardial dysfunc-
tion with echocardiography during trastuzumab therapy.
This patient should undergo surveillance with echocardiog
raphy (Option B). Trastuzumab is an anti-HER2 monoclonal
antibody that decreases the risk for breast cancer recurrence
and breast cancer related death. It may result in revers-
ible cardiotoxicity that manifests as left ventricular systolic
dysfunction, causing symptoms of heart failure in 3'7, to
7'7, of patients. Routine surveillance imaging with echocar-
diography may be offered during treatment in asymptom
atic patients at increased risk for cardiac dysfunction. The
2017 American Society of Clinical Oncologz (ASCO) practice
guideline recommends echocardiography as the surveil-
lance imaging modality of choice. ASCO identifles patients
receiving trastuzumab as having increased risk for cardiac
dysfunction if any of the following are present: two or more
traditional cardiovascular risk factors (smoking, hyperten-
sion, diabetes mellitus, dyslipidemia, and obesity), older
age (>60 years) at cancer treatment, borderline left ventric-
ular ejection fraction (50'1, to ss'z,), history of myocardial
infarction, moderate or greater valvular heart disease, and
treatment combined with lower dose anthracycline. This
patient has three risk factors (hypertension, hyperlipidemia,
and borderline ejection fraction) and should undergo echo-
cardiographic surveillance. ASCO recommends that the fre
quency of cardiac imaging for each patient be determined
by the patient's physician on the basis of clinical judg-
ment and patient circumstances. Common practice includes
a baseline evaluation and repeat imaging at 3, 6, 9, and
12 months after trastuzumab initiation. ASCO suggests that
an echocardiogram be obtained between 6 and 12 months
after completion of cancer directed therapy in asymptomatic
patients considered to be at increased risk.
ASCO recommends echocardiography in patients who
develop heart failure symptoms. Cardiac magnetic reso
nance (CMR) imaging (Option A) or multigated acquisition
(MUGA) scan (Option C) is recommended if echocardi-
ography is not available or has resulted in poor image
qualify, with preference given to CMR imaging. Neither
CMR imaging nor MUGA scan is recommended for routine
surveillance.
Performing no surveillance (Option D) is not in the
best interest of this patient. The incidence of signiflcant left
ventricular ejection fraction decline in trials has ranged from
7.1"1, to 18.6'1,, with a rate of New York Heart Association
functional class III or IV heart failure ranging from 0.4'X, to
4.1"1,. Early intervention in asymptomatic patients, including
cessation of trastuzumab and consideration of cardioprotec
tive medications, may help reduce this risk. The decision to
discontinue trastuzumab is made by the oncologist in con
sultation with a cardiologist.
XEY POITII
. In patients receiving trastuzumab therapy at high risk
for cardiac dysfunction, periodic echocardiographic
surveillance is recommended.
Bibliography
Armenian SH. Lacchetti C. Lenihan D. Prevention and monitoring ofcardiac
d1'sfunction in survivors of adult cancers: American Society of Clinical
Oncolos' clinical practice guideline summary. J Oncol Pract. 20u;13:
270 s. [PMID, 279227 96] doi:10.1 200/JoP.20i6.018770
Item 41 Answer: C
Educational Objective: Manage persistent symptomatic
chronic stable angina.
Coronary angiography (Option C) is the most appropriate
management for this patient who has persistent angina
despite maximally tolerated medical therapy. In this set
ting, invasive imaging for revascularization assessment is
appropriate to improve symptom status and quality of life.
Revascularization targets are identifled on the basis of ana
tomic and functional physiologic characteristics associated
with myocardial ischemia. Techniques such as fractional
flow reserve and instantaneous wave-free ratio provide
information on the functional hemodynamic signiflcance of
indeterminate lesions identifled on angiographic imaging,
reducing both unnecessary stenting and the need for urgent
revascularization. In contrast, in unstable or in many acute
presentations, revascularization is indicated to prevent
future events and improve survival. The risks and beneflts of
and alternatives to angiography should be discussed, along
with potential findings and therapeutic options. Other indi
cations for coronary artery imaging include the presence ol
clinical flndings or indications on noninvasive testing that
are associated with severe ischemic heart disease. Other
patients who may benefit from coronary artery imaging
are those with Ieft ventricular systolic dysfunction and the
presence olischemia and other high-risk criteria on nonin-
vasive testing.
Aspirin reduces the risk for myocardial infarction and
cardiovascular death in patients with stable angina. Guide
lines recommend low dose aspirin (75 162 mg/d) for sec
ondary prevention because it is as effective as high dose
aspirin (SZS mg/d) in preventing myocardial infarction and
confers a lower bleeding risk. In aspirin-intolerant patients,
clopidogrel, a platelet P2Y,, receptor inhibitor, is an accept-
able alternative. There is no role for dual antiplatelet therapy
with aspirin and clopidogrel (Option A) in patients with
chronic stable angina in the absence ofrevascularization.
This patient's current medication regimen has been
optimized. With a resting pulse rate in the range of 55 to
60/min and a well-controlled blood pressure, additional
vasoactive medical therapy, such as isosorbide mononitrate
(Option B), is unlikely to be necessary. In addition, given the
presence of orthostatic symptoms with his current regimen,
I
176

189. he is unlikely to tolerate further uptitration of vasoactive
medications.
Exercise testing, such as exercise echocardiography
(Option D), may provide additional prognostic informa-
tion. However, it cannot identily target lesions that might
be amenable to revascularization to improve this patient's
quality of life.
I(EY POI l{I
r In the setting of persistent symptoms despite maximally
tolerated medical therapy, invasive imaging for revascu-
Iarization assessment is appropriate to improve slmptom
status and quality of life.
Bibliography
Katz D, Gavin MC. Stable ischemic heart disease. Ann Intern Med. 2019;177:
ITCIT-lTC32. IPMID: 313822881 doi:10.7326lAITC201908060
Item 42 Answer: C
Ed u cati o na I O bjective : Perform noninvasive anatomic
imaging to identiS the location of an abdominal aortic
aneurysm.
In this patient with an abdominal aortic aneurysm (AAA)
greater than 5.5 cm in diameter, the most appropriate next
step is CT angiography (CTA) (Option C) to identify the loca-
tion of the AAA (suprarenal, juxtarenal, or infrarenal) and
plan for repair. Because of the high mortality rate associated
with aneurysm rupture, the U.S. Preventive Services Task
Force recommends one time screening with duplex ultra-
sonography in all men aged 65 to 75 years who have smoked
at least 100 cigarettes in their lifetime and selective screening
in men in this age group who have never smoked. The choice
between open surgical repair and endovascular aneurysm
repair (EVAR) is driven by the location of the AAA and
involvement of the renal and mesenteric arteries. Suprarenal
and juxtarenal aneurysms often necessitate open surgical
repair, whereas infrarenal aneurysms often can be treated
with EVAR. Other factors, including patient age, comorbid
conditions, and ability to tolerate open surgical repair, also
determine which procedure should be performed in patients
with an infrarenal AAA.
Although abdominal aortography (Option A) has been
used fbr AAA sizing and location, the advent of CTA and
magnetic resonance angiography has relegated invasive aor-
tography to a second- or third-line option.
There is limited evidence that ACE inhibitors (Option B)
and angiotensin receptor blockers can halt the progression
of AAA growth. However, this patient has an AAA greater
than 5.5 cm in diameter and thus needs aortic repair fol
lowing CTA.
A decision to perform open surgical repair (Option D)
is premature. The most prudent next step is to determine the
anatomic location of the AAA with CIA to plan for surgical
or endovascular repair.
Repeat duplex ultrasonography (Option E) is not indi-
cated in this patient because the maximal diameter of this
Answers and Critiques
patient's aneurysm is greater than 5.5 cm. The risk for rup-
ture is signiflcantly higher once the maximal diameter is
greater than 5.5 cm or if the AAA grows quickly (>0.5 cm/
year); therefore, proceeding with a plan for aortic repair is
warranted.
KIY POIilTS
o The U.S. Preventive Services Task Force recommends
one-time screening with duplex ultrasonography in
all men aged 65 to 75 years who have smoked at least
100 cigarettes in their lifetime and selective screening
in men in this age group who have never smoked.
.
CT angiogfaphy or magnetic resonance angiography is
the imaging procedure of choice to identiflz the exact
location of an abdominal aneurysm and plan opera-
tive repair.
Bibliography
Chaikof EL, Dalman RL, Eskandari MK, et al. The Society for Vascular
Surgery practice guidelines on the care of patients \a.ith an abdominal
aortic aneurysm. J Vasc Surg. 201&67:2 77 .e2. IPMID:29268916] doi:10.
l0l6r'j.jvs.2017.10.044
Item 43 Answer: A
Educational Objective: Assess the severit5r of mitral
stenosis with exercise echocardiography.
The most appropriate management is to perform exercise
echocardiography (Option A). The patient's history (fatigue
and exertional dyspnea) and examination flndings (open
ing snap and diastolic rumble) are consistent with mitral
stenosis, which the echocardiogram conflrms. However,
quantitation of the mitral stenosis reveals a gradient and
valve area consistent with moderate, rather than severe,
mitral stenosis. Severe mitral stenosis is deflned by a mitral
valve area of 1.5 cm2 or less, which usually corresponds to
a mean mitral gradient of more than 5 to 10 mm Hg at a
normal heart rate. This patient's symptoms are predomi
nantly exertional, and decreased diastolic fllling time with
exertion typically results in an increased mitral gradient
and left atrial and pulmonary pressures in mitral stenosis.
In patients with a discrepancy between the clinical and
echocardiographic flndings, exercise echocardiography or
exercise testing during cardiac catheterization should be
pursued to assess the response of the mitral gradient and
pulmonary pressures to an increased heart rate. Exercise
testing is important because percutaneous balloon mitral
commissurotomy is indicated for patients with severe mitral
stenosis and favorable valve morphology.
Although cardiac catheterization (Option B), cardiac
magnetic resonance (CMR) imaging (Option C), or trans-
esophageal echocardiography (Option E) may be used to
measure the transmitral gradient and calculate or measure
mitral valve area, each does so with the patient in a resting
state and thus would not likely demonstrate the heart rate-
related increases in mitral gradient, pulmonary pressures,
and left atrial pressures often seen in mitral stenosis. None of
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these tests would provide evidence to explain this patient's
symptoms.
Conclusively demonstrating the severilz of mitral ste
nosis with exercise echocardiography would be necessary
before recommending an intervention as invasive as percu
taneous balloon mitral commissurotomy (Option D).
XEY POIilI
. In patients with rheumatic mitral stenosis and a dis
crepancy between resting echocardiographic findings
and clinical symptoms, exercise echocardiography or
exercise testing during cardiac catheterization is rec
ommended to further assess the valve.
Bibliography
Otto CM, Nishimura RA, Bonow RO. et al. 2020 ACC/AHA guideline for the
management of patients with valvular heart disease: a report of the
American College of Cardiolog//American Heart Association Joint
Committee on Clinical Practice Guidelines. Circulation. 2021 ;143:e72
e227. IPMID: 333321501 doi:10.1161'CIR.0000000000000923
Item 44 Answer: C
Ed ucationa I Objective: Manage acute bradycardia.
'lhis
patient should receir.e intrar.cnous atropine (Option C).
llc has altered mental status ancl hypotcnsion in the set
ting of profbund bradycardia. According to the advanccd
carcliovtrscular life support algorithnr for bradl,cardirr in
patients lr,ith a pulse. atropine is the most appropriate first
stcp. It may acutely, ir.nprove hexrt rate and perlusion but
r,r'ill likell, require repeat closing if efl'ectir.e. Alternatir,cs
include intravenous doparline or epir-rephrine or trans
cutaneous pacing. rvhich is painful. Ultimatell-. the goal is
to determine the nnderll,ing cxusc of'his acute condition.
'Ihc
brrrd-v-cardia m:r1, be either the cause or the rcsult
ol his decompensation. Pathologic sinus bradl,cardia is
most commonly causecl b1, sinus node dysfunction clue
to age re'lated myocarclial fibrosis. Less comnronh: sinus
nocle dysf unction ma!, result trom right coronary iscl.rernia.
Irypothl'roidism. intracranial hr,pertension. postoperative
scirrring or fibrosis fion.r carcliothrtracic surgery or ir.rfiltra
tive or inflirmmatory disurders (e.g.. sarcoidosis).lhe nrost
common extrinsic cause is mcdication use (B blockers.
doncpezil, neostigmine, pyriclostigrnine). Permanent pirc
i ng nriry' be indicatecl eventual 11:
Amiodarone (Option A) is l rnultichannel antiarrhyth
nric agent used prirnarily to treat vcntricular arrhythmias
ancl atrial fibrillation. It lras no role in the management
ol symptomatic bradycardia ancl is n.r<tre likely to worsen
his conclition through its
B bl<tcking and negative inotropic
properties.
'lhis
patient is profbundlv bradvcardic and hlpoten
sive. but he does har,e a pulse and is arousable. Therefbre.
chcst conrpressions (Option B) arc not currentlv indi
cated. If the pulse beconres no longer palpable, he n,ould
be in pulseless electrical ilctivit), :rrrest. in which case
chest compressions to facilitate svstemic perfusion may
br. lif'e saving.
In patients u'ith sinus rtode dysfhllction associated u ith
s_ynrptoms or hemociyllxnlic contpromise rt'ho are at lou'
likelihood of coronary ischemia, isoproterenol (Option D).
dopamine. dobutamine, or epinephrir.re may be considered
to increase heart rate ancl intprove symptoms. In general.
guidelines relegate chronotropic irgents as second-line ther
apy to atropine in the ilcute resuscitiltion of patients w'ith
hemodl'nanricalll' r-urstable bracll'cirrdia.
XEY POIXIS
. The treatment of hemodynamically unstable sinus
bradycardia in patients with a pulse is intravenous
atropine.
. The most common extrinsic cause of sinus bradycardia
is medication use (p-blockers, donepezil, neostigmine,
pyridostigmine).
Bibliography
Kusul.ltoto FNI. Schoenfeld MH. Barrett c. et al. 2018 ACC AHA HRS guide
line on the e\aluation and mirnagement ofpatients t{ith bradlcardia and
cardiac conduction delay: a report ofthe American College ofCardiobg'
American Heart Association Task Force on Clinical Practice Cuidelines
irnd the Heart Rhythm Society. Circulation.2019:1.10:e382 e.182. [PMIL):
:1O5a677 2l doi:10.1161 /CIR.0000000000000628
Item 45 Answer: C
Educational Objective: Manage uptitration of p-blocker
therapy in a patient with heart failure with reduced ejection
fraction.
The most appropriate management is to increase the dos
age of carvedilol (Option C). This patient has recent-onset
heart failure with reduced ejection fraction (HFTEF) and is
undergoing medication titration. B-Blockers should be ini
tiated in all patients with HFTEF. These agents can improve
remodeling, increase left ventricular ejection fraction, and
reduce hospitalization and mortality when added to ACE
inhibitor and diuretic therapy. In contrast to ACE inhibi
tors, the beneflts ofp blocker therapy do not seem to be a
class effect, and one of three agents shown to have a mor
tality beneflt (bisoprolol, carvedilol, metoprolol succinate)
should be used. p Blockers are generally well tolerated but
should not be started when the patient is acutely decom
pensated. These agents have negative inotropic properties
and may exacerbate heart failure in patients with acute
volume overload. B Blockers should be initiated at low
dosages and uptitrated slowly over weeks (not days) until
the patient achieves the guideline-directed target dos
age or maximum tolerable dosage. The target dosage for
carvedilol is 25 mg twice daily (SO mg twice daily if weight
>85 kg [187 1b]).
Ivabradine has been shown to reduce hospitalizations
in patients with New York Heart Association functional class
III to IV heart failure on maximally tolerated B blocker ther-
apy. This patient's heart rate is elevated; however, she is not
receiving the maximum dosage of carvedilol, and carvedilol
should be increased before considering adding ivabradine
(Option A).
178

191. Answers and Critiques
In patients with symptomatic heart failure, valsartan
sacubitril has been shown to reduce morbidity and mor-
tality compared with enalapril. It will occasionally cause
symptomatic hypotension, a reason to lower the dosage. This
patient has no indication to lower the dosage (Option B).
This patient has no evidence of volume overload on
physical examination (no jugular venous distention or
edema). Therefore, there is no need to increase the dosage of
furosemide (Option D).
rtv Potr{Ts .
o p-Blockers should be initiated in all patients with heart
failure with reduced ejection fraction.
. In patients with heart failure with reduced ejection
fraction,
B-blockers should be initiated at low dosages
and slowly uptitrated over weeks (not days) until the
patient achieves the guideline-directed target dosage
or maximum tolerable dosage.
Bibliography
Yancy CW, Januzzi JL Jr, Allen LA, et al. 2017 ACC expert consensus decision
pathway for optimization ofheart failure treatment: answers to 1O pivotal
issues about heart failure with reduced ejection fraction: a report ofthe
American College ofCardiolopy Task Force on Expert Consensus Decision
Pathways. J Am Coll Cardiol. 2018;71:201 230. IPMID: 29277252] doi:LO.
1016/j.jacc.2017.11.02s
Item 45 Answer: D
Educational Objective: Treat acute limb ischemia with
anticoagulation.
lhe most appropriate next step in management is to admin.
ister intra\enous unfiactionated heparin (Option D). Clas-
sically, patients nith acllte limb ischemia present with at
least one of the six P's: paresthesia, pain, pallor, pulseless-
ness, poikilothermia (coolness), and paralysis. Acute limb
iscl-ren.ria is most commonly caused by acute thrombosis
of a lower extremity artery stent, or bypass graft. Other
causes include thromboembolism, vessel dissection (usually
occurring periprocedurally), or trar-lma. This patient's pre
sentation is consistent with acute lirr,b ischemia ciue to atrial
fibrillation related thromboembolism, and the most appro'
priate first steps are to (l) initiate intrar,enous anticoagula
tion with unfractionated heparin, (2) perforrn angiography,
and (3) establish a plan for reperfusion ofrhe leg.
Invasive angiography of the lower extremity (Option A)
should be expedited, as endovzrscular or surgical rev:rs.
cularization is warr:rnted to preserve limb Iunction and
prevent major amputation. llow'ever. anticoagulation,
typically r,l'ith unfraction:ited heparin, should be initiated
as soon as the diagnosis is suspected, beliire perfbrming
diagnostic testing.
This patient, r,r,ho has atrial fibrillation, needs antico
agulation fbllorving this e'rent. However, because interuen
tion is needeci to restore blood flovrl unf'racticlnated heparin
should be initiated no r, not an oral irnticoagulant snch as
apixaban (Option B). Unfractionated heparin has a rapid
onset and offset of action, alkru''ing for more flexibility in
dose titration or discontinuation when needed for inr,asive
procedures.
Intravenous systemic thrombolysis, such as with tenec
teplase (Option C), has not been provecl efl'ective in most
patients with acute limb ischemi:i. lJor,r,ever, catheter based
thrombolvsis is elfective for patients with acute limb
ischemia and a salvageable limb.
Venous duplex ultrasonography (Option E) is rTot incli
catecl in this patient r,r,,ho has no evidence of massive venous
thron-rbosis. Iliof'emoral \enous thrombosis is a rare cause
oi acute limb ischemia and should be suspected in patients
r,vith sudden severe pain, sr,velling. cyanosis, and edenra.
These findings are not present in this patient.
XEY POIlIIS
. Classically, patients with acute limb ischemia present
with at least one of the six P's: paresthesia, pain, pal-
1or, pulselessness, poikilothermia (coolness), and
paralysis.
o Patients with acute limb ischemia should receive
emergent systemic anticoagulation and diagnostic
angiography in preparation for revascularization.
Bibliography
Gerhard-Herman MD, Gornik HL, Barrett C, et a]. 2016 AHA/ACC guideline
on the management of patients with lower extremity peripheral artery
disease: executive summary: a report of the American College of
Cardiologr/American Heart Association Task Force on Clinical Practice
Guidelines. Circulation. 2017;135:e686 e725. [PMID: 27840332] doi:10.
1161 /CIR.0000000000000470
Item 47 Answer: C
Ed ucationa I Objective: Diagnose peripartum sponta
neous coronary artery dissection.
The most likely cliagnosis is spontaneous coronary artery
dissection (SCAD) (Option C). SCAD is the most common
cause ol pregnancy associated myocardial infirrction iind
commonly occurs during pregnancy or in the first month
postpartum. SCAD involves development of a nontraumatic
and non iatrogenic intramural hematoma with or r,rrithout
intimal dissection with luminal conrmunication. the enlarg
ing hematoma in the false lumen compresses the true lumen
of the coronary artery and in potential combination rvith
obstructing dissection leads to chest pain, ischemia. and/or
infarction. the diagnosis requires coronary C'f or coronary
angiography to confirm the characteristic imaging features.
When associated r,r,ith ST-elerzation myocardial infarction,
SCAD rnay be managed inrasivelyr however, percutaneous
coronary intervention may be safely detbrred r.then coro-
nary flow is preserved and symptoms can be controlled and
closely monitored.
'ITe
pathogenesis of ST elevation myocardial infarction
typically ir.r,,,olves plaque rupture (Option A) within a coro
nary artery
'lhe
rupture causes platelet adhesion, activation,
irnd aggrcgation, resultiug in a thrombosed coronary artery
irnd acute vessel occlnsion.
'lhe
sudden loss of coronary
blood flort, leacis to transmural ischenria of the nryocardiunt
tr
EI
UI
(u
CT
tJ
t,
IE
UI
o
=
tt
179

192. Answers and Critiques
UI
=
.D
art
q,
EL
n
ll
o
ta
tr
c0NI.
tr
and the ECG manifestation of ST segment eleration. Acute
coronary thrombosis is a less likely cause of m1'ocardial
infarction than coronary artery dissection in this postpar
tum young woman with no atherosclerotic cardiovascular
disease risk factors.
This patient's clinical picture is inconsistent
"t,ith
peripartum cardiomyopathy (Option B), which is r-rert'ly
noted left ventricular systolic dysfunction with onset in the
rnonths after delivery or toward the end of pregnancy in the
absence ofanother identifiable cause. Patients nith peripar
tum cardiomyopathy usually present
'"t,ith
features of heart
tailure. Echocardiography in peripartum cardioml,opathy
usually demonstrates gtobal reduction in systolic function.
not tbcal hypokinesis, as seen in this patient.
Stress-induced (takotsubo) cardiomyopathy (Option D)
is characterized by transient regional cardiac dysfunction.
usually involving the apical and mid portions of the left ven
tricle. This is commonly precipitated by a stressful physical
or emotional event. PostpartLlm cases ofstress-induced car
diomyopathy have been reported. especially after cesarean
delivery. Patients rn,ith stress induced cardiomyopathy pre
sent with features that mimic an acute coronary syndrome.
this patient's clinical picture is inconsistent with stress
induced cardiomyopathy given the regional r.tall hypokine
sis, which is more typical of coronary artery ischemia.
I(EY POI TI
. Spontaneous coronary artery dissection is the most
common cause of pregnancy-associated myocardial
infarction and occurs most commonly in the first
month postparhrm.
Bibliography
Tweet MS, Hayes SN, Codsi E, et al. Spontaneous coronary artery dissection
associated with pregnancy. J Am Coll Cardiol.2OtTJo:426-435. [PMID:
287 28686) doi:10. 1016 /j. jacc. 2O17. 05.055
Item 48 Answer: B
Educational Objective: Treat patent foramen ovale in a
patient with embolic stroke of undetermined source.
The rnost appropriate management is patent foramen ovale
(PFO) device closure (Option B). The fbramen ovale. a pas
sage in the superior portion of the fbssa ovalis that allows
oxygenated placental blood to transfer to the fetal circulation,
remains patent in 25"1,Io 30'/,, of the population. Patients are
usually asymptomatic. PFO has been identified at increased
rates in patients with embolic stroke of undetermined
source, and several randomized studies have demonstrated
that percutaneous PFO closure is more efi'ective for prevent
ing recurrent ischemic stroke than antiplatelet therapy for
patients younger than 60 years who have an embolic stroke
of'undetermined source with PFO. Device closure decreases
the risk for recurrent stroke by approximately 60',?, com
pared with medical therapy. Current guidelines recommend
PFC) closure in this population fbllowing a discussion of
potential heneflts (absolute recurrent stroke risk reduction
of 3.4')i, at 5 )€ars) and risks (periprocedural complication
rate of 3.9'),, and increased absolute rate of non periproce
dural atrial fibrillation of 0.113'/, per year). Data regarding
benefit of PFO closure in patients older than 60 years are
lacking. Honever, current guidelines indicate that PFO clo-
sure may be considered in patients aged 60 to 65 years u'ith
feu' traditional vascular risk factors lor stroke and no other
mechanism of stroke detected after a thorough evaluation.
including prolonged monitoring fbr atrial fibrillation.
ung patients witl.r a PFO and embolic stroke of unde
termined source should be treated u'ith PFO closure in
addition to antipiatelet therapy: Aspirin alone (Option A)
is generally recommended for patients u'ho are older than
60 years or patients who decline PFO closure.
In patients who opt to receive n.redical therapy alone
without PF-O closure. either an antiplatelet medicatiort. such
as aspirin. or anticoagulation (using a vitamin K antago
nist [Option C], a direct thrombin inl.ribitor. or a factor Xa
inhibitor) seems to be equally efl'ective in reducing the risk
for stroke by about 30')1,, but confidence inten'als are u'ide.
leading to uncertainty of benefit. In patients $'ith a history of
venous thromboembolism or hypercoagulable state. expert
opinion endorses anticoagulation.
Observation alone (Option D) has been associated lvith
I.righer event rates in patients u,ith PFO and stroke. There
fbre, it is not appropriate to simply observe u'ithout therapl:
TEY POIilTS
. In patients younger than 60 years with a patent fora-
men ovale (PFO) and embolic stroke of undetermined
source, current guidelines recommend PFO closure
following a discussion of potential benefits and risks.
. In patients 60 years or older, patent foramen ovale
closure may be considered for those who have few
traditional risk factors for stroke and no other mecha-
nism ofstroke detected, although the benefit is more
uncertain than in younger patients.
Bibliography
Messd SR, Gronseth GS, Kent DM, et al. Practice advisory update summary:
patent foramen ovale and secondary stroke prevention: report of the
Guideline Subcommittee of the American Academy of Neurolog,:
Neurologr. 202O:94:876 85. IPMID: 32350058] doi:10.1212 WNL.
0000000000009443
Item 49 Answer: E
Educational Obiective: Evaluate a cardiac murmur with
transthoracic echocardiography.
The most appropriate diagnostic test is transthoracic echo
cardiography (fff) (Option E) in this patient with physical
examination flndings suggestive of structural heart disease,
speciflcally aortic regurgitation. T'TE is the mainstay of non-
invasive imaging to detect structural heart abnormalities
and to evaluate new or worsening murmurs. This patient's
murmur suggests aortic regurgitation, which should ini-
tially be evaluated with TTE. TTE also allows for evaluation
180

193. Answers and Critiques
of hemodynamic and functional consequences of valvular
heart disease, which will assist in managing this patient.
In patients with moderate or severe aortic regurgita-
tion and suboptimal TTE images or a discrepancy between
clinical and TTE flndings, transesophageal echocardiogra
phy (TEE), cardiac catheterization (Option A), or cardiac
magnetic resonance imaging (Option B) is indicated for the
assessment of left ventricular systolic function, systolic and
diastolic volumes, aortic size, and aortic regurgitation sever
ity. However, before consideration ofany ofthese tests, initial
evaluation with TTE should be performed.
In patients with suspected low-flow low gradient severe
aortic stenosis with reduced left ventricular ejection frac
tion, low-dose dobutamine stress testing with echocardio-
graphic (Option C) or invasive hemodynamic measurements
is reasonable to further deflne severity and assess contractile
reserve. However, this patient's auscultation flndings are most
consistent with aortic regurgitation. Even if this patient had
aortic stenosis, the initial diagnostic test remains TTE.
TEE (Option D) is not the most appropriate next step
for this patient because it is unnecessarily invasive at this
point in her evaluation. TEE is usually performed if TTE
image quality is iow; it also can be particularly helpful if
an evaluation of left atrial appendage thrombus or a cardi
oembolic source is indicated or when there is suspicion of
endocarditis, prosthetic valve dysfunction, or aortic disease.
TEE is also useful in patients with moderate or severe aortic
regurgitation and suboptimal TTE images or a discrepancy
between clinical and TTE lindings.
I(IY POI ]II
. Transthoracic echocardiography is the mainstay of
noninvasive imaging to detect structural heart abnor
ffi,J*"ff*,o
evaluate new or worsening cardiac
Bibliography
otto cM, Nishimura RA, Bonon, RO. et al. 2020 ACC/AHA guideline for the
management of patients with valvular heart disease: a report of the
American College of Cardiolory/American Ileart Association Joint
Committee on Clinical Practice Guidelines. Circulation. 2021;143:
e72 e227. IPMID: 33332150] doi:10.1161 /CIR.0000000000000923
ttem 50 Answer: D
Ed u cati o n a I O bj ective : Treat hypertrophic cardiomy-
opathy with obstructive symptoms with septal reduction
therapy.
Septal reduction therapy (SRT) (Option D) is the most appro
priate treatment. Pharmacotherapy and lifestyle modification
are appropriate initial choices for treatment of hypertrophic
cardiomyopathy (HCM) with obstructive symptoms. Non
vasodilating p blockers and/or nondihydropyridine calcium
channel blockers (verapamil, diltiazem) are flrst-line choices.
For patients with persistent symptoms, adding disopyramide,
a class IA antiarrhythmic drug with potent negative ino
tropic activity, to one of the other drugs is a recommended
option. Patients receiving guideline-directed medical therapy
but with New York Heart Association functional class III to
IV heart failure symptoms or recurrent syncope believed to
be related to left ventricular outflow tract (LVOT) obstruction
and an LVOT gradient of 50 mm Hg (resting or provoked) or
greater should be considered for SRT. Adult patients in whom
surgical septal myectomy is contraindicated or the risk is
considered unacceptable because of serious comorbidities
or advanced age, alcohol septal ablation, performed at expe-
rienced centers, is recommended. This patient is young and
without significant comorbidities; thus, he should be consid
ered for surgical septal myectomy.
Both valsartan-sacubitril and carvedilol may reduce
afterload. Adding valsartan sacubitril (Option A) or switch
ing metoprolol to carvedilol (Option C) may worsen this
patient's symptoms and degree of LVOT obstruction.
This patient has no clear indication for implantable
cardioverter deflbrillator (lCD) placement (Option B). An
ICD is recommended for patients with HCM and previous
documented cardiac arrest or sustained ventricular tachycar-
dia. ICD placement is considered reasonable in the presence
of one or more major risk factors for sudden cardiac death,
including sudden death in a flrst degree or close relative at
age 50 years or younger, lelt ventricular (LV) hypertrophy of
30 mm or greater, syncope thought to be arrhythmogenic, LV
aneurysm, and an LV ejection fraction of 50% or less.
I(EY POII{I
o Patients with hypertrophic cardiomyopathy who are
receiving guideline-directed medical therapy but with
New York Heart Association functional class III to IV
heart failure symptoms or recurrent syncope believed
to be related to left ventricular outflow tract (LVOT)
obstruction and an LVOT gradient of 50 mm Hg (rest
ing or provoked) or greater should be considered lor
septal reduction therapY.
Bibliography
Ommen SR, Mital S, Burke MA, et al. 2020 AHA/ACC guideline for the diag-
nosis and treatment of patients with hypertrophic cardiomyopathy: exec
utive summary: a report of the American College of Cardiologl/American
Heart Association Joint Committee on Clinical Practice Guidelines.
Circulation. 2020:142:e533 e557. IPMID: 33215938] doi:10.1161/CIR.
0000000000000938
Item 51 Answer: C
Educational Objective: Treat hemodynamic complica-
tions of inferior myocardial infarction with right ventricu
lar involvement.
Intravenous
(t.9')1, saline (Option C) is indicated firr this
patierlt lvith iut inferior rnyocarclial infarctiot.t (MI) compli
cated tr1' right r,entricular (RV) inf'arction. RV infarction is
rarely nn isolirted cvent: it rlccurs in as nlan!'JS olle third
ol paticnts with an inf'erior Ir4l and is nrost comnlonly asso
ciltecl r,r,ith proxitrtll rigl'tt coronary artery cvents. Acutc
resuscil:rtiotr of patients with RV ischenlic dysiunction is
irinrecl itt reversing tlre resultant clecrease in lelt ventricular
(tV) preloacl itnd sr-rbseqr'rent recluctiorl in cardiac output.
1
tr
vt
q,
.g
L'
=,
t!
UI
o
=
ta
E
181

194. Answers and Critiques
aa
€
.D
UI
q,
a
rl
lt
(D
UI
:
ffi Prompt reperfusion follor.ted by intravenous volume expan-
El s16n with up to sereral liters of intrarenous iluirls increases
coNT
right ventricular preload and contractility. tl-rus increasing
ftow through the pulmonary circuit into the lell atrium
and ultimately enhancing LV preload and carcliac output.
Although long-term prognosis is good r,r,hen appropriale
treatment is initiated promptly, RV infarction is associated
with high inpatient morbidity and morlality. Early recog
nition is therefore essential and may be facilitated by iden
tiflcation of 1 mm or more of ST segment elevation in lead
V, or V.,R (right sided V). Findings of normal or modestly
elevated central venous pressure r.r,ith clear lungs despite
tachycardia and/or hypotension are suggestive of an RV
infarction complicating infbrior ST-elevation MI: honever,
these findings also may be seen in cardiac tamponade, ancl
conflrmation of RV infarction physiolog, and absence of
pericardial effusion by echocardiography is indicated.
Support with intra'aortic balloon pump placement
(Option A) or other mechanical IV supportive measllres shoulcl
be reserved fbr stabilization in the setting ofpersistent shock,
especially with evidence of concomitant LV systolic failure.
When volume resuscitation does not improve signs of
cardiogenic shock associated r.tith RV infarction or when
intravenous fluids result in development ol pulmonary
edema, inotropic therapy may be necessary. ln this patient
r.tith tachycardia on presentation. clear lungs. and stable
hypotension. dobutamine infusion (Option B) may be asso
ciated with worsening tachycardia and further l.remody-
namic deterioration. Inotropic therapy should be resenred
fbr iailure of volume erpansion.
Nitrates, diuretics, opioid analgesic agents. calcium
channel blockers, and B blockers (metoprolol) (Option D)
can aclversely contribute to reduced cardiac preload and
cardiac output and should be avoided in patients rvith acute
RV infarction physiologr.
'
trl.Y. rPOlt.f]:ir
r. ri,
. A patient with an inferior myocardial infarction with
right ventricular involvement and hypotension should
receive intravenous fl uids.
Bibliography
Ibanez B, James S, Agewall S, et al; ESC Scientific Document Group. 2017
ESC guidelines for the management of acute myocardial infarction in
patients presenting with ST segment elevation: the Task Force for the
Management ofAcute Myocardial Infarction in
patients presenting
With
ST Segment Elevation of the European Society of Cardiologz (ESC). Eur
Heart J. 2018;39:1 19 77 . lPMlDt 288866211 doi:10.1 093/eurheartj/ehx393
Item 52 Answer: C
Ed ucatio n a I O bj ective : Prevent healt failure readmission
with early follow-up.
A telephone call within 2 to 3 days (Option C) is recom
mended to prevent this patient's early readmission to the
hospital. The 2019 American College of Cardiolog; expert
consensus decision pathway fbr patients hospitalizecl lvith
heart failure notes that up to 25')l, of patients are readrnitted
with heafi failure r,r'ithin 30 days oi the index hospitaliza
tion. Tr,r,o key elements are associated r,rrith a successful tran
sition fiorn hospital to home: a follor,r, r-rp phone call u,ithin
2 to l3 days of discharge and an oflice visit $'ithin 7 to 1,1 days
ol'hospital discharge. The pttrpose of the fbllor,l'-up phone
call is to address signs ofcongestion. provide education and
review adherence to the meclication regimen, and confirm
fbllor,", up appointments and adequate transportation. The
expert consensus decision pathr,r,ay recommends a stan
clardized approach to the fbllou, up telephone call, including
use ofa checklist to help organize the call.
For patients with an initial dingnosis ol heart firilure. it is
allpropriate to repeat echocardiography in 3 rnonths (Option A)
1r; zrssess the eflect ot medical therapy on ejectior-r lraction
and need for an implantable cardioverter defibrillator (lCl)).
lichocardiographl,was perlbrmed in this patient in the hos
pital, and fbr a patient lvith kno.,vn lou' ejection 1'raction
and an lCD, there is l1o reason for echocardiography so soon
unless there is a clinical change.
1he first postdischarge appointment lbcuses on changes
in clinical status, patient education. medication rer,ierv and
acljustn.rent of dosages, and iclentification and correctioll
of issues that might leaci to rvorsening of heirrt lailure and
readmission.
'Ihe
recommended tinring ol the first follort up
visit is u,ithin 7 to 10 days of hospital discharge: 30 days
(Option B) is too 1:rte.
lnadequate diuretic dosage is a common cause of heart
failure readmissions. This patieltt required an increased furo-
semide dosage to achier,e adequate diuresis.
'lhis
suglests
that the prerrious home diur-etic dosage uus inadequate, and
an increase of at least double that dosage should be consid
ered. Restarling the previous home dosage (Option D) might
be considered fbr a patient w'ho did not adhere to the medi
c:rtion regimen befbre hospitalization.
XEY POIIII
r Two key elements are associated with a successful
transition to home following hospitalization for heart
failure: a follow-up phone call within 2 to 3 days of
discharge and an office visit within 7 to 14 days of
hospital discharge.
Bibliography
Hollenberg SM, Warner Stevenson L, Ahmad T, et al. 2019 ACC expert consen-
sus decisionpathway on risk assessment, management, and clinical trajec
tory ol patients hospitalized with heart failure: a report of the American
College of Cardiolos/ Solution Set Oversight Committee. J Am Coll Cardiol.
2019;74:7966 20ll- IPMID: 31526538] doi:10.1016/j.jacc.2019.08.001
Item 53 Answer: A
Educational Objective: Diagnose severe aortic stenosis
in a patient with discrepant clinical and echocardiographic
flndings.
The most appropriate next step in management is to perform
cardiac catheterization (Option A). This patient's symptoms of
dyspnea and syncope are consistent with qrmptomatic, poten
tially severe aortic stenosis. When caused by aortic stenosis,
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195. syncope is usually a late finding and raises concern for sud
den cardiac death if aortic stenosis is not adequately treated.
Severe aortic stenosis is typically defined by a small valve area
(<t.0 cm2), high peak velocity (>a m/s), and/or high mean gra
dient (>40 mm Hg). However, althclugh the two-dimensional
morphologic description of this patient's aortic valve is consis
tent with severe aortic stenosis (severely thickened, minimally
mobile tricuspid aortic valve), the mean valve gradient and
aortic valve area are consistent with moderate aortic stenosis.
Because technicai considerations may result in either over or
underestimation of aortic valve gradient and aortic valve area
by echocardiography, further hemodynamic testing with car
diac catheterization should be pursued in cases ofdiscrepant
clinicai and echocardiographic fi ndings.
CT of the aortic valve (Option B) is a useful diagnostic
modality fbr severe aortic stenosis in low flow iow gradient
disease with normal or reduced cardiac output. However, the
issue at present is discrepant clinical and echocardiographic
flndings. A study that can provide hemodynamic data, such
as cardiac catheterization, is pref'erred.
Exercise stress testing (Option C) is not appropriate
fbr this patient. Exercise stress testing is contraindicated
in most patients with symptomatic severe aortic stenosis,
given the increased risk lbr sudden cardiac death during the
test. A symptom limited treadmill test may be perfbrmed in
asymptomatic patients with severe aortic stenosis to conflrm
asymptomatic status.
Neither surgical aortic valve replacement (Option D)
nor transcatheter aortic valve implantation (Option E) is
appropriate until hemodynamic confirmation of severe
aortic stenosis is made.
KTY POIl{I
o Guidelines recommend cardiac catheterization to
evaluate patients with suspected significant aortic ste
nosis when there is a discrepancy between the clinical
evaluation and the echocardiogram.
Bibliography
Otto CM. Nishimura RA. Bonou' RO. et al. 2020 ACC AHA guideline fbr the
nranagement of patients u'ith lalvuhr herrt disease: a report of the
r'nerican College of CarclioloS''American Ileart Association Joint
Comnrittee on Clinical Practice (lui(lelines. Circulation. 2o2l:143:e7')
e227. IPl\'llDr 33332150] doi: 10. I l6l Cl R.0000000000000923
Item 54 Answer: A
Educational Objective: Treat a patient with peripheral
artery disease with statin therapy.
Tl.re most appropriate treatment is to reintroduce atorvasta
tin (Option A). Peripheral artery disease (PAD) is considered
ir clinical atherosclerotic cardiovascular disease (ASCVD),
ancl high intensity statin therapy (atorvastatin or rosuva
statin) is recommended in patients with clinical ASCVD
tbr secondary prevention, accordir.rg to guidelines from the
American Heart Association/American College of Cardiolory
(AF{A/ACC). Patients with PAD who are older than 75 years
or intolerant of high intensity statins should be treated with
Answers and Critiques
moderate intensity statin therapy. Guidelines from the U.S.
Department of Veterans AIIairs/U.S. Department of Delbnse
recommend at least moderate intensity statin therapy for
the secondary prevention of ASCVD. As many as 45'X, ol
patients with PAD are not prescribed or do not flll a prescrip
tion for statin medications. Patients with PAD may confuse
symptoms of intermittent claudication with adverse efI'ects
of statins (namely myalgia), which may contribute to the low
adherence rate. The most appropriate treatment is patient
education and reintroduction of atorvastatin.
Colestipol (Option B) is a bile acid sequestrant. The
AHATACC cholesterol guideline suggests consideration of
a bile acid sequestrant in patients aged 20 to 75 years with
a baseline LDL cholesterol level o1'190 mg/dl or greater
(>4.9 mmol/L) who achieve less than a 50'2, reduction in LDL
cholesterol and have a fasting triglyceride level of 300 mg/dL
or less (<3.4 mmol/L) while receiving maximally tolerated
statin and ezetimibe therapy. Monotherapy with a bile acid
sequestrant is not recommended as a substitute for statin
therapy in the secondary prevention of ASCVD.
Ezetimibe (Option C) is a cholesterol absorption inhibi
tor. the AHAi'ACC cholesterol guideline suggests considering
the addition of ezetimibe to high intensity statin therapy in
patients with clinical ASCVD whose LDL cholesterol level
remains 70 mgldL (1.8 mmol/L) or greater on maximally
tolerated statin therapy. Monritherapy with ezetimibe is not
a substitute fbr statin therapy.
Icosapent ethyl (Option D), a highly purilied eicosa
pentaenoic acid ethyl ester, lowers triglyceride levels. In
patients with ASCVD or other cardiac risk I'actors on a statin
with controlled LDL cholesterol but elevated triglyceride
levels (135 499 mg/dl [t.sz s.os mmol/L]), the addition of
icosapent ethyl may be considered to reduce cardiovascular
risk. Therapy with icosapent ethyl monotherapy is not rec
ommended for the secondary prevention of ASCVD and is
not a substitute Ibr statin therapy.
t(Ev P0ltTs
. Peripheral artery disease is considered a clinical athero
sclerotic cardiovascular disease.
o Statin therapy is recommended for secondary preven
tion of atherosclerotic cardiovascular disease in patients
with peripheral artery disease.
Bibliography
(;rundy
Sl\4, Stone NJ, Bailey At.. et rl. 20l8 AIIA/ACC/AACVPRIAAPA/AB(l/
ACPM/ADAIAGSlAPhA/ASPC/NLA/l'CNA
guideline on the management
of blood cholesterol: a report ol the American College ol Cardioloryr
American Heart Association'lirsk Iirrce on Clinical Practice Cuidelines'
Circulation. 2019;139:e1082 ell'1i:1. IPMID: :105867741 doi:10'1161 /ClR'
0000000000000625
Item 55 Answer: C
Educational Objective: Treat symptomatic premature
ventricular contractions with a p-blocker.
'lhe
most appropriate treatment is propranolol (Option
C). This patient has symptomatic premature ventricular
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Answers and Critiques
contractions (PVCs) with a normal heart on echocardio
gram. Laboratory studies have excluded common second-
ary causes of PVCs (anemia, hyperthyroidism, pregnancy).
The PVCs are likely contributing to her anxiety, which may
in turn increase their number. p Blockers, particularly
those with some anxiolytic effect (such as propranolol), are
first-line therapy for PVCs. B-Blockers are also preferred in
patients with ventricular dysfunction. Among patients with
depression or very poor tolerance of B-blockers,
a nondihy
dropyridine calcium channel blocker, such as verapamil,
may be used.
This patient's PVC burden may be quite high if she
has several PVCs on a standard l2-lead ECG. Ambula-
tory ECG monitoring can help clarify whether her PVCs
are episodic and of low overall burden or if they persist
throughout the day. Patients with PVCs that account for
more than l0% to 15% of beats may be at risk for develop-
ing PVC induced cardiomyopathy and subsequent heart
failure, which is an additional reason to treat this patient's
symptomatic PVCs. If she develops cardiomyopathy, it may
be reversible with more aggressive treatment of PVCs (e.g.,
catheter ablation).
Amiodarone (Option A) is an antiarrhythmic agent that
may be very effective at suppressing this patient's PVCs.
However, it may be toxic over the long term, potentially
causing devastating injury to the lungs, liver, and/or thyroid.
Furthermore, it may be unnecessary in this young patient.
Amiodarone is therefore not an appropriate flrst-line choice
in this clinical setting. When amiodarone is used, it should
be taken for the shortest possible time at the lowest effective
dose, with close monitoring.
This patient has no exertional symptoms, no speciflc
risk factors for coronary ischemia, and normal echocar
diogram results. Exercise ECG (Option B) is unlikely to be
high yield in this setting. However, PVCs often cease during
exercise in a structurally normal heart, which would be
reassuring.
This patient's symptoms are likely attributable to
pVCs
and are described as intolerable; therefore, she warrants
treatment, and reassurance alone (Option D) is not appro
priate.
rEY POIilTS
o First-line treatment for q.rrnptomatic premature ven-
tricular contraction suppression is p-blocker or calcium
channel blocker therapy; p-blockers are preferred in
patients with ventricular dysfunction.
o Ambulatory ECG monitoring can help clariflz the bur-
den of premature ventricular contractions (pVCs);
patients with PVCs that account for more than 10%
to 15% of beats may be at risk for pVC
induced
cardiomyopathy.
Item 56 Answer: A
Educational Objective: Treat atrial septal defect.
Atrial septal defect (ASD) closure (Option A) is indicated in this
patient with a 1.5 cm ASD associated with right heart enlarge-
ment, symptoms, and no pulmonary hypertension. An ASD is
a defect in the atrial septum resulting in a left-to-right shunt
with eventual right sided cardiac chamber dilatation in most
patients. Ostium secundum defects, the most common ty'pe of
ASD (757, of cases), are typically located in the mid portion of
the atrial septum and are usually isolated anomalies. In asymp-
tomatic patients with a small ASD and no right heart enlarge-
ment, periodic clinical monitoring and echocardiographic
imagrng are recornnended. The main indications for ASD clo-
sure include right sided cardiac chamber enlargement, dys-
pnea, or paradoxical embolization. Most patients with ostium
secundum ASD are candidates for device closure ofthe ASD. a
procedure performed in the cardiac catheterization laboratory.
Cardiopulmonary exercise testing (Option B) may be
helpful in evaluating dyspnea if the initial evaluation is unre-
vealing, deconditioning is a possibility. or several problems
may be contributing to dyspnea. This patient's dyspnea and
right heart enlargement are caused by the ASD. and cardio-
pulmonary testing is not necessary.
Coronary angiography (Option C) is considered before
cardiac surgery or intervention for noncoronary cardiac
issues in patients with symptoms of angina, evidence of cor
onary ischemia, decreased left ventricular systolic function,
a history ol coronary artery disease, or risk factors for coro
nary artery disease. This patient's symptoms and evaluation
are consistent with ASD, not cardiac ischemia, and coronary
angiography is not required.
Echocardiographic surveillance (Option D) is considered
for patients with an ASD if there is no associated right heart
enlargement, if severe pulmonary hypertension is present, or
if there are no severe comorbid conditions that may adversely
affect outcome. Transesophageal echocardiography may be
needed to conflrm the presence of an ASD if transthoracic
echocardiography is nondiagnostic or for the purposes of
procedure planning. ASD closure, not serial surveillance with
echocardiography, is recommended for this patient with a
symptomatic ASD and right heart enlargement.
TEY POIXTT
o Atrial septal defect closure is indicated in patients
with symptoms or evidence of right-sided cardiac
chamber enlargement and without severe pulmonary
hypertension.
o Most patients with ostium secundum atrial septal defect
are candidates for device closure performed in the cardiac
catheterization laboratory.
Bibliography
Stout KK. Daniels CJ. Aboulhosn JA. er al. 2018 AHA,ACC guideline for the
management of adults w.ith congenital heart disease; a report of the
American College of Cardiologr/American Heart Association Task Force
on Clinical Practice Guidelines. J Am Coll Cardiol. 2O79:23:e81-e192.
IPMID: 30121239] doi:10.1016/j.jacc.2018.08.1029
Bibliography
Marcus GM. Evaluation and management of premature ventricular com
plexes. Circulation. 2020;141 :1404 18. [PMID: 32339046] doi:10.1161 /
CIRCULATIONAH A.179.042434
184

197. Answers and Critiques
tr
Item 57 Answer: D
Educational Objective: Evaluate chest pain using
vasodilator single-photon emission CT.
Vasodilator single photon emission CT (Option D) using per
fusion witl.r adcnosine, regadenoson, or dipyridamole is the
most appropriate and pref'erred stress testing option for this
patient with lcfl bundle branch block (LBBB). In patients with
LBBB undergoing my'ocardial perfusion imaging, vasodilator
stress minimizes septal abnormalities frequently seen with
exercise and dobutamine, which are associated with higher
heart rates. Although this paticnt can exercise, the presence
of LBBB lirnits interpretation fbr heart rate dependent stress
modalities.
Dobutamine echocardiography (Option A) is not recom-
mended fbr this patient because ol the presence of LBtsB, which
may limit echocardiographic image interpretation. Other sce
narios in which dobutamine echocardiography is not an opti-
mal stress option include resting wall motion abnormalities
and contrainclications to using dobutamine. Contraindications
to dobutamine stress ech<lcardiography include ircute coronary
syndromes; severe aortic stenosisr hypertrophic obstrrrctive
cardiomyopathy; and uncontrollecl hyperterrsion, urrhyhmias,
or heart failure. Dobutamine echocardiography is recom-
mended in patients who cannot exercise or when infbnnation
on an area of myocardium at risk is needed.
Exercisc ECG (Option B) is usually the preferred stress
test lbr patients unless there are compellirrg reasons to add
imaging to the stress test or if'the patient cannot exercise.
The compelling indication to avoid exercise ECG in this
patient is thc presence of LtsBB. LBBB renders the exercise
ECG nearly impossibie to appropriately interpret unless the
patient devclops the equivalent of'an ST segment elevation
myocardial intarction during the test.
Exercise single photon ernission CT (Option C) is not
the most appropriate stress modality fbr this patient because
ol the prcsence of LBBB. Inraging modalities that require
heart rate increases, such as exercise, are not rccommended
for patients with LBBB because the abnormal septal wall
motion may limit interpretation. With the exception of
LBBB, exercise single photon ernission CT is recommended
when baselinc ECC flndings are abnormal or whcn infbrma
tion on a particular area ot myocardium at risk is needed.
With LBBB, c<-rnduction delay in the septunl may cause
false positive abnormalities: vasodilator stress can improve
the accurlcy oI perlusion itnlgittg.
IEY POITI
o In the evaluation of patients with chest pain, vasodila-
tor myocardial perfusion imaging with adenosine,
regadenoson, or dipyridamole minimizes septal
abnormalities frequently seen with exercise or dobu-
tamine myocardial perfusion imaging.
Bibliography
Balfour PC Jr, Conzalez JA, Kramer CM. Non-invasive assessment of low-
and intermediate risk patients with chest pain. Trends Cardiovasc Med.
2017 :27 :182 189. IPMID: 27717538] doi:10.1016/i.tcm.2016.08.006
Item 58 Answer: C
Educational Objective: Diagnose constrictive
pericarditis.
The diagnostic findings are consistent with constrictive
pericarditis (Option C), which typically presents with
ir.rdolent, progressive signs and symptoms of righl l.reart
failure, including latigue and excrtional dyspnea. On
physical examination, the central venous pressure is ele
vated in nearly all patients, with prominent x and y
descents. The height of'the wavefbrm does not fall or may
increase during inspiration (Kussmaul sign), reflecting
the fixed diastolic volume of the right heart. Early dia-
stolic filling is unimpaired or even accentuated and is fbl
lowed by sudden cessation when total acceptable volume
is met, resulting ir.r a high frequency early diastolic sound
(pericardiai knock, heard in <50'X, ol patients). Pulsus par
adoxus is less frequent (<2O"/,, of patients) in constrictive
pericarditis than in cardiac tamponade. Peripheral eclema,
irscites, hepatomegaly, and pleural eftusions are com
mon. Diagnosis of constrictive pericarditis is made rvith
imaging studies and hemodynamic evaluation. Trans
thoracic echocardiography reveals nrlrmal right and lcll
ventricular size and systolic function despite prominenl
symptoms ancl finclings suggestive of heart failure. Dilat:r-
tion olthe inf'erior vena cava reflects elevated right sided
filling (right atrial) pressure. Doppler echocardiography
and tissue Doppler velocity are rcquired to differentialc
constrictive pericarditis fiom restrictive cardiomyopathy.
Al t hough an underlying cause o{' constrictive pericardi tis
is not always identified, previous pericarditis, cardiac
surgery, chest irradiati<in, connectivc tissue disorders, and
uremia are comnron precipitants.
Cardiac tamponade (Option A) can easily be conlused
with constrictive pericarditis. However, cardiac tamponade
is typically associated with pulsus paradoxus and not asso
ciated with Kussmaul sign.
A not uncomrnon misdiagnosis in patients with con
strictive pericarditis is cirrhosis. Likc patients with con
strictive pericarditis, those with cirrhosis may have a
palpable livet ascites, pleural eftusions, and periphcral
edema. Patients with chronic liver dise:rse (Option B) do not
have jugular venous distention, Kussmaul sign, or a pericur
dial knock. as detected in this patient.
In most cases. clinically diflerentiating restrictivc cilr
diomyopathy (Option D) from constrictive pericarditis is
impossible. Restrictive cardiomyopathy is more likely in a
patient with a predisposing systemic clisease, such as clia
betes mellitus or arnyloidosis. In this case, constrictive peri
carditis is suggested by the previous coronary artery bypass
surgery. Restrictive cardioml'opathy is not associated with a
pcricardial knocl<. lrut this flnding is sometimes difficult ttt
distinguish fiom irn S.,.
f,EY POIt{T
. Kussmaul sign and pericardial knock, if present, are
helpfirl clues to the presence ofconstrictive pericarditis.
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Bibliography
Geske JB, Ana"ekar NS, Nishimura RA. et al. Differentiation of constriction
and restriction: complex cardiovascular hemodl'namics. J Am Coll Cardiol.
2Ol6:68:2329 -17. IPMID: 278842.52] doi:10.1016 j.iacc.2016.08.050
Item 59 Answer: D
Ed ucati ona I Objective : Manage non- ST-elevation acute
eoronary syndrome with coronary angiography.
Urgent or irnrnediate angiographl' (Option D) uitl.r intent
to perftrnn revascularization is indicatecl in pitients $'ith
non S1'clevation acute coronirry syndronre
(NS'I'E-ACS)
who have retiactory angina or hemodynamic or electri
cal instabilityr
'll.ris
patient's presentatioll is l.riglrlv sugges
tive ol Nsl'lj ACS given his l.ristorl: ph1'sical exirmination
findings, ischernic ECG clunges. and troponin level. This
patient has an intermecliate'l'lNI I risk score ol,1 given his age
(>65 years): evidence of myocardial ischenria. including
ischemic ECG ST segment changes and elevatecl t()ponin
level; and recurrent chest pain episodes. Cornpared vlith
an ischernia guided approach. an invasive strrrteg,' ofl'ers
improved olltcomes ancl is inclicated in most prtients $'ith
higher risk ACS. r,r,ith underll,ing patient risk determining
the urgency and timir.rg. Whereas an invasivc assessment
with coronary angiography ancl consideratiou of revascu
larizatior.r are appropriate lvitl.rir.r 25 Io72 l.ururs ol presen-
tation in patients u'ith a'flN'll score of 3 or.1. this patient has
ongoing chcst cliscomfbn refiactor\, to stanclarcl rneasures:
thereibre, urgent angiography (r,r,ithin 2 honrs ol presenta
tion) is indicated.
ln low risk patients with NSI'E-ACS or in those r'vho
elect an initial ischemia guided therapeutic pathuay. 9616
cardiographl, (Option A) or stress testing can iclentifl' those
rvho nright benefit fiorn lurtl.rer ir.rvasive assessnrent rvith
subsequent revascularization. ln patients rvith increased
risk and active mlocardial ischemia or hemodynirnric or
electrical instrbility. echocardiography oflers lirnited early
benefit in the absence of signs or s).rlptolls ot rnechanical
cardiopuh.nonar y conrplicatirlns.
Eplerenone (Option B). an irldosterone ilntagonist, hits
provecl beneficial in pttients u'ith myocarclial intarction
who are rc'ceiving therapeutic closes ot ACE inhibitrtrs ancl
B blockers and have a lelt ventricular ejection fiactior, of'
40'X, or less ancl either diabetes nrellitus or heart Iailure. lhis
patient cloes not meet the criteria fbr eplerenone tl.rerapl:.
Routine oxygen therap), (Option C) l.ras lteen shou'n to
provide no benefit in ACS anci ma1, increase rclverse events
in the absence olhypoxenria. Cuidelines recontntend against
orygen aclnrinistration in patients rvith ACS irnd rixygen sat
uratior] greater than 90')1, to 92'X,.
t(tY PottT
. Urgent or immediate diagnostic angiography with
intent to perform revascularization is indicated in
patients with non-ST-elevation acute coronary syn
drome who have refractory angina or hemodynamic
or electrical instability.
Bibliography
Anderson JL, Morrow DA. Acute myocardial infarction. Engl J \'led. 2017:
376:2053 64. IP\'llD: 28538121] doi:10.1056 NEJ\{ra1606915
Item 60 Answer: D
Educational Objective: Evaluate a patient with palpita-
tions using a 30-day event monitor.
A 30-day event monitor (Option D) is the most appropriate
diagnostic testing option for this patient $,ith palpitations.
Event monitors are patient-triggered devices that are ideally
suited to capture symptomatic arrhythmias. The choice of
ambulatory cardiac rhl.thm monitor depends on the fre-
quency and duration of the symptoms as uell as any other
associated symptoms, signs, or findings. For patients such
as this one, with symptoms that occur frequently over the
course of a month but not daily, a 30 day event monitor
is most appropriate. The patient can activate the monitor
when symptoms occur, provided that the symptoms last
long enough to be captured by the device's active and ret
rospective capture. Symptoms that last longer than 1 to
2 minutes are ideally suited for event monitors.
An exercise ECG (Option A) allows diagnosis of exercise-
related arrhythmias and also allows lor assessment of the
efl'ect of exercise on blood pressure and symptoms. Most
arrhythmias are not exercise induced, and this patient
clearly indicated that there were no triggering events for her
palpations; therefore, exercise ECG is not indicated.
An implantable loop recorder (Option B) is most appropri
ate for infrequent or highly syrnptomatic arrhlthmias in which
the symptoms, such as syncope, might preclude a patient from
activating the device. These monitors are implanted under the
skin and can be used for months to years. This patient's symp
toms occw weekly and are not associated with slmcope; thus,
an implantable loop recorder is not necessary.
Mobile cardiac telemetry (MCT) (Option C) provides con
tinuous ambulatory ECG recording for precise quantification
or capture of rare arrhlthmias. MCT is not indicated for this
patient whose symptoms have not yet been correlated to any
specific arrhyhmia; thus, quantification of arrhlthmia bur-
den is unnecessary at this time. MCT is often used to capture
arrhythmias that may not be associated with symptoms (e.9..
occult atrial flbrillation). but this patient's symptom complex
seems fairly reproducible. Furthermore, her symptoms occur
frequently enough that an event monitor would be the most
resource-appropriate test to order at this time.
TEY POIilIS
. A 30-day event monitor is most appropriate for evalu
ating patients with palpitations who have symptoms
that occur less than daily but frequently over the course
of a month.
o An implantable looping event recorder is most appro-
priate for infrequent or highly symptomatic arrhyth-
mias in which the symptoms might preclude a patient
from activating the device, such as syncope.
:
186

199. Answers and Critiques
tr
Bibliography
Steinberg JS, Varma N, Cygankiewicz I, et al. 2017 ISHNE HRS expert consen
sus statement on ambulatory ECG and external cardiac monitoring/
telemetry. Heart Rhythm. 2017;14:e55-e96. [pMID: 28495301] doi:10.1016/
i.hrthm.2017.03.038
Item 61 Answer: D
Educational Objective: Diagnose takotsubo (stress-
induced) cardiomyopathy.
The most likely diagnosis is takotsubo (stress.induced)
cardiomyopathy (Option D). Takotsubo cardiomyopathy
is a syndrome characterized by transient regional systolic
ventricular dyslunction mimicking myocardial infarcticln
(reduced ejection fraction, eievated cardiac enzymes, and
signs of ischemia on ECC) in the absence of angiographic evi-
dence olobstrrrctive coronary afiery disease. In most cases of
takotsubo cardiomyopathy, the regional wall motion abnor-
mality extends beyond the territory perf'used by a single
epicardial coronary afiery. The syndrome is of'ten precipitated
by extreme emotional stress, such as death of a loved one.
The pathogenesis of' takotsubo cardiomyopathy is unknown,
but the condition is postulated to result from reversible myo-
cardial toxicily induced by very l-righ catecholamine levels.
Women are affected more fiequently than are men. Treat-
ment is generally srlppoftive and is similar to that for heart
lailure ofother causes; most patients recover cardiac function
over several months. Additional imaging studies may inciude
cardiac magnetic resonance imaging to exclude myocarditis.
Acute ascending aortic dissection (Option A) c'lassically
presents as severe, sudden onset chest or back pain that has a
tearing or ripping quality Other presenting fbatures may inc{ude
hypertension, syncope, a murrnur of aortic regurgitation, and
heafi failure. Acute ascending aortic dissection may cause acute
coronary occlusion; however, wall motion abnormalities would
most likely involve a single coronary artery distribution, and
obstructior.r would be detected by angiography In addition, acute
ascending aortic dissection can be visuaiized on echocardiogram.
Coronary aftery dissection (Option B) is usualiy identified
by coronary angiography, although the lindings may be subtle.
A single coronary artery dissection causes regionalwall motion
abnormalities in a single coronary artery distribution pattem
rather than global mid- and apical ventricular dysfunction.
Coronary artery embolism (Option C) is a potential
cause of acute myocardial infarction, resulting in abnormal
ventricular function in a patient with no obstructive coro-
nary artery disease; however, a coronary artery embolism
generally causes regional wa1l motion abnormalities in a
single coronary artery distribution rather than global mid
and apical ventricular dysf unction.
o Takotsubo cardiomyopathy is a syndrome character-
ized by transient regional systolic ventricular dysfunc-
tion mimicking myocardial infarction in the absence
of angiographic evidence of obstructive coronary
arterydisease. (Continued)
. Treatment of takotsubo cardiomyopathy is similar to
that for heart failure of other causes; most patients
recover cardiac function over several months.
Bibliography
Pal S, Broker M, Wagner H, et al. Stress (takotsubo) cardiomyopathy: a
review of its pathophysiolo$/, manifestations, and factors that affect
prognosis. Cardiol Rev 2021 Jul Aug OL;292O5-2O9. [PMID: 32282392]
doi :10.1097/CRD.0000000000000309
Item 52 Answer: C
Educational Objective: Treat acute ascending aortic
dissection with open aortic repair.
The most appropriate treatment fbr this patient with acute
type A aortic dissection is immediate open aortic repair
(Option C). The in-hospital mortality rate for patients
with acute type A aortic dissection is greater than 50'i1,
with conservative measures and typically 1O"/,, to 20"/,,
fbllowing surgery. In this patient with hypotension, asym
metric arm blood pressures and pulses, and murmur of
aortic regurgitation, emergency repair of the aorta and
aortic valve repair or replacement are indicated. Delaying
surgical repair has been associated with higher morbid-
ity and mortality rates, and expert consensus guidelines
recommend emergency repair in patients without clear
contraindications.
A retrospective study evaluated the role of coronary
angiography (Option A) in patients r,r,ith grpe A aortic
dissection who underwent emergency aodic surgery. In-
hospital mortality rates \,\,ere no difl'erent in those who
underwent coronary angiography cclmpared with those whr-r
did not. I.-urthermore, coronary angiography had no impact
on coronary aftery bypass grafting, as most of these proce
dures were performed fbr coronary artery dissection, not
atherosclerotic coronary ar1ery disease. These flndings sup
port the need to perlbrm aortic surgery as soon as possible
and not delay surgery fbr coronary angiography.
Although most guidelines recommend that patients
with acute aortic dissection be treated aggressively with
blood pressure reduction (systolic blood pressure goal of
<120 mm Hg in the first hour), this patient should undergo
emergent aortic repair due to evidence of' cardiogenic
shock. Interventions to reduce his blood pressure are not
needed. When indicated, intravenous p-blocker therapy is
used initially to lower blood pressure; decrease the veloc-
ity of left ventricular contraction; and reduce heart rate,
contractility, and aortic shear stress. Vasodilator therapy,
using agents such as nitroprusside (Option B), foilows
B-blockade.
There are reports of endovascular repair (Option D)
of ascending aortic dissection; however, open surgical
repair remains the standard of care and is recommended
by current guidelines. Furthermore, this patient requires
aortic valve repair or replacement due to evidence of aor-
tic regurgitation, and endovascular repair has not been
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200. Answers and Critiques
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shown to be efl'ective in patients with evidence of valvuiar
dysfunction.
xEY potltT
. In patients with ascending aortic dissection, immediate
open aortic repair is imperative to improve survival and
reduce morbidity.
Bibliography
Lawton JS, Liu l, Kulshrestha K, et al. The impact ofsurgical strate$/ on
survival after repair of type A aortic dissection. J Thorac Cardiovasc
Surg. 2015;150:294 301.e1. IPMID: 260050601 doi:10.1016/j.jtcvs.2015.
03.023
Item 63 Answer: B
Educational Objective: Treat primary (degenerative)
severe mitral regurgitation with surgical mitral valve
repair.
The most appropriate next step in management is surgical
mitral valve repair (Option B). This patient's echocar-
diogram is consistent with severe mitral regurgitation,
deflned as an effective regurgitant oriflce area of 0.4 cm2
or greater, a regurgitant volume of 60 mL or greater, or
a vena contracta of 0.7 cm or greater. The mitral regur-
gitation is primary (degenerative), as indicated by the
patient's midsystolic click and late mitral regurgitation
(both seen in mitral valve prolapse) and the demonstra-
tion of anterior prolapse by echocardiography. Although
the presence of symptoms resulting from severe mitral
regurgitation (such as shortness of breath and volume
overload) is an indication for intervention, class 1 indica-
tions for intervention in asymptomatic patients include a
left ventricular (LV) ejection fraction of 60% or less and/or
an LV end systolic dimension of 40 mm or greater. Surgi-
cal mitral valve repair is flrst-line therapy for patients with
primary severe mitral regurgitation meeting indications
for intervention.
In most cases, transthoracic echocardiography (TTE)
provides the data needed for adequate cardiac evaluation
of the patient with mitral regurgitation. However, in cases
in which TTE image quality is poor, cardiac magnetic reso-
nance (CMR) imaging (Option A) may be of value in mitral
regurgitation evaluation. This patient does not have an indi
cation for CMR imaging.
In severely symptomatic patients (New York Heart Asso
ciation class III or IV) with primary severe mitral regurgi
tation and high or prohibitive surgical risk, transcatheter
mitral valve repair (transcatheter edge to edge repair [TEER])
(Option C) is reasonable if mitral valve anatomy is favorable
for the repair procedure and the patient's life expectancy is at
Ieast 1year. This patient meets no indication for TEER.
Transesophageal echocardiography (Option D) may
be pursued when TTE is insufficient to determine either
the exact severity or the mechanism of mitral regurgita
tion (primary versus secondary); this patient's TTE was
sufflcient to identify the nature and severity of the mitral
regurgitation.
r(EY P0il{TS
. Surgery for chronic primary severe mitral regurgitation
is indicated in the presence of symptoms, left ventric-
ular dilation, or reduced ejection fraction.
. Surgical mitral valve repair is first line therapy for
patients with primary severe mitral regurgitation
meeting indications for intervention.
Bibliography
Bonow RO, O'Gara PT, Adams DH, et al. 2020 Focused update ofthe 20U
ACC expert consensus decision pathway on the management of mitral
regurgitationr a report ofthe American College ofCardiologr Solution Set
Oversight Committee. I Am Coll Cardiol. 2020;75:2236 2270. IPMID:
320680841 doi:10.1016/j.jacc.2020.02.005
Item 64 Answer: B
Educational Objective: Manage cardiac implantable
device infection.
Urgent pacemaker extraction (Option B) is the most
appropriate additional management. this patient pre-
sents r,^"'ith clinical signs of a pacemaker pclcket infection,
possibly due to skin contamination from a superficial
wound. Altl-rough infections most commonly are seen
shortly after pocket instrunlentation (e.g., following ini
tial implantation or generator change). they may occur
spontaneously and may present lvith only localized pain
or erythema over the pocket u,ithout systemic signs or
synlptoms ol inf'ection. This patient l.ras relatively clear
erythema. scabbing, and dimpling, likely rvith impending
erosion: however, pocket infection may be more insidious
ancl subtle. ln the absence of'major contraindications to
extraction andror extreme risk factors, curative therapy
requires extraction ol all implanted cardiac hardr,r,are,
debriden-rent ot the pocket, sustained antibiotic therapy
and reimplantation at a new location at'ter int'ection has
been eradicated. This patient does not har,e any obvious
contraindications to device extraction or extreme risk tac
tors (primarily age and comorbid conditions) that would
be prohibitive.
Aspiration of a cardiac implantable device pocket
(Option A) should never be perfbnr.red fbr diagr.rostic pur
poses. Device pocket infection is a clinical diagnosis. and
aspiration will only sene to inoculate a possibly sterile.
uninlected pocket. Furthermore. the diagnostic 1,'ield is
likely to be very lor,r,.
Topical mupirocin (Option C) is indicated for very
superficial skin infections. such as impetigo or fblliculitis.
'lbpical
antibiotics are ineft'ective fbr deeper skin or soft tis-
sue infections, such as cellulitis. and are totally inadequate
for this patient r,l'ith a possible pacemaker pocket inf'ection.
In addition, topical antibiotics often \'orsen the clinical sit
uation, as they may improve the appearance of the skin and
delay definitive care.
Clinical observation and reassessment (Option D)
are not appropriate in the setting ofdevice pocket infec
tion. The inf'ection tikely will progress and might cause
CONT
tr
188

201. Answers and Critiques
tr
Iurtl-rcr pocket erosion and,.or blrcteremia urith endo
ca r.i it is.
xlY P0ttTs
o Although infections of the cardiac device pocket most
commonly are seen shortly after pocket instrumenta-
tion (e.g., following initial implantation or generator
change), they may occur spontaneously and may pre-
sent with only pain or ery.thema over the pocket
without systemic signs or symptoms of infection.
r Effective treatment of cardiac device infection usually
includes complete extraction of all hardware, debride-
ment of the pocket, sustained antibiotic therapy, and
reimplantation at a new location after infection has
been eradicated.
Bibliography
Baddour LM, Epstein AE, Erickson CC, et al; American Heart Association
Rheumatic Fever, Endocarditis. and Kawasaki Disease Committee.
Update on cardiovascular implantable electronic device infections and
their management: a scientific statement fiom the American lJeart
Association. Circulation. 20101121:458 77. IPMID: 20048212] doi:10.1161/
CIRCU LATIONAHA.1O9.192665
Item 65 Answer: C
Educational Obiective: Discontinue clopidogrel after
drug-eluting stent placement in a patient with high
bleeding risk.
The most appropriate management is to discontinue clopido
grel (Option C). Current guidelines suggest treating patients
with stable angina with dual antiplatelet therapy (DAPT) for
at least 6 months after elective drug eluting stent (DES) place-
ment, although the optimal duration for any patient should be
selected on the basis of the patient's relative risk for throm-
botic and/or ischemic complications. Factors associated with
increased bleeding risk include advanced age; female sex;
anemia; low body weight; chronic kidney disease (CKD); dia-
betes mellitus; and concomitant therapy with glucocorticoids,
NSAIDS, or oral anticoagulant therapy. Risks for ischemic or
thrombotic complications include a complex or suboptimal
outcome of percutaneous coronary intervention (PCI), an
acute coronary syndrome, left ventricular dysfunction, exten-
sive coronary disease/ischemic history advanced age, diabetes
mellitus, and CKD. Current guidelines suggest that at least
3 months of DAPT in patients at high risk for bleeding may be
reasonable. This patient has several conditions with increased
risk for bleeding; after 4 months of DAPT, discontinuation of
clopidogrel is a reasonable consideration.
Platelet function analysis (Option A) has been proposed
as a method to optimize antiplatelet therapy in those at high
risk for bleeding or thrombosis. Routine use of platelet reac
tivity assessment to guide medical therapy, however, has not
been associated with improved clinical outcomes and is not
indicated.
In the absence of contraindications, lifeiong aspirin
(ls toz mg/d) is recommended for secondary prevention in
patients with established coronary artery disease. Although
recent studies have evaluated the potential benefit of P2Y,
inhibitor monotherapy (ticagrelor) after DES-based pCI,
currently no guideline consensus exists for this practice;
thus, discontinuing aspirin (Option B) is not appropriate.
Proton pump inhibitors (PPIs), such as omeprazole, are
indicated in patients treated with DAPT who have a history
ofupper gastrointestinal (UGI) bleeding and are reasonable
in patients at high risk for UGI bleeding. Whereas pharma-
codynamic studies suggested that omeprazole may attenuate
the effect of clopidogrel, subsequent clinical studies have
not shown a meaningful increase in adverse cardiovascular
outcomes among patients taking these medications together.
Discontinuing omeprazole (Option D) may be appropriate
for this patient if she remains without symptoms of gas-
troesophageal reflux disease. PPI use is not recommended
routinely in patients receiving DAPT.
f,rY POtl{TS
. In a patient treated with dual antiplatelet therapy for
coronary artery disease at low bleeding risk, guide-
Iines suggest treatment duration of at Ieast 6 months
after elective drug-eluting stent placement.
o Dual antiplatelet therapy for at least 3 months may be
reasonable following elective placement of a drug-
eluting stent in some patients at high risk for bleeding.
Bibliography
Levine CN, Bates ER, Bittl JA, et al. 2016 ACC/AI IA guideline fbcused update
on duration ofdual antiplatelet therapy in patients with coronary artery
disease: a report ofthe American College ot Cardiolos//American Heart
Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol.
2016;68:1082-115. IPMID: 27036918] doi:10.1016/j.jacc.2Ol6.03.513
Item 66 Answer: B
Educational Objective: Treat end stage primary restric-
tive cardiomyopathy with cardiac transplantation.
Cardiac transplarrtation (Option B) is the most eppropri
ate treatment lbr this patient.
'lhe
patient has syr.nptonrs of'
refractorl' heart failure despitc eflofls at diuresis and rhl thnr
control.
'lhe
f'eatures of preserved sy'stolic tlrnction. signif
icant biltrial dilatation, ancl pulmonary hypcrtensit.rr.r on
cchocanliogram supl)ofi thc diiigr.rosis of reslrictire carclio
m1opathl, (RCIVI). Sun'ival is poor in patients with RCM. r,r'ith
:r 5 year rnortality rete of 36'/;, and a lO-year rnortality rltc
o{ 63')1,. Cardiovascular nrurtaliff is predominlntly rclatecl
to progressire l.rerrt failure and arrhl'thnrias. Nlortality rates
increasc with malc sex, scvcrity ol left atrial dilatation.
age oldcr than 70 _years, iind Nerv \irrk lIeart Association
(NYHA) functional class.
-lhe
patient should be retbrrecl to
ln ad.uuncecl heart failure ancl transplantation specialist ltrr
evirluation lbr possible cardiac transplanlatior.r. Heafi trlns
pl antati on r.na1' be I i rn iteci b1' pulmonrr
ry, h1'pertension.
Cardiac resyrrchronization therirl'ry (CR'l') (Option A)
is indicated for patients rtho have ref'ractory hearl lirilure
svnlptonls despite guidelinc clirectecl tneclicrrl thcrapl' 'nr itl't
lett ventricular ejcction filctiott less than l)S')i, and QItS
duratiorr grcater than 150 ms, optimally
"vith
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189

202. Answers and Critiques
CONT.
branch block configuratittn ancl sinus rhythnl. Ihis l"rittient
does not have an indicltior.r for CItl.
Placement of an implantable cardiovertcr defibrillator
(Option C) is recommended in patients r,t'ith heart lailure
who have an ejection liactior.r of 35',{, or less and \'H,\
lunctional class Il or III symptoms while receiving guicleline
directed medical therap1,.'[here is no accepted indication lbr
primary prevention IOI) placernent in patiellts \'ith RCM
rvho have preserved systolic function.
Pulmonary vein isolation ttrr rhythrn control (Option D)
nray be appropriate in c<tnjunction with thcrapy tci
reduce stroke risk in patients with s)'mptornatic atrial
fibrillation unresponsive to irntiarrhytl.rmic ttedicatiot-ts.
However. in this patient with severe left atrial dilation.
persistcnt atrial fibrillation fbr more than 1 year. ancl
evidence of underlyir.rg RCM. it is unlikely to prttvide a
durable solution to the patierlt's problcnl and carries
procedural risk.
I(EY POII{I
o Heart transplantation is an effective therapy for select
patients with end-stage restrictive cardiomyopathy.
Bibliography
Pereira NL, Grogan M, Dec CW. Spectrum of restrictive and infiltrative car
diomyopathies: part 2 ofa 2.part series. J Am Coll Cardiol. 2018171:1149
66. [PMID: 29519356] doi:10.1016'j.Fcc.2018.01.017
Item 67 Answer: B
Educational Objective: Diagnose coronary artery disease
with exercise ECG.
'lhe
most appropriate managemcnt is exercise IICG (Option B)
lbr this patient with typical angina sy'rnptor.t.ts.
'lhe
eval
uation of' undiagnosed chesl pain begins with assessittg
the patient's pretest probabilitl' ol coronarl' artery diseasc
(CAD). This patient's age. sex. and nreclical hiskrry place lrim
in the ir.rtcrmediate risk category. and exercisc llCG fttr CAI)
is best applied to patients in this group. Unless there are
contraindications to exercise. exercise testing is pref'erred
to pharmacologic stress testing. Absolute contraindicatiot.ts
tci exercise include unstable angina or acute myocirrdial
infarction. uncontrollecl arrhythmias. decompensated heart
Iailure, acute pulmonrry embolism or deep venous throm
bosis. acute pericarditis or myocarditis, acute aortic dissec
tion. and severc symptonlatic aortic stenosis. Exercise ECC
is recommended as the initial test of choice in patients lt ith
normal findings on baseline ECG.
'llris
patient's baselinc
irCC has no findings that u,ould in.rpair interprctation of the
results. such as S'f segment depression greater than 1 mm.
lctt bunclle branch block. left ventricular hypertrophl: paccd
rhythm. or preexcitation.
Calcification of the coronirry arteries is indicutive ol
atherosclerosis and may be quantifled with C'l. Although
coronary artery calcium scoring (Option A) provides inlbr
mtrtion regarding the burder.r ot disease, i1 canl-rot confirnr
that thc patient's symptoms are due to obstructive CAD or
guide imrnecliate treatlxent decisions, st"tch as the' neecl tbr
revl scula rizatictt-t.
Lxercise echocirrdiogrlphy
(Option C) adcls echocardio
grrrphic irnaging to an EC(l stress lest. F]xercise stress testing
n itl.r imirgirrg is irtdicated in pirticnts u'ho lrirve baseline F-CG
rrbnormalities that limit interpretatioll of'the cxcrcise F.CG.
or u,ho have indeterntinate firrclir.rgs on the erercise UCG.
lor this patient, adding echocarcliogrlphy is not necessary
ancl nould not be a cost ef fective methoci to diagtrose CAI).
Ambulatory l.CG (Option D) woulcl be appropriate it the
patient's s!'mptolxs or ll(l(l lirlclings sttggestcd arrhythmia
as the cause ol his slinlpttlms. Irirst clegree atrioventricular
block is il comnton finclirtg ort tiCG and does not neces
strrih' increase tl.tc suspicit)n that the patient is havirlg an
arrhllhmil. Tl-tis patient's sylllptoms ilrc nlosl consistent
rvith angina.
t(EY P0l llTs
o The evaluation of undiagnosed chest pain begins with
assessing the patient's pretest probability of coronary
artery disease.
o Stress testing is most effectively used in patients with
an intermediate pretest probability of coronary artery
disease. and exercise ECG is recommended as the ini-
tial test of choice in patients who are able to exercise
and have normal findings on baseline ECG.
Bibliography
Ferraro R. Latina JM. Alfaddagh A, et al. Evaluation and management of
patients with stable angina: beyond the ischemia paradigm: JACC state
of the art review. J Am Coll Cardiol. 2020176,2252 2266. IPMIDI
331535861 doi:10.10161j.jacc.2020.08.078
Item 68 Answer: D
Educational Objective: Continue warfarin during preg-
nancy in a patient with a prosthetic mechanical valve.
Continuing the INR adjusted warfarin regimen (Option D)
is most appropriate for this patient. Mechanical mitral valve
prostheses increase risks to the patient and fetus during
pregnancy. Warfarin anticoagulation seems to be the safest
agent to prevent maternal prosthetic valve thrombosis; how
ever, warfarin poses increased fetal risks, including terato
genicity, miscarriage, and fetal loss because of intracranial
hemorrhage. Risk to the fetus is dose related; warfarin is
the preferred anticoagulant during the first trimester if the
dosage is 5 mg/d or less. During the second and early third
trimesters, warfarin is the preferred anticoagulant.
Bivalirudin (Option A) has not been demonstrated to
provide adequate anticoagulation coverage for a pregnant
patient with a prosthetic mechanical valve and should not
be used.
For patients with a mechanical valve prosthesis, the
direct thrombin inhibitor dabigatran (Option B) should
not be used because of increased risk for harm. A ran
domized clinical trial of dabigatran in nonpregnant patients
with a mechanical heart valve showed an increased rate of
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190

203. thromboembolic and bleeding complications with dabiga
tran compared with rrarfarin. The effectiveness of apixaban,
rivaroxaban, and edoxaban (anti-Xa direct oral anticoagu
lants) has not been established in patients with a mechanical
heart valve, and the safety ofthese agents in pregnancy is not
known; therefore, they are not recommended.
In patients who prefer not to take warfarin during the
flrst trimester of pregnancy or if the warfarin dosage is more
than 5 mg/d, intravenous dose adjusted unfractionated hep
arin may be used. However, subcutaneous unfractionated
heparin (Option C) does not provide adequate anticoagu
lation coverage for a patient with a mechanical prosthetic
valve. Intravenous unfractionated heparin is the preferred
anticoagulant option for patients with a mechanical valve
around the time of delivery.
l(EY P0r1{rs
o For pregnant patients with a mechanical valve pros
thesis, warfarin is the preferred anticoagulant during
the first trimester if the dosage is 5 mg/d or less; war-
farin is the preferred anticoagulant during the second
and early third trimesters.
o Direct oral anticoagulants are not recommended in
patients with a mechanical valve prosthesis.
Bibliography
otto CM, Nishinrura RA, Bonow RO. er al. 2020 ACO AIIA guideline tbr rhe
nranagement of patients with valvular heart disease: a report of the
American College of Cardiologr/American Heart Association Joint
Committee on Clinical Practice Guidelines. Circulatirn.2O2l:143:e7 ) e))7.
IPMID: 333321501 doi:l0.ll6l/CIR.0000000000000923
Item 69 Answer: D
Educational Objective: Avoid unnecessary testing in a
patient with asymptomatic peripheral artery disease.
No additional testing (Option D) is necessary. This patient
has both coronary afiery disease and peripheral artery dis
ease (PAD), a combination associated with a higher risk
fbr major adverse cardiovascular events. In patients with a
history or physical examination flndings suggestive of PAD,
the American College of Cardiologr recommends resting
ankle brachial index (ABI) testing to establish the diagnosis.
This patient's diagnosis of PAD was confirmed by a low ABI
in both lower extremities (0.80 on the right and 0.86 on
the left), and he does not require additional testing. He is
being treated appropriately with guideline directed medi
cal therapy, including tvvo antiplatelet agents, a p-blocker,
an ACE inhibitor, and a statin. There is also a 45-mm Hg
difference in arm blood pressures in this patient. Although
a difference in arm systolic pressures of greater than 15 to
20 mm Hg suggests subclavian or innominate artery ste
nosis, in the absence of symptoms (arm claudication or
symptoms olvertebral artery steal), no further imaging or
intervention is warranted.
Exercise ABI testing (Option A) is useful in establish
ing the diagnosis of PAD in the symptomatic patient when
Answers and C
resting ABIs are normal or borderline. ABI testing is also
useful to differentiate claudication from pseudoclaudication
(spinal stenosis) in persons with exertional leg symptoms.
This asymptomatic patient has established PAD based on an
abnormal resting ABI, and additional testing with exercise
ABI is not needed.
CT angiography (Option B) and magnetic resonance
angiography are often reserved for planning endovascular
or surgical revascularization in patients with PAD. Because
this patient is asymptomatic, there is no indication for CT
angiography.
In patients with conflrmed PAD, toe-brachial index
testing (Option C) has no added utility. The toe brachial
index is useful when the resting ABI is greater than 1.40,
indicating the presence of noncompressible, calcifled arter-
ies in the lower extremities. In these patients, an appropriate
next step is great toe pressure measurement or toe-brachial
index calculation (systolic great toe pressure divided by sys
tolic brachial pressure).
t(EY POtltrS
r The American College of Cardiologz recommends
resting ankle brachial index testing in patients with
history or physical examination findings suggestive of
peripheral artery disease.
o Patients with both coronary artery disease and
peripheral artery disease are at higher risk for major
adverse cardiovascular events.
Bibliography
Gerhard Ilerman MD, Gornik HL, Barrett C, et al. 2016 AHA/ACC guideline
on the management of patients with lower extremity peripheral artery
disease: executive summary: a report of the American College of
Cardiology/American []eart Association Task Force on Clinical
Practice (luidelines. Circulation. 201 7;135:e686-e725. IPMID: 27840332)
doi:10.1161 iCIR.00000O0000000470
Item 70 Answer: C
Educational Objective: Treat new onset symptomatic
heart failure with an ACE inhibitor.
'lhc
most uppropriirte trcatment is rrn ACI: inhibitor. such as
cnalapril (Option C), ttrr this patient l'nith new onsct
1>eri
l)artum crrrcliomyr4rrrtIr1' anci hcart lailure. Follou,ing dcliv
er\. patients u,ith peripirrtunr clrcliomyoplthy are treated
the samc rrs rrll other l)atients r,r,ith heart tailure. ACI inhib
itors reclucc morbidity and nrurtalitlr in pltients rtith hcart
Iirilure."n'ith reclucccl cjection fiaction (HFrl.-l) and should be
r-rscd in botl.r s1-r.nplonratic and ilsymptonlatic patients. r\CE
irrlribitors cirn be startecl immccliately in l)atients r,r,ith acute
hcart failurc in thc absence of hyperkalenria; the estinrated
gkrmerular liltration rxte shollld lre monitoleri during upti
trirtion. lrnrrlapril. captopril, cluinapril, ancl benazcpril are
I)rcsenl in vcry low lcvels in breasl milk ancl are consiclered
s:rfe during breastll'ccling. \hlslrtan-sacubitril shoulcl r.tot
be used cluring brclstf-eeding beciruse ol thc potcntial fbr
serious aclverse reactions in infants.
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191

204. Answers and Critiques t
3
art
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lhe p blockers rnetoprolol. can'edilol. and bisoprolol
llJ
19p1;6n A) aiso reduce mortalig in patients rvith HFTEF.
coNT
but trextment should be clelayed in patierlts lr,ith volunre
ove'rload (jugular venous distentior.r. crackles. ederna) and
reduced cardiac output (S.) bccause treatment in these
patients may \'orselt symptonts. Guidelines recommend
reclucing intrar,'ascular volume with a loop diuretic and ini
tiating an ACE inhibitor;
B blockers ntay be cautiously intro
ducecl rvhen the patient is closer to being euvolemic.
In small trials, the nondihldropyridine calcium chan
nel blockers diltiazern (Option B) and',eraparnil have been
associated w-ith :rn increase in rnortali[,- in patients with
heart failure. Ifthis patient's hypertension persists after she
receives maximal doses of an ACF. inhibitor ancl B blocker.
one might consider adding a dihydropvridine calcium chan
nel blocker (e.g.. anrlodipine. felodipine); these agents have
been shown to be saf'e in patients with heart failure.
lvabradine (Option D) inhibits the sinus node and
reduces heafi rate in patients with heart lailure. lt has been
sholr,n to reduce heirrl failure hospitalizations r,r'hen added
to nr:rximally tolerated B blocker therapyl This patient is uot
tal<ing a B blocker, making this an inappropriate addition at
this time.
rEY POITTS
. ACE inhibitors reduce morbidity and mortality in
patients with heart failure with reduced ejection frac-
tion and should be used in both symptomatic and
asymptomatic patients.
o Metoprolol, carvedilol, and bisoprolol reduce mortal-
ity in patients with heart failure with reduced ejection
fraction, but treatment should be delayed in patients
with volume overload until the patient is closer to
being euvolemic.
Bibliography
Yancy CW, Jessup M, Bozkurt B, et al; American College of Cardiologr
Foundation. 2013 ACCF/AHA guideline for the management of heart
failure: a report of the American College of Cardiolory Foundationr
American Heart Association Task Force on Practice Guidelines. J Am Coll
Cardiol.2013;62:e147 n9.lPMlDt23747642ldoi:LO.1O16/j.iacc.2013.05.019
Item 71 Answer: A
Educational Objective: Diagnose Brugada syndrome.
The most likely diagnosis in this patient with recurrent
syncope is Brugada syndrome (Option A). Cardiovascular
syncopal events often occur suddenly and usually with
out a signiflcant prodrome, although chest pain and palpi
tations may be present. Causes of cardiovascular syncope
include cardiac arrhythmia; coronary artery disease; and
structural and obstructive disease, including aortic and
pulmonary valve stenosis, obstructive hypertrophic car-
diomyopathy, aortic dissection, and cardiac tamponade.
Brugada pattern is distinguished by a structurally normal
heart (normal echocardiogram) and right precordial ECG
abnormalities, including ST segment coving (concave or
linear downsloping ST segment) in leads V, through V" with
or without right bundle branch block. Brugada syndrome is
the association of Brugada pattern with ventricular fibril-
lation, arrhythmogenic syncope, or cardiac arrest. Brugada
syndrome has an increased prevalence in men and persons
of Asian descent. Arrhythmic events, including sudden car
diac death, in patients with Brugada syndrome are more
common at night, during sleep. Abnormalities on ECG may
be intermittent and may be elicited by fever or pharma
cologic challenge with sodium channel blockade, such as
procainamide infusion. The presence of Brugada pattern on
ECG, an unexplained syncopal event, and relevant family
history (father who died in his sleep at age 45 years) are
highly suggestive of Brugada syndrome.
This patient has no exertional symptoms or ischemic
changes on ECG, and his symptoms are paroxysmal. Inter-
mittent syncope would be a very unusual presentation of
coronary artery disease (Option B), particular$ in this age
group.
Long QT syndrome (Option C) is among the most com-
mon inherited arrhythmias, affecting between 1 in 1000
and 1 in 5000 persons. The presence of a prolonged
QTc
(>440 ms in men and >460 ms in women) alone is insuf-
flcient to diagnose long QT syndrome. This patient's
QTc
interval is normal, and long QT syndrome is not associated
with the findings seen on this patient's ECG.
This patient has no prodrome offlushing, light-headedness,
or dizziness, which is typical of vasovagal slmcope (Option D).
Finally his s,.ncope and associated ECG findings are of great
concern and are consistent with Brugada qmdrome.
f,IY POI lII!
. Cardiovascular syncopal events often occur suddenly
and usually without a significant prodrome, although
chest pain and palpitations may be present.
o Causes of cardiovascular syncope include cardiac
arrhythmia; coronary artery disease; and structural
and obstructive disease, including aortic and pulmo-
nary valve stenosis, obstructive hypertrophic cardio
myopathy, aortic dissection, and cardiac tamponade.
Bibliography
Al-Khatib SM. Stevenson WG. Ackerman Ml. et al. 2017 AHA/ACC/HRS
guideline for management of patients with ventricular arrhythmias and
the prevention ofsudden cardiac death: a report ofthe American College
ofCardiologr/American Heart Association Task Force on Clinical Practice
Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2018;72:
e91-e220. IPMID: 290972961 doi:10.1016/j.jacc.2017.10.054
Item72 Answer: B
Educational Objective: Treat infective endocarditis with
early valve replacement surgery.
The most appropriate nlrnagement is earlv surgical aortic
valve replacement (Option B). This llatient's history and
physical examination findings are consistent with infec-
tive endocarditis (lE), severe aortic regurgitation (murmur,
bounding pulses. widened pulse pressure), and heart failure.
192
tr

205. :
Answers and Critiques
tr
In addition. the liCG denronstrating prolonged first dcgree
atrior,entricular block is concerning fbr involvernent of the
atrio'"entricular nocle. rn,hich sits close to the aortic vllve
and may be involved in aortic valve endocarditis, especially
ilan aortic abscess is present. In patients r,r,ith IE r,vho pre
sent with valve dysfunction resulting in sympk)ms ol heart
failurc, or [E complicated by hearl block, annular or arlrtic
abscess, or destructive penetrating lesions, early surgery
during the initial hospitalization ancl before completiorr of a
full therapeutic course of antibiotics is indicatecl.
Occasionally. cardiac crtheterization (Option A) to
define coronary ilnatomy rnay be pursued before surgical
aortic valve replaccment to determine whether adjunctive
coronary artery bypass grafting is needed. l]owever. the
catheterization finclings would not change the need fbr aor
tic valve replacement and should not clelay surgical manage
ment in tl.ris patient.
Sinrilarly', ltlacement of a tentporary pacemaker
(Option C) does not address the undcrlying worsening rtrr
tic valve infection ar.rd conductioll systeln contpromise by an
abscess: therefbre, it shoulcl llot delay surgical management.
Patients with IE and heart failure treated with surgery
have a 2l'X, in hospital mortality rate c<tmpared with a ratc of
45'li in those tlcated medicallli In aclclition. extensive pxra-
valvular inf'ections (including annular or ilortic abscesses
and destructive penetrating lesions or fistulae) irre associated
n'ith a mortality rate of
.,l0')1, or higher ar.rd heart block. Earll'
surgery is associated vvith ln actuarill survival rate ol 75')1, at
.5 years. Continuing current therapy withollt early surgery
(Option D) is not inclicatecl.
I(EY POI ]IT
. Early surgical valve replacement is indicated in
patients with infective endocarditis and heart failure,
annular or aortic abscess, or destructive penetrating
lesions.
Bibliography
Otto CM. Nishimura RA. Bonow RO, et aI.2020 ACC/AIIA guideline for the
management of patients with valvular heart disease: a report of the
American College of Cardiology/American Heart Association Joint
Committee on Clinical Practice Guidelines. Circulation. 2O2l:143:e72
e227. I PMID: 33332lsol doi:10. I 161 /CIR.000000000000o923
Item 73 Answer: B
Educational Obiective: Treat iron deficiency in cyanotic
congenital heart disease.
The most appropriate management is a short course of
iron therapy (Option B). This patient has patent ductus
arteriosus related Eisenmenger syndrome and symptom-
atic relative anemia. Most patients with cyanosis have com-
pensated erythrocytosis with stable hemoglobin levels. Iron
deflciency and resultant microcytosis in these patients are
often caused by inappropriate phlebotomy or blood loss,
such as menorrhagia, as in this case. This patient's baseline
hemoglobin level was nearly 18 g/dl (180 g/L), and initial
therapy should include oral iron therapy, which often causes
a rapid increase in erythrocyte mass. When the hemoglobin
level and hematocrit begin to increase in 7 to 10 days, iron
therapy should be discontinued. Correction of iron defl
ciency is associated with increased exercise capacity and
improved quality of Iife.
Menorrhagia is the presumed cause of relative anemia in
this patient, and management of menorrhagia is indicated.
Treatment may involve endometrial ablation, intrauterine
device placement, and, rarely, hysterectomy (Option A). Sur
gical interventions are generally avoided in patients with
Eisenmenger syndrome unless they are necessary owing to
increased perioperative risk. The initial treatment is to cor
rect iron deflciency anemia.
In patients with cyanotic congenital heart disease,
phlebotomy (Option C) is recommended for symptomatic
hyperviscosity (headaches, reduced concentration) with a
hemoglobin level greater than 20 g/dl (200 g/L) and hema-
tocrit greater than 65% in the absence ofdehydration. Phle
botomy is not indicated in this patient with a hemoglobin
level that is low for a patient with Eisenmenger syndrome.
In patients with cyanotic heart disease, guidelines rec-
ommend supplemental oxygen (Option D) as needed for
symptom relief of dyspnea but not to a target oxygen satura-
tion level and not to be continued if there is no symptomatic
beneflt. A more physiologic strateg/ to address this patient's
exertional dyspnea is to treat her iron deflciency anemia.
Her symptoms will improve quickly with appropriate iron
replacement.
TEY POIl{IS
o Correction of iron deficiency is associated with
increased exercise capacity and improved quality of
life in patients with cyanotic congenital heart disease.
. Adaptive erythrocytosis is well tolerated in patients
with cyanotic congenital heart disease, and therapeutic
phlebotomy is not indicated until hemoglobin con
centration is greater than 20 gldL (2OO glL).
Bibliography
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the
management of adults with congenital heart disease: a report of the
American College of Cardiolog//American Heart Association Task Force
on Clinical Practice Guidelines. J Am Coll Cardiol. 2019:73:e81-e192.
IPMID: 30121239] doi:10.1016/j.jacc.2018.08.1029
Item 74 Answer: C
Educational Objective: Treat ST elevation myocardial
infarction with thrombolysis.
This patient u'ith an inf'erolateral S'l' elevation myocardial
inlarction (STEMI) is best mirnaged by thrombolysis folloned
by transfer to a pcrcutaneous coronilry intervenlion (l']Cl)
center within 24 hours (Option C).
'lhrombolytic
thcrapy
is recomnrended lirr patients with STEMI if symptom <lnset
is $,ithin 12 hours and primary PCI is r.rot available rt,ithin
120 minutes of first medical contact. If symptonrs began l2 to
24 hours befbre presentatior.r and/or tl.rere is hemodynamic
CONT.
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Answers and Critiques
instability, significant myocardium at risk (such as rvith
anterior MI). or ercessive bleeding risk. transler fbr printlry
PCI (thc preferrecl strategn,) can bc consiclered. although
thrombolytic therirpy shoulcl be consiclered if timeil.transf'er
(<120 minutes) is not arailiible. Thronrbolytic therapl,is n.rost
effectirre u,ithin the flrst lJ to 6 hours irom syrnptom onset.
ln additior-r to thrornboll,tic therapy. all patier.rts rtithout a
specific contrainclication should recei\e a loacling dose of
aspirin as rtell as intravenous unfractionatecl heparin. error
aparin. or fbndaparinux. Olopidogrcl loading (SOO mg oralty)
has been demonstrated to increase rittes of vcssel patencl'
and is also recommended ir1 this setting.
This p:rtient has clear evidence of :rcute Sl'Fllvll. ancl ther
apeutic clelal,-fbr completion olC'f angiographl' (Option A) or
other inraging nrodalities is not rvarranted.
thron.rbolytic therapy' is r,r,idely availablc and is a rc':r
sonable therapeutic choicc u-hen primary I'Cl is delal,ed
b1, more than 120 minutes.
'[hromboll,tic
therapv has been
showr.r to be most beneficial rvhen incorporatccl into a phar
macoinvlrsive trelrtment protocol at non-PCI centers. This
involves delivery'of thron.rboly'tic therapy earll' alter st'rnp-
tom onsct fbllowcd by transler to a l)CI capable hospit:rl fbr
elective angiography ancl re'vascularization. Early routine
transfbr has shor,r,n benef it in allou'ing earl1, "rescue" PCI fbr
patierlts r,vho do not denlonslrate evidence o{ reperfusion or
sufl'er rcocclusion irnd reinfarction af ter delivery of throm-
bol)'tic therapl.l Thromboh,sis and adrnission to a telenretry
bed (Option B) is not the most appropriate option.
Whcreas primary PCI delilered rvithin 12 htiurs of synrp
tom ulset is associirted r'r'ith louer rates of deatl.t. reinf'arction.
and blcecling tlran thrombollrtic therapy, delays in reperfusior-t
limit this potential benefit firr patients unable to receive PCI
rvithir.r
'l20
minutes of first medical contact (Option D). Pri
rnary I'}Cl rentains tl.re prelbrred stratcp5' urhen rcadill, ar,ailirble
(first nrcdical contirct to balloon time ol <90 minutes). Prinrlry
PCI is also reconrmended fbr high risk patients rvith a late
presentation or hernodl,narnic,'electrical instabilitli at the clis
cretion o1'providers assessing the perceived risk benefit rltio.
XEY POIIIT
. For patients with ST-elevation myocardial infarction,
thrombolytic therapy is recommended if symptom
onset is within 12 hours and primary percutaneous
coronary intervention is not available within 120 min-
utes of first medical contact.
Bibliography
Anderson JL, Morrow DA. Acute myocardial infarction. N Engl J Med. 2017;
376:2053 64. [PMID: 28538121] doi:10.1056 NEJMral606915
Item 75 Answer: A
Educational Objective: Diagnose athlete heart.
The most likely diagnosis is athlete hearl (Option A). It is
important, and sometimes difficult, to distinguish normal
training-related adaptive changes of the heart from poten
tially life-threatening pathologic processes. This distinction
aflects the patient's ability to continue to participate in ath-
letics as well as the consideration of further testing and treat-
ment. Structural adaptations of the left ventricle in response
to rigorous training include dilatation and increased wall
thickness; these flndings do not necessarily indicate pathol
ogr. In a study of elite athletes, left ventricular (LV) end
diastolic diameters ranged from 38 to 66 mm in women (mean,
48 mm) and from 43 to 70 mm in men (mean, 55 mm).
Markedly dilated LV chambers (>60 mm) were most common
in athletes with higher body mass and those participating
in endurance sporls. Increased wall thickness is common,
but wall thickness greater than 13 mm is uncommon in elite
athletes and should raise suspicion for possible underlying
pathologr. LV diastolic fllling in athletes is most often normal
but may show enhanced passive LV fllling. Abnormal diastolic
fllling patterns are more common in patients with pathologic
conditions. LV ejection fraction is usually normal in athletes.
Fabry disease (Option B) is associated with increased
LV wall thickness. However, it is also associated with fatigue,
burning dysesthesia in the extremities, and angiokeratoma,
none of which is present in this patient.
This patient has no history of hypertension, making
hypertensive heart disease (Option C) an unlikely cause of
load dependent hypertrophy.
Although the patient has no family history of hyper
trophic cardiomyopathy (HCM), spontaneous genetic muta-
tions may occur. This patient's flndings are consistent with
athlete heart, but il wall thickness were greater or if clinical
concern remained high, a period of several months of decon
ditioning lollowed by re evaluation, or cardiac magnetic
resonance imaging with gadolinium, would be useful to
differentiate betvveen athlete heart and nonobstructive HCM
(Option D) or another condition.
I(EY POIl{I
. Increased left ventricular (LV) wall thickness and LV cav-
ity dilatation may be normal findings in highly trained
athletes; s).irnmetric wall thickness of 13 mm or less and
normal diastolic filling favor the diagnosis of athlete heart
over the diagrosis of hypertrophic cardiomyopathy.
Bibliography
Baggish AL. Wood MJ. Athlete's heart and cardiorascular care ofthe athlete:
scientitic and clinical update. Circulation. 2oll;123:2723 35. IPMID:
2767 0241 I doi:10.1 l 6l /cIRCULA IIONAHA. 110.981s71
Item 76 Answer: C
Educational Objective: Diagnose radiation-associated
coronary artery disease.
The most likely diagnosis is coronary artery disease (Option C).
Although the patient has no typical risk factors for athero
sclerosis, she underwent significant radiotherapy at an early
age that included the heart within the fleld. Cancer survivors
who received such radiotherapy are at risk for the late devel-
opment of cardiovascular complications, including pericar
dial constriction, valvular heart disease, and coronary artery
194

207. Answers and Critiques
disease. Stenosis is often ostial within the coronary arteries
and may be more fibrous or fibrocalciflc than typical ather
oma. Anginal symptoms, especially in women, may not be
typical. Given the exertional nature of this patient's symp
toms and the lack of other features supporting a pericar
dial or valvular underlying cause, a high index of suspicion
should be maintained for ischemic disease.
Patients with signiflcant aortic or mitral valvular disease
may present with exertional dyspnea, but this patient's normal
flndings on auscultation make the diagrosis of valr.ular heart
disease less likely. Severe aortic stenosis (Option A) is associated
with a late-peaking systolic murmur, a diminished or absent
aortic component of the Sr, and a delay in the carotid upstroke
(pulsus tardus). Mitral stenosis (Option D) produces a low
frequency diastolic murmur heard best at the cardiac apex.
Pericardial disease is a potential cardiac sequela ofradio
therapy, but late constrictive pericarditis (Option B) generally
presents in an indolent fashion with symptoms of fatigue and
dyspnea limiting exertion. Jugular venous distention is usually
present, and the Kussmaul venous sign may be seen (no change
or rise in jugular pressure with inspiration). An early diastolic
sound (pericardial knock) may be heard. Increased abdominal
girth (ascites) may be present, and peripheral edema is com
mon. This patient's presentation is not compatible with the
diagnosis of constrictive pericarditis.
Radiation also damages the microvasculature, caus
ing endothelial dysfunction and ischemia that result in
myocardial flbrosis, diastolic dysfunction. and restrictive
physiology. Radiation induced cardiomyopathy presents
similarly to primary restrictive cardiomyopathy (Option E),
with symptoms ol dyspnea, peripheral edema, and exercise
intolerance. There is usually evidence of significant pulmct
nary hypertension (loud S, and eventually widely split 52),
and tricuspid and mitral valve regurgitation are commonly
present. In this patient with a normal cardiac examination.
coronary artery disease is the most likely cause of exertional
dyspnea and burning throat pain.
I( EY PO I lII
o Cancer survivors who received chest radiotherapy are
at risk for the late development of cardiovascular
complications, including pericardial constriction, val
vular heart disease, restrictive cardiomyopathy, and
coronary artery disease.
Bibliography
Groarke JD, Nguyen PL, Nohria A, et ,1. Cardiovascular complications ol
ridiation therapy fi)r thoracic malignancies: the role tor non invlsive
imilging for detection ofcardiovascular disease. Eur I Ieart J. 2014i35:612
23. IPMID: 23666251] doi:10.109i1 eurhearti,'eht11.l
llem77 Answer: A
Educational Objective: Treat heart failure with a cardiac
resynchronization therapy defibrillator.
A cardiac resynchronization therapy (CRI') deflbrillator
(Option A) is indicated in this patient with relatively new onset
symptomatic heart failure who is receiving guideline-
directed medical therapy. CRT is indicated in patients with
an ejection fraction of, 35% or less, New York Heart Asso
ciation (NYHA) functional class ll to lV symptoms despite
guideline directed medical therapy, sinus rhythm, and
Ieft bundle branch block (LBBB) with a QRS duration of
150 ms or longer (class 1 recommendation). For patients
with LBBB and QRS duration of 120 to 149 ms, CRT can be
useful and should be considered (class 2a recommenda
tion). A recent echocardiogram shows that this patient's
ejection fraction is still less than 35')(,, and his ECG shows
LBBB; therefore, it would be appropriate to place a biven
tricular pacemaker (which will improve ejection fraction)
with a cardioverter deflbrillator. This therapy would give
him a survival benefit over continued medical therapy and
improve his functional capacity.
Implantable cardioverler defibrillators (lCDs) (Option B)
have also been shown to provide a sun,ival benefit in patients
with symptomatic heart failure and an ejection fraction less
than 35',1,. In contrast to CRT. ICDs have no eflect on either
ejection lraction or functional capacity.
Implantabie pulmonary ar1ery presswe sensors (Option C)
are placed in the distal pulmonary artery and are used
to remotely monitor pulmonary hemodynamics. In the
CHAMPION trial. the use of these sensors was shown to
reduce hearl failure hospitalizations in patients with NYHA
class III s),mptoms compared with patients in the control
arm. These monitors are often placed in patients who have
frequer.rt hospitalizations to more closely monitor their heart
failure hemodynamics, but they have no efl'ect on ejection
fractior.r. Despite significant ongoing symptoms, this patient
has not had frequent hospitalizations and would not be a
candidate fbr this device.
A randomized controiled trial showed that a wearable
cardioverter defibrillator (Option D) after acute myocar
dial inlarction did not reduce the incidence of sudden
cardiac death but did reduce the secondary outcome of
all cause mortality. There are no guideline recommenda
tions on the use of a wearable cardioverter.defibrillator
in patients with heart failure, but this device might be
an option for patients at high risk for arrhythmias as a
bridge to ICD therapy. Unlike resynchronization therapy,
a wearable cardioverter defibrillator will not improve
this patient's ejection fraction.
I(EY POIl{T
o Cardiac resynchronization therapy is indicated in
patients with New York Heart Association functional
class II to IV symptoms despite guideline-directed
medical therapy who have an ejection fraction of 35'1,
or less, sinus rhythm, and left bundle branch block
with a QRS duration of 150 ms or longer.
Bibliography
Henin M. Ragr I [, Mannion J, et al. Indications ofcardiac resynchronization
in non left bundle branch block: clinical review of available evidence.
Cardiol Res. 2020;ll:l 8. [PMlDr 32095190] doi:10.1,1740/cr989
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208. Answers and Critiques
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Item 78 Answer: B
Educational Objective: Treat aortic dissection with
emergent surgery.
The most appropriate next stcp in managenlent is emer
gent surgery (Option B), inclucling surgical aortic dissec
tion repair and aortic valve replacement. Causes of acute
aortic regurgitation it.tclude er.rdocarditis. blunt trauma
to the chest. iatrogenic causes (such as cornplicatiot.ts
of balloon aortic valvuloplastl'). and aortic dissection.
Patients with acute aortic dissection rt,ithout evidence
of cardiogcnic sl-rock or acutc aortic regurgitation sl-rould
be treated rt,ith medicaI tl.rcrapy'to control heart rate and
reduce blood pressure. Current guidelines recommend
reducing systolic blood pressure to 120 mnt IIg or less
in the lirst hour in patier.rts r,r,ith aortic clisscctior.r. Pain
control is oflen necessary and is best accornplished
"t'ith
intravenous opioids. Acute aortic regurgilation due to
aortic dissection is a surgical emergency and is espe
cially urgent in the prescnce of hvpotension. pulmonarl'
edema. or cardiogenic shock: it generalll' requires aortic
valve repair or replacement, even for moclcrate aortic
regurgitdtion.
Cardiac magnetic resonance imagir.rg (Option A) may
be used k) quantitate tl.re degree of aortic regllrgitation
and to diagnose aortic dissection: hou'evcr, in tl.ris case,
further quantitation of aurtic regurgitation ancl diagnosis
ofaortic dissection are unnecessarl,becanse the CT angio-
grarn and echocardiogram have already established type
A dissection rt"ith involvement of the aortic valve (aortic
regurgitation).
Intra aortic balloon counterpulsation (Option C) is
contraindic:lted in patients \,ith acute severe iiortic regur-
gitation because inflation of the balloor-r in cliastole r.r'ould
worsen thc rcgurgitation severil).
'l'ransesophageal
echocardiography ('ltiD) (Option D)
also may be used to qualitatively and quantitatively evaluate
aortic regurgitation. but in cases of native valvc iiortic regur
gitation. it typically does not provide additional inforntation
beyoncl that provided by transthoracic echocardiographl:
TEE r,toulcl be usef'ul if CT lr.rgiography lr,erc unavailable. In
that case,
'l'Flti
could providc inlormation on both the aortic
valve and the ascending aorta.
'lEE
is also useful in intraop
eratirre assessment of aortic valr,e function befbrc and after
the surgical intenention.
rtY P0ttT
. In patients with acute aortic regurgitation due to aortic
dissection, emergent surgery including aortic valve
replacement or repair and aortic dissection repair, is
indicated.
Bibliography
Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the
management of patients with vahular heart disease: a report of the
American College of Cardiolos//American Heart Association Joint
Committee on Clinical Practice Guidelines. Circulation. 2O2t;743:e72-
e227. IPMID: 33332150] doi:1O.1161/CIR.O0OO00OOOOOO0923
Item 79 Answer: B
Educational Objective: Treat a patient with diabetes
mellitus and atherosclerotic cardiovascular disease with a
glucagon-like peptide I receptor agonist.
The most appropriate additional treatment is liraglutide
(Option B). The 2021 Standards of Medical Care in Diabe-
tes from the American Diabetes Association, endorsed by
the American College of Cardiologr, recommends either a
sodium-glucose cotransporter 2 (SGLT2) inhibitor or a
glucagon-like peptide 1 (GLP-1) receptor agonist in patients
with type 2 diabetes mellitus who have established atheroscle
rotic cardiovascular disease, as part ofcomprehensive cardio-
vascular risk reduction and/or the glucose lowering regimen.
In this population, both drugs are associated with reduced
rates ofadverse cardiovascular events, including stroke, myo
cardial infarction, and cardiovascular death. In this patient
with obesity, the GLP 1 receptor agonist liraglutide may be
preferred to an SGUI2 inhibitor because it is associated with
weight loss. Furthermore, the hypoglycemic effect of SGLI2
inhibitors is diminished if the estimated glomerular flltration
rate is less than 45 ml/min/1.73 m2, as it is in this patient.
Long-term treatment with dual antiplatelet therapy,
such as aspirin and clopidogrel (Option A), should be con
sidered for patients with prior coronary intervention, high
ischemic risk, and low bleeding risk to prevent major adverse
cardiovascular events. However, this patient is already taking
rivaroxaban and aspirin. Combination therapy with aspirin
plus low-dose rivaroxaban should be considered in patients
with stable coronary and/or peripheral artery disease and
low bleeding risk to prevent major adverse limb and cardio
vascular events. The addition of clopidogrel to this regimen
(triple antithrombotic therapy) is generally avoided because
of the increased risk for bleeding.
Statin plus niacin (Option C) combination therapy has
not been shown to provide additional cardiovascular beneflt
above statin therapy alone, may increase side effects, and is
generally not recommended.
Pramlintide (Option D), an injectable agent used to
treat diabetes, is an amylin mimetic that slows gastric emp
tying, suppresses glucagon secretion, and increases satiety.
It is associated with weight loss but has no known effects on
the incidence of cardiovascular events, including myocardial
infarction, stroke, or cardiovascular death, and would not be
the best choice for this high risk patient.
rEY POIXT
o In patients with type 2 diabetes mellitus, sodium-
glucose cotransporter 2 inhibitors and glucagon-like
peptide 1 receptor agonists are associated with reduced
rates of adverse cardiovascular events, including stroke,
myocardial infarction, and cardiovascular death, com-
pared with placebo.
Bibliography
American Diabetes Association. 10. Cardiovascular disease and risk man
agement: standards of medical care in diabetes-2o2l. Diabetes Care.
2O21;44:5125 S150. IPMID: 33298421) doi:10.2337ldc21-S01O
)
196

209. Answers and Critiques
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Item 80 Answer: D
Educational Objective:
'l'reat
left atrial myxoma.
'ilre
most appropriilte managcnlent is rlrgent sr_rrgical exci
sion (Option D).
'lhis
paticnt's ecl.rocirrcliograrn demor.r
strates a large left atrial ntass consistcnt r,r,ith ntyxonra,
with attachment by a stalk to the intcratrial scptum. t,elt
rtrial myxon-ras tnost cornntonly occur in rniddle agcd
persolls and are more frc'clucnt in uonlen.
'll-rcy
ntay prc
scnt rvitl.r valvular occlusivc symptolls, such as dyspnct
irnd syncope, or with ernbolic phenomena, including
stroke or transient ischentic events. Atrial myxomas ntily
produce cytclkines that result in constitutional symptortrs.
such as fever. anorexia, ancl weight loss, which are pre
sent in up to one third of patients. Central ner\ous systcltl
embolic cvents shoulcl herald urgent cirrdiac sirrgical eval
Llation xnd excision in paticnts with acceptaltle surgical
risk. Because of' the possibility of' recurrence. l<tr.rgitudinal
screening \^,ith echocardiogrrrphy is appropriate.
Anticoagulation with a direct rtral anticoagulant
(Option A) or vitan.rin K ar.rtagonist would be appropriatc
fbr prevcntion or treatment of thrombus of thc lcft atriultl
or lell atrial appendage. I lowever, the rounded nature
of this nrass and attachment [o the interatrial septulx is
ry'pical firr mvxor.na. Lelt atrial or left atrill appendage
thrombus would be more likely ir.r pltients with under
lying atrial fibrillation, mitral stenosis, or :r mitral valvc
prosthesis with abnormal lur.rction. none of rvhich is pre
scnt in this patient.
'lhcrc is no indicati<ln fbr thrornbolytic therapy
(Option B) in this patient r,r,hose neurologic svmptoms
clemonstrated complete and rapid resolution, and thc
administration of'thrombolytic therapy ir-r such a patielrt
n.ray be harmful.
Bloocl cultures and erxpiric antibiotic therapy (Option C)
\'voulcl bc considcrcd fbr suspectecl infective endocardi
tis. Fkrwever, lhis prtient's mass docs not appear to be
clirectll, iissociatecl with thc valve in its attachrnent. and it
nould be unusually large fbr a vegetition. In adclitiot-t. this
tl:rtient has no ur.rclcrtying risk factors lilr enciocarditis rtrd
hirs not undergone a recent procedure likely to etrgender
bacterenr ia.
XEY POTIITS
. Patients with a myxoma may present with valvular
occlusive symptoms, such as dyspnea and syncope;
embolic phenomena, including stroke or transient
ischemic events; and/or constitutional symptoms,
such as fever, anorexia, and weight loss.
. In patients with atrial myxoma and a central nervous
system embolic event, urgent cardiac surgical evalua-
tion and excision are indicated.
Bibliography
Wang Z, Chen S, Zhu M, et al. Risk prediction fbr emboli and recurrence of
primary cardiac m,r(omas after resection. J Cardiothorac Surg. 2016ill:
22. [PMID: 268328O6] doi:10.1186/s13019 016 O42O-4
Item 81 Answer: C
Educational Objective: Treat a symptomatic patient
with transient heart block following an inferior myocardial
infarction.
'l
he most appropriate trcatment tbr this patient is temporary
pacing (Option C). Gtnduction al)normalitics are cornntonly
idcntified in the settillg ol acute rnyocardial infarction (Ml) and
irrc marutgccl on the basis of'the type oi block and the location
ot nryocardial injury Symptomatic patients with inferior MI and
transic'nt heirft block rnty be trcated lr,ith tcr.nporury, pacing
beciluse concluction block in this setting, including Mobitz t),pe
1 irncl complcte hearl block, is causcd by high vagal tone affect
iug the atrioventricular (AV) node. is genemlly transient, and is
accompanic'cl b1'an adcquate escapc rhlthrn. Whereas tentpo
r-irry pacing support may occasionally be neccssary pcrmirnent
pacing (Option D) is rarely indicatect. Vagally mediatcd hearl
block occur-ring at the AV node ntust be distinguishecl fiom the
less In-rigr and potentially lethal Mobiz type 2 seconci degree
AV block, which occurs more fieclnently in the settir.rg of'ex1en.
sire anterior M I and danrage to the c<tnduction system bclow the
AV nocle. Ahhough Mobitz tlpe 1 bl<rck occurs at the AV node ar-rd
is iclcntified by cyclical. repetitive prolongatirin of the PR ir.rterval
Ieacting to a "dropped beat" ancl a regularly irregular rlrythm.
Mubitz tlpe 2 btock is typified by block conduction r,vithout
associated PR prolongation and nray preclispose to extendecl
periods of asysklle, requiring permanent pacemaker support.
Atropine (Option A) may ir.npr<)ve AV noclalconduction ancl
incrcase the sinus rate througl-r vagolytic activity. Hove\er, it pro
vicles only lcrxporary benefit and is neither necessary nor sufti-
cient as treatnrent for asymptomatic heart bkrck in this setting.
Although unfiactior.rated heparin (Option B) and other
intravenous antithronlbin therapy would bc indicated in the
presence of' thrombotic con-rplications or atrial librillation,
it is not irrdicated roLltinely after successtul primary percu
tancous coronary inlervention rir lor treatment of irregular
rhytl.rms associated with heart block. However. dual anti
platelet thcrapy is indicated.
KEY POITT
. Symptomatic patients with transient heart block,
including Mobitz type t heart block and complete
heart block, after inferior myocardial infarction may
be treated with temporary pacing.
Bibliography
O'Gara PT, Kushner FG, Ascheim DD, et al. 2013 ACCF/AHA guideline for the
management of ST elevation myocardial infarction: a report of the
American College of Cardiolory Foundation/American Heart Association
Task Force on Practice Guidelines. J Am Coll Cardiol. 2013;61:e78 e14o.
IPMID: 232569141 doi:10.1016/j.jacc.2012.11.019
Itenn 82 Answer: A
Educational Objective: Diagnose heart failure with
preserved ejection fraction.
The most likely diagnosis is heart failure with preserved ejec-
tion fraction (HFpEF) (Option A). The diagnosis of HFpEF
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should be suspected in patients who meet the following
three criteria: symptoms of heart failure, left ventricular
(LV) ejection fraction of50'/, or greatet and no other appar
ent cause of heart failure symptoms. I{FpEF accounts tbr
approximately half of heart failure cases. Classic symptoms
include exertional dyspnea, paroxysmal nocturnal dyspnea,
and orthopnea. Physical flndings in heart failure. such as:rn
S.,, elevated central venous pressure, crackles. and peripheral
edema. are highly speciflc but insensitive, and their absence
does not exclude heart failure. Natriuretic peptide levels
may be normal in patients with heart failure. particularly
in those with obesity or only exertional symptoms. Echo
cardiographic features of HFpEF include normal LV cavity
size, increased LV wall thickness, left atrial enlargement.
abnormal diastolic function. and elevated pulmonary artery
systolic pressure (>35 mm Hg).
High output heart failure (Option B) is characterized by
symptoms of heart failure in the setting of a cardiac index
greater than 4 L/min/m2. Causes include obesity, anemia,
hyperthyroidism, Paget disease of bone, thiamine deficiency,
and arteriovenous fistula. The patient's normal cardiac index
rules out this diagnosis.
Hypertrophic obstructive cardiomyopathy (Option C)
may cause dyspnea; however, a systolic murmur would
be present on examination, and the echocardiogran.r
would demonstrate resting or provoked outllow tract
obstruction.
The patient's t.V hypertrophy and elevated right
ventricular systolic pressure make noncardiac dyspnea
(Option D) less likely. Predictive scores can exclude non
cardiac causes of' dyspnea with a high degree of reli
ability. The Heart Failure Preserved Ejection Fraction
(HrFpEF) risk score, which relies on simple clinical and
echocardiographic characteristics, is a means to assess
the likelihood of HFpEF and is used to discriminate
cardiac versus noncardiac causes of dyspnea. Predictive
variables include obesity (2 points), atrial fibrillation
(3 points), age older than 60 years (l point), treatment
with at least two antihypertensive drugs (1 point), echo
cardiographic E/e' ratio greater than 9 (1 point), and
echocardiographic pulmonary artery systolic pressure
greater than 35 mm Hg (1 point). This patient's HTFpEF
risk score is B, suggesting that her dyspnea has a cardiac
cause and that further diagnostic evaluation of dyspnea
is unnecessary.
f,tY POIilT
o Heart failure with preserved ejection fraction should
be suspected in patients who meet the following three
criteria: symptoms of heart failure, left ventricular
ejection fraction of 50'7, or greater, and no other
apparent cause of heart failure symptoms.
Bibliography
Rcddy YN\,i Crrter RI.l. Obokata M, et al. A sinrple. o,idcnce basecl
approach to help guide diagnosis of heart fai lurc with presrnr'ed eject iotl
fraction. Circulation. 2018;138:u6t 70. IPMll): 297g22gql doi:lO.l t(>1,'
ClRCUI.r\TIONAHA. I t8.03,16.16
Item 83 Answer: D
Educational Objective: Prevent stroke in a patient with
incidentally diagnosed atrial fibrillation.
The most appropriate management is to initiate oral antico-
agulation (Option D). This patient likely has persistent, and
possibly symptomatic, atrial fibrillation (AF). AF is usually
the result of long standing risk factors, such as diabetes mel
litus, obesity. hypertension, coronary artery disease, heart
failure, and obstructive sleep apnea. In stable patients with
AF, the primary goals of therapy are to (1) prevent stroke.
(2) control heart rate, (3) minimize or eliminate symptoms,
and (4) modify underlying risk factors. The 2019 American
College of Cardiologr/American Heart Association/Heart
Rhythm Society focused update on AF recommends antico
agulation to prevent stroke in patients with nonvalvular AF
who have a CHATDST-VASc score of 2 or greater in men or 3
or greater in women. In this patient with a CHATDS, VASc
score of 4 (age >75 years [2 pointsl, female [1 pointl, hyper-
tension [f pointl), consideration oforal anticoagulation for
stroke prevention is warranted. Aspirin alone (Option A) is
insufflcient for stroke prevention in this patient at high risk
for stroke.
In addition, given this patient's exertional limitation,
her AF may be symptomatic, and rhythm control, with
appropriate stroke prevention, should be considered as a
means of improving her fatigue. Lastly, her loud snoring may
suggest underlying obstructive sleep apnea, which may be
driving the AF, and she should be asked about symptoms of
excessive daytime sleepiness.
This patient does not require emergent cardioversion
(Option B), as she is not hemodynamically unstable, has a
well controlled heart rate, and has no signs of heart failure.
Increasing the metoprolol dosage (Option C) is not indi
cated in this patient with a well-controlled heart rate and
normal blood pressure. Increasing the metoprolol dosage
also may worsen her flatigue.
Reassurance (Option E) is not appropriate management
because this patient's stroke risk, possible symptoms of AF,
and possible sleep apnea should be addressed.
rEY POIilIS
o In stable patients with atrial fibrillation, the primary
goals oftherapy are to (1) prevent stroke, (2) control
heart rate, (3) minimize or eliminate symptoms, and
(4) modify underlying risk factors.
. Anticoagulation is indicated for stroke prevention in
patients with nonvalvular atrial fibrillation who have
a CHATDST-VASc score of 2 or greater in men or 3 or
greater in women.
Bibliography
January CT. \Urnn LS. Calkins H. et al. 2019 AHr A(-(l I IRS focused r-rpdate
ol the 201.1 AIIi ACC IIRS guideline ti)r the nttnagement ol patients
with atrial fibrillation: a report ol tlte American College of Oanliologl
American Heart Association Task Rrrce on Clinical Practice Guidelines
rnd the Ilcart Rhytlrm Society. J Ant Coll (lardiol.2019;71:104
132.
I PMID: 30703 13ll doi:10. l0l6 j.jacc.2019.01.011
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211. Answers and Critiques
Item 84 Answer: A
Educational Objective: Assess the severity of mitral
regurgitation with cardiac magnetic resonance imaging.
The most appropriate next step in management is cardiac
magnetic resonance (CMR) imaging (OptionA). The patient's
history and examination findings are consistent with heart
failure and volume overload (dyspnea, orthopnea, S.,
elevated central venous pressure, puimonary crackles, pul-
monary edema on the chest radiograph) despite guideline-
directed medical therapy. The systolic click and radiation of
the murmur to the back suggest posteriorly directed mitral
regurgitation (MR) with prolapse (primary MR). Despite
these flndings, the quantitative echocardiographic data are
consistent with moderate MR. However. two dimensional
quantitative echocardiographic data may underestimate the
severity of MR. In that case, additional imaging is required.
In patients with primary MR, iltransthoracic echocardiogra-
phy provides insufficient or discordant information, current
guidelines recommend either transesophageal echocardiog
raphy or CMR imaging to assess the severity of MR (class
1
recommendations). This assessment is important in deter
mining whether mitral valve intervention is indicated. Surgi
cal and transcatheter interventions are performed primarily
in patients with severe valvular heart disease, but diagnosis,
patient education, periodic monitoring, and medical therapy
are essential elements in the management of patients with
mild to moderate val'ue dyslunction.
ln symptomatic patients with confirmed mild to mod
erate MR, starting guideline directed medical therapy with
periodic imaging surveillance is appropriate. Repeat echo-
cardiographic imaging is recommended every 1 to 2 years
(Option B) in patients with moderate primary MR. How-
ever, the severity of this patient's symptoms is at odds with
the echocardiographic flndings, and additional imaging is
required to establish the degree of valve dysfunction and
select the appropriate treatment.
Neither surgical (Option C) nor transcatheter (Option D)
mitral valve repair is appropriate before confirming the
severity of MR. Documentation of severe MR would be an
indication fbr surgical mitral valve repair. ln patients with
high or prohibitive surgical risk, transcatheter mitral valve
repair (transcatheter edge to edge repair) is reasonable.
KEY POII{T
o For patients in whom there is a discrepancy between
physical examination flndings and transthoracic echo
cardiographic assessment of mitral regurgitation severity,
cardiac magnetic resonance imaging or transesophageal
echocardiography should be used to quantitatively
assess mitral regurgitation and resolve the discrepancy.
Bibliography
tsonow RO, o'(;Nra Pl'. Adams DH. et al. 2020 Focused Llpdrte of the 2017
ACC exper( consensus decision pathway on the manrgcment of mitral
regurgitNtion: J report ofthe American College ol Carditllol5, Solution Set
Oversight Committee. J Am Qrll Crrdiol. 2020;75:22116 2270. [PMID:
3206808.1 | doi: I 0.r 016 ',j.jacc.2020.o2.005
Item 85 Answer: A
Educational Objective: Evaluate for coronary artery
disease in a patient with new-onset heart failure.
Cardiac catheterization (Option A) is the most appropriate
test to perform in this patient with new onset heart failure
ofunclear cause. Echocardiography should be performed ini
tially in all patients with new heart failure; this patient's echo
cardiogram is notable for a low ejection fraction, anterior wall
hypokinesis, and absence of left ventricular (LV) hlpertrophy.
The anterior wall motion abnormality increases suspicion fbr
coronary artery disease (CAD), the most common underlying
cause of heart failure. The patient also has exertional chest
heaviness; thus, clinical suspicion tbr CAD should be high.
Cardiac catheterization offers the most direct information
about coronary anatomy and therefore is the best method fbr
assessing ischemia in such patients. If the patient has CAD,
revascularization has been associated with improved survival
rates and often with improved ejection fiaction.
Cardiac magnetic resonance (CMR) imaging (Option B)
wtiuld be reasonable to perfbrm afterechocardiography and after
CAD has been excluded. CMR imaging can help identi! infiltra-
tive processes, such as sarcoidosis, hemochromatosis, amyloido-
sis, I.-abry disease, or endomyocardial fibrosis, and has distinct
imaging pattems that can suggest the diagnosis of myocarditis.
CMR imaging is also becoming more widely used to evaluate fbr
ischemia and hibernating myocardium, but guidelines recom
mend either stress testing or cardiac catheterization as the first
study in the evaluation of possible ischemic cardiomyopathy.
Cardiac PET (Option C) can aid in assessing LV function
and evaluating for ischemia. It may help distinguish between
inflammation and fibrosis and therefbre can guide decisions
about therapy. However, given this patient's high likelihood
of CAD, cardiac catheterization is the most direct and apprcr
priate modality to assess for CAD.
Cardiac amyloidosis is suggested by heart failure with
restrictive physiologi, increased wall thickness, and low
ECG voltage. Technetium-99m pyrophosphate scintigraphy
(Option D) is used to diagnose the transthyretin variant of
cardiac amyloidcisis. This patient's lack ol LV hypertrriphy
on echocardiogram and anterior hypokinesis argue against
the diagnosis of amyloidosis, an infiltrative cardiomyopathy.
KEY POITI
. Patients with new-onset heart failure and a high like-
lihood ofcoronary artery disease should be evaluated
with cardiac catheterization.
Bibliography
Wu A. I leart failure. Ann Intcrn Med. 20l8r168:l lC8l I lC96. [PMID: 2986tt8161
rloi:10.7326 1AITC20l 806050
Item 86 Answer: E
Educational Objective: Treat a patient with intermittent
claudication with supervised exercise training.
The most appropriate treatment for this patient with symp
tomatic peripheral artery disease (PAD) is to start a supervised
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exercise program (Option E), regardless ofthe ankle brachial
index value. In patients with intermittent claudication, super-
vised exercise training is recommended to improve functional
status (maximal walking distance and pain-free walking
distance) and qualiff of life and to reduce leg symptoms.
Studies with intermediate and long term follow-up also have
demonstrated a persistent beneflt of supervised exercise in
these patients. The risk benefit ratio for supervised exercise is
favorable for patients with PAD (including those with comor
bid conditions), and training is recommended for a minimum
of 30 to 45 minutes, at least three times weekly for a minimum
of 12 weeks. The Centers for Medicare & Medicaid Services
provides reimbursement for supervised exercise programs in
patients with symptomatic PAD. Cilostazol, a phosphodies-
terase inhibitor, is also recommended to improve symptoms
and increase walking distance in patients with claudication.
Noninvasive imagrng, such as CT angiography (Option A),
magnetic resonance angiography, or duplex ultrasonogra
phy, should be reserved for patients being considered for
revascularization. In this patient, guideline directed medical
therapy and a supervised exercise program should be started
before consideration of CT angiography.
Chelation therapy with ethylenediaminetetraacetic acid
(Option B) is not beneflcial for treatment of claudication
(American Heart Association/American College of Cardiol
ogr class 3 recommendation). A Cochrane systematic review
and meta-analysis concluded that there was no signiflcant
difference in maximal and pain-free walking distance with
chelation therapy versus placebo.
Pentoxifylline (Option C) has no beneflt for the treat-
ment of symptomatic PAD (class 3 recommendation) and
should not be prescribed to this patient.
Although this patient has progressive symptoms, revas
cularization (Option D) is not appropriate at this time.
Patients with intermittent claudication should be started
on a supervised exercise program and medical therapy (e.g.,
cilostazol, vascular disease risk reduction) before referral for
revascularization. If symptoms persist despite these inter-
ventions, counseling about the risks and beneflts ofrevascu,
larization (endovascular or surgical) is appropriate.
I(EY POIilT5
. In patients with peripheral artery disease and inter-
mittent claudication, supervised exercise training is
the most effective treatment to improve maximal
walking distance and pain free walking distance.
o Cilostazol, a phosphodiesterase inhibitor, is recom-
mended in addition to a supervised exercise program
to improve symptoms and increase walking distance
in patients with claudication.
Bibliography
Gerhard-Heman MD, Gomik HL, Barrett C, et al. 2016 AHA/ACC guideline on
the management of patients with lower extremity peripheral artery disease:
executive summary: a report of the American College of Cardiologr/
American Heart Association Task Force on Clinical
praclice
Guidelines.
Circulation. 2017;135:e686 e725. IPMID: 27540382) doi:10.1161/CIR.
0000000000000470
Item 87 Answer: C
Educational Objective: Manage a patient with Marfan
syndrome and aortic root dilation who is considering
pregnancy.
Aortic repair before pregnancy (Option C) is the most
appropriate management option for this patient. All
women with Marfan syndrome have an increased risk for
pregnancy-related aortic dissection and rupture. To reduce
this risk, aortic repair surgery is recommended before con-
ception for women with Marfan syndrome who have an
ascending aortic diameter greater than 4.0 cm and risk
factors for aortic dissection, such as a family or personal
history of aortic dissection or aortic dilatation of more
than 5 mm per year. Pregnancy is considered Iow risk if
the aortic diameter is smaller than 4.0 cm. This patient has
both a family history of aortic dissection and evidence of
an expanding aneurysm; aortic repair before attempting
pregnancy should be advised.
There is limited information and no randomized con-
trolled trials on the clinical outcomes of statin therapy, such
as atorvastatin (Option A), in slowing the expansion of the
aortic root in patients with aortic or thoracic aneurysm.
In addition, statin therapy is contraindicated in patients
who wish to become pregnant or are pregnant. Population
studies suggest that there may be an increase in congen
ital central nervous system and limb abnormalities with
exposure to lipophilic statins during the flrst trimester.
Starting atorvastatin and continuing with pregnancy is not
recommended.
Angiotensin receptor blockers, such as losartan (Option B),
are contraindicated during pregnancy because of fetal toxic-
ity. Exposure to these agents during the flrst trimester may
cause central nervous system and cardiovascular malfor
mations; exposure during the second trimester may lead to
urogenital and renal developmental malformations. These
agents are often used in nonpregnant patients with Marfan
syndrome as alternatives to B-blocker therapy for slowing
aortic root expansion.
The size of this patient's aortic root in the context of
the patient's family history of aortic dissection and the rate
of aortic root expansion are all factors that support aortic
repair prior to pregnancy. Proceeding directly to pregnancy
(Option D) is not advisable.
I(EY POIlIT
o Women with Marfan syndrome have an increased risk
for pregnancy-related aortic dissection and rupture;
aortic repair surgery is recommended before concep
tion to reduce this risk in those with an ascending
aortic diameter greater than 4.0 cm and who have
risk factors for aortic dissection.
Bibliography
Regitz Zagrosek V Roos Hesselink IW, Bauersachs J, et al; ESC Scientific
Document Croup. 2018 ESC guidelines fbr the management ofcardiovas
cular diseases during pregnancy. Eur Heart J. ZO1S;S9,gtOS-aZ+1. [pMlD:
301655441 doi:10.1093/eurheartj/ehy340
200

213. Answers and Critiques
Item 88 Answer: C
Educational Objective: Evaluate atypical angina with
pharmacologic myocardial perfusion imaglng.
Pharmacologic myocardial perfusion imaging (Option C)
is the most appropriate next test. The evaluation of angina
should include a physical examination to detect conditions
that may mimic symptomatic coronary artery disease (CAD),
as well as a focused history to assist in determining the
pretest probabiliff of CAD. A resting ECG is required to
evaluate for ongoing ischemic insult and to guide the choice
of subsequent diagnostic testing. This 66-year-old woman
with clinical features of atlpical angina has an intermedi-
ate pretest likelihood of CAD, an indication for stress test
diagnostic evaluation. Stress testing with imaging is indi-
cated in patients with an inability to exercise, baseline ECG
abnormalities (such as left bundle branch block [LBBB]) that
limit interpretation of the exercise ECG, or indeterminate
flndings on the exercise ECG. In patients able to exercise but
with underlying LBBB, such as this patient, pharmacologic
stress testing with imaging (myocardial perfusion, cardiac
magnetic resonance, or echocardiographic) is appropriate to
reduce the rate of false positive test results associated with
exercise ECG (Option B) in this population.
Coronary angiography (Option A) is not indicated for
stable, low-risk clinical features. It should be reserved for
patients with a high pretest likelihood of CAD and unstable
symptoms, or for those in whom optimal medical therapy
has been ineffective.
Resting transthoracic echocardiography (Option D) can
provide helpful prognostic information related to ventricular
function and is usefui in assessing for noncoronary cardiac
causes of chest pain. It is not as helpful as exercise stress
testing as an initial test for symptomatic CAD.
Reassurance without additional testing (Option E) may
be appropriate when an alternative cause of chest discomfort
has been identifled in a patient with a low pretest probability
of CAD, but this patient's ongoing exertional chest pain and
intermediate likelihood of CAD require further investigation.
I(EY POITI
o Stress testing with imaging is indicated in patients
with an inability to exercise, baseline ECG abnormali-
ties that limit interpretation of the exercise ECG, or
indeterminate findings on the exercise ECG.
Bibliography
Katz D, Gavin MC. Stable ischemic heart disease. Ann Intern Med. 2019;
171:lTC17lTC32. [PMID: 31382288] doi:10.7326/AITC201908060
Item 89 Answer: A
Educational Objective: Evaluate a patient with ventric-
ular tachycardia.
The most appropriate initial management is an evaluation
lbr structural heart disease r.tith cardiac magnetic reso
nance (CMR) imaging (Option A). The presence or absence
of structural heart disease is an impofiant distinction in the
nlarlagenlent of'ventricular tachycardia (VT). CMR imag
ing generally allows for tissue characterization, rnaking it
an important method fbr evaluating myocardial diseases
that may manifest as Vl'. including arrhythmogenic right
ventricular cardiomyopathy, cardiac sarcoidosis, and other
infiltrative cardiornyrpathies (e.g., amyloidosis).
Fllectrophysiologic testing (Option B) is not indicated
as part of the initial management ol this patient. It may be
uselul to establisl.r the diagnosis of sustained monomor
phic tachycardia if the diagnosis is in doubt. It is similarly
useful to establish the mechanism of VT and to identify the
krcation of the arrhythmogenic lbcus if ablation is being
considered. However, the initial management should fbcus
on (1) assessment fbr structural heart disease and (2) iden
tification of correctable causes of V'1. including myocarclial
ischemia, heart failure, drug effects, anemia, and electrolyte
abnormalities.
It is premature to consider an implantable cardioverter
defibrillakir (lCD) (Option C) fbr this patient. lf CMR imag
ing demonstrates significant structural heart disease, lCIf
placement or additional testing may be warranted before
discharge. An ICD is indicated for secondary preventittn of
sudden cardiac death as well as for primary prevention ol
sudden cardiac death in patients with an ejection fraction
of 35'/, or less and New York Heart Asstlciation functional
class II or III hearl failure symptoms while receiving guide
line directed medical therapy. ICD placernent is generally
unnecessary in idiopathic V'f, owing to the benign prognrtsis
and high eflicacy ol'other therapies.
lf the patient has idiopathic V'l', which is Vf in the
absence of structural heart disease, an implantable loop
recorder (ll-R) (Option D) may be appropriate. Idiopathic
VT typically n-ianifests as palpitations in the third to fifth
decades ol lif'e, otten triggered by stress, emotion, or
sleeplessness. It would be premature to consicier ll,R
placement befbre assessment for structural heart disease.
An ILR rn:ry be valuable to assess rare recurrences o['
arrhytl-rmia and/or to assess symptoms, but further eval
uation is needed first.
f,EY POIilTS
o The initial evaluation ofventricular tachycardia focuses
on the identification of reversible causes and includes
echocardiography, cardiac magnetic resonance imaging,
and exercise ECG.
r Idiopathic ventricular tachycardia occurs in the absence
of structural heart disease, $pically
manifesting as
palpitations in the third to fifth decades of life, often
triggered by stress, emotion, or sleeplessness.
Bibliography
Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS
guideline for management of patients with ventricular arrhlthmias and
ihe prevention ofsudden cardiac death: a report ofthe American College
of CardioloryiAmerican Heart Association Task Force on Clinical Practice
Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2018;72re91
e220. [PMID: 29097296] doi:10.1016/j.jacc.20i7.10.054
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Item 90 Answer: B
Ed u cationa I O bj ective : Diagnose low-fl ow low- gradient
severe symptomatic aortic stenosis.
The most appropriate next step in management is dobu
tamine stress echocardiography (Option B).
-lhis
patient's
examination findings (late-peaking crescendo decrescendo
murmur at the right upper sternal border, crackles, and
elevated central venous pressure) and elevated B type natri
uretic peptide level are consistent with signiflcant aortic ste
nosis and associated volume overload. On echocardiography,
low-flow low gradient severe aortic stenosis is suggested
by a severely reduced valve area (<1.0 cm2) and a modestly
increased peak aortic velocity (<+ m/s) in the setting of a
reduced ejection fraction (<sO'2,). The most appropriate next
step in management is to confirm the diagnosis of severe
aortic stenosis with low-dose dobutamine stress echocar
diography. If dobutamine infusion results in an increase in
stroke volume by at least 20')(,, severe aortic stenosis is diag
nosed by an increase in peak velocity to 4.0 m/s or greater
with the valve area remaining 1.0 cm2 or less. ln patients
with suspected low flow low gradient severe aortic stenosis
with normal or reduced left ventricular ejection fraction,
measurement of aortic valve calcium score by CT is also rea-
sonable to further deflne aortic stenosis severity.
In this patient who likely has low cardiac output, direct
measurement of the aortic valve gradient by cardiac cath-
eterization (Option A) would result in iow gradients that
underestimate the severity of the aortic stenosis, similar to
gradient and velocity values on resting echocardiography.
Dobutamine catheterization may be performed in lieu of
dobutamine echocardiography in this case.
Proceeding to surgical (Option C) or transcatheter
aortic valve implantation (Option D) is inappropriate at
this timel the resting echocardiogram does not adequately
differentiate between pseudosevere aortic stenosis due to
insufflcient stroke volume and low flow Iow gradient severe
aortic stenosis.
l(tY P0tltT
. Dobutamine stress echocardiography can be used to
differentiate between pseudosevere aortic stenosis
and low-flow low-gradient severe aortic stenosis.
Bibliography
Ofto CM. Nishimura RA, Bonow RO. et al. 2020 ACC,,AI1A guideline for the
management of patients with valvular heart disease: a report of the
American College of Cardiologr' American Heart Association Joint
Committee on Clinical Practice Guidelines. Circulation. 2O21:143:e72-
e227. IPMI I): 333321501 doi:10.1161 /ClR.O00OOOOOOOOoO923
Item 91 Answer: B
Educational Objective: Treat thoracic aortic aneurysm
with aortic repair in a patient who requires open heart
surgery.
The most appropriate additional inten'ention to intprove
clinical orltcomes in this complex patient is aortic l,alrre
relrlacement and aortic repair (Option B). When patients
have an indication ttrr cardiac surgell' (such as coronary
artery bl.pass grafting [CABG]) and have concomitant aortic
stenosis and thoracic aofiic aneurysnr. the risks and bene
fits of aortic rah,'e replacement ancl lortic repair should be
considered. Aortic rcpair should be perfbrmed at the tirne
of cardiac surgery if' the ascenclinll aofta or aortic rorll is
llrgcr than .1.5 cnr. ln this patient r,r'ith a maxinral aortic
dimension of 5.6 cn.r. the annual risk tbr rllpture of the thcr
rxcic aofia is significant enough to refer l.rer lor aorlic repair.
'll.rc
additional risk associated with aortic val-ne replacement
cluring CABC is srnall, and replacenlellt nor,r'r.tould prevcnt
subsequent aortic valve clperations tbr symptomatic severe
aortic stenosis.
Performing CABG and aortic r,alve replacentcnt
(Option A) concurrently is appropriate in patients \'ith
three-r,essel coronary artery diselsc ((lAD) and moderate tc)
severe aortic stenosis. However. in the presence of thoracic
aortic aneurysnr with maxirnal aortic climension grcatcr
than 4.5 c111. aortic repair also shoulci lre performecl.
Although transcatheter aortic virlve irnplantation (Tl\'l)
(Option C) has beconle a conrnlon treiltlnent for patients
u'ith severe aortic stenosis. the proccdure is contraindicatecl
in tl.ris patient because of her conconritant CAD and thorrrcic
xortic aneurysnr. TAVI is often perfbrn.red in patients rvitlr
severe aortic stenosis and conconritar-rt CAD: hou'ever. the
coronary anatom)' typically should fhvor percutaneous cor
onarf intervention (rthich it does rlot in this case). ancl the
pr-ocedures shoulcl be performed in sequence.
Perforrning no rrclditional interyention (Option D) in
this patient with two surgically correct:rble life linritir-rg
lcsions rtho is lrlrcady nndergoing open heart surger-t' is
inappropriate.
'lhe
benefits of aortic r':rlve replacement rrr-rcl
aurtic repair u'ill n.rost likelv outucigl.r the harnts in this
pirtient.
I(EY POIilT
. In patients with an ascending aorta or aortic root
larger than 4.5 cm in diameter who require surgery
for coronary artery disease or valve pathologr, aortic
repair should be performed at the time of cardiac
surgery.
Bibliography
Su,erdlon NJ, Wu WW Schermerhom ML. Open and endovascular manage
ment of aortic aneurysms. Circ Res. 2019112,1:617 661. [PMID: 30761]2061
doi:10.1161 ClRCRESAHA.ll8.313186
Item 92 Answer: D
Educational Objective: Diagnose pulmonary regurgita-
tion in a patient with repaired tetralory of Fallot.
'I
he most likel1'cliirgnosis iu this prlticnt \,ith repaired tetral
ogy of Fallot ('l OIi) is pulmonary regLrrgitation (Option D).
'l'Ol
is characterized lry a large subaortic ventricular septal
defL'ct (VSD), intirr.rdibr-rlar and/or v:tlvular pulmonary ste
nosis. aortic ovcrriclc. ancl right ventricnlar \pertroph1,.
tr
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202

215. Answers and Critiques
tr
TOF replir involves VSD putch closure and relief'of pul
lnor1ary stenosis, right r,entricular ontflon tract <tbstruction
by transannular patch plirccr-nent.
'fhe
trans:rnnulirr patch
disrupts integrit),of the pulmor.rary ralve. resulting in severe
pulmonary regurgitation, which in turn caLlses right heart
eniargement, tricuspid rcgllrgitation, excrcise Iimitation,
and both atriirl and ventricular arrhythn.rias. l)uhnonary
regurgitation is the mctst contnton reason fbr reoperatior-r
after TOF repair. Severe pulntonary regurgitiition causes
a diastolic [lurmur heard at the left sternal burder that
increases in intensity with inspiration. Adclitional clinical
fbatures include a parasternal lift ancl a soft sysblic pulmo
nary outllor,r, lnurmur. A singlc S, is heard because pulmo-
nary rralve iutcgrity is disruptcd bt,the transannular p:rtch.
Aortic corrctation (Option A) is associated \{ith a s}-s-
tolic or continuous murntLtr hcard in the left infiaclarricular
region or over the back. A lrurrrlur fion.r collatcral intercos
tal vcsscls nray also be audible and palpablc over the chest
r'r,a1l. Filty percent of patients rvith aortic corrrctation hare
a bicuspicl aortic valve. Auscultation of the hcirrt mir1, rereal
an ejection click. a sl.stolic nrurnrur at the cardiac base. or.
sometinles, arr Sr. Tl-ris paticnt's murmur is not compat-
ible with coirrctation. and coarttation is not a long term
expectecl outcome forTOF repair.
In aortic regurgitation (Option B), :r cliastolic murmur
is generalll,heard at the lclt stcrnal borcler ancl cloes not
increase in inter-rsit1' $'itli inspiration. Right ventricular
prominence on physical exarrirldtioll would not be erpectecl
in a paticnt rvith aortic regurgitation.
The rumbling diastolic murmur of mitral stenosis
(Option C) is best hearci at thc apex and does not vary with
inspiration.
'lhe
murmur is typicalll,preceded b1'an opening
snap. N,{itral stenosis is not a recognized late con.rplication of
TOF repair.
IEY POIl{IT
o Severe pulmonary regurgitation causes a diastolic
murmur heard at the left sternal border that increases
in intensity with inspiration, a parasternal lift, and a
soft systolic pulmonary outflow murmur.
o Pulmonary regurgitation is the most common postop
erative sequela of tetralory of Fallot repair.
Bibliography
Stout KK. Daniels CJ. Aboulhosn JA. et al. 2018 AHA ACC guideline fbr the
management of adults with congenital heart disease: s report of the
American College of Cardiologr,rAmerican Heart Association Task Force
on Clinical Practice Guidelines. J Am Coll Cardiol. 2019r73:e81-e192.
IPMID: 301212391 doi:10.1016/j.jacc.2018.08.1029
Item 93 Answer: A
Educational Objective: Treat a patient with type 2 dia-
betes mellitus and atherosclerotic cardiovascular disease
with a sodium-glucose cotransporter 2 inhibitor.
The most appropriate management of this patient's athero-
sclerotic cardiovascular disease (ASCVD) is to begin treatment
with empagliflozin (Option A). Optimal medical therapy,
including
qqgressive
risk factor reduction and glucose control,
is fbundational for patients with diabetes mellitus to reduce
the risk for clinical ASCVD events. The American Diabetes
Association (ADA) and the American College of Cardiologz
recommend introducing a sodium-glucose cotransporter 2
(SGLI2) inhibitor or glucagon-like peptide 1 (GLP 1) receptor
agonist with demonstrated cardiovascular benefit as part of
a glycemic control regimen in patients with tlpe 2 diabetes
and clinical ASCVD. If the patient is already taking metformin
combined with another therapeutic agent or agents and is not
taking an SGLI2 inhibitor or GLP-I receptor agonist, the ADA
recommends considering switching to one of these agents,
which reduce the risk for ASCVD events regardless of the
hemoglobin A,. level.
'lhis
patient has high risk cardiovas
cular features given his diabetes and history ofnondisabling
stroke. Use of SGLI2 inhibitors in this population is associ
ated with a 14'1, reduction in cardiovascular death. Patients
with previous amputation, severe peripheral vascular disease,
neuropathy, or diabetic foot ulcers should exercise caution
with canagliflozin and ertugliflozin. It is unclear whether this
caution should be applied to all drugs in the class.
This patient's LDL cholesterol level is well controlled, and
the potential benefit of lowering LDL cholesterol levels below
this Ievel using ezetimibe (Option B) has not been shown.
Screening for asymptomatic coronary artery disease
in patients with diabetes is controversial. Without proven
outcome beneflt, routine stress testing with exercise ECG
(Option C) in asymptomatic individuals, such as this patient,
is not recommended.
No additional management (Option D) is inappropriate
for this patient at increased risk for additional ASCVD events.
Tight glycemic control reduces microvascular complications;
however, it does not reduce the risk fbr myocardial infarction
(MI). The use of SGLI2 inhibitors and GLP-I receptor agonists
in patients with type 2 diabetes has been shown to reduce
rates oflacute MI, stroke, and cardiovascular death. These ben-
efits seem to be unrelated to their glucose lowering eflects.
f,EY POIf,Is
. A sodium-glucose cotransporter 2 inhibitor or glucagon-
like peptide 1 receptor agonist with demonstrated car-
diovascular disease benefit should be included as part
of a g$cemic control regimen in patients with type 2
diabetes mellitus and clinical atherosclerotic cardio-
vascular disease.
. The benefits of sodium-glucose cotransporter 2 inhib-
itors or glucagon-like peptide 1 receptor agonists in
reducing atherosclerotic cardiovascular disease events
seem to be unrelated to their glucose-lowering effects.
Bibliography
I)rs SR, Everett BM, Birtcher KK, et al. 2018 ACC expert consensus decision
pathway on novel therapies for cardiovascular risk reduction in patients
with type 2 diabetes xnd atherosclerotic cardiovascular disease: a report
of the American College of Cardiologl Task Ft)rce on Expeft Consensus
Decision Pathways. J Am Coll Cardiol. 2018;72:3200 3223. [PMID:
304978811 doi:10.1016rj.jacc.2018.09.020
CONT,
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216. Answers and Critiques
Item 94 Answer: C
Ed ucatio n a I O bj ective : Treat high-risk acute pericarditis.
The nlost appropriate management is to hospitalize the
patient and begin tl,erapy with ibuprofen and colchicine
(Option C). This patient meets the diagnostic criteria defin-
ing acute pericarditis, with pericarditic chest pain, a friction
rub on examination, ECG ch:rnges consistent rt'ith acute
pericarditis, and a pericardial eflusion. Most patients u'ith
acute pericarditis can be managed as outpatientsr hor,r,ever,
those rn,ith accompanying high risk features may require
hospitalization for treatment and monitoring. Patients with
pericarditis and high-risk features, including temperature
higher than 38.0'C (100.4'F), subacute onset. a large peri
cardial effusion or tamponade at presentation, oral anti
coagulation therapy, or lack of response to treatment, may
require hospitalization. This patient has a pericardial eflu
sion smaller than the size connoting high risk (diastolic
echo free space, 2 cm), and she does not ha\€ clinical or
echocardiographic f'eatures ol tamponade. However. she
does have t\ivo high risk features: subacute onset over the
course ofa week and fever greater than 38.0 "C (100.4'F).
Because olthese features, hospitalization to initiate therapy
and evaluate early response is indicated. First-line therapy
fbr acute idiopathic pericarditis is aspirin or NSAIDs. Colchi
cine is recommended as adjunctive therapy to shorten symp
tom duration and reduce treatment failure and recurrence.
Patients who respond to therapy initially but develop recur
rent pericarditis after treatment completion may benefit
fiom a longer course ofstandard therapy lvith slow tapering.
or ttom interleukin-1 signaling blockade with rilonacept.
Outpatient treatment with ibuprof'en and colchicine
(Option A) is appropriate fbr patients with acute pericarditis
who do not have high risk f'eatures.
Glucocorticoid therapy is reserved fbr patients with peri
carditis that is recurrent, incessant (>4 6 weeks' duration),
or chronic (>3 months' duration) despite standard therapy;
uremic pericarditis not resporlsive to intensive dialysisr con
traindications to NSAID therapy; or autoimmune mediated
pericarditis. This patient has no indication for glucocorticoid
tl.rerapy, either as an inpatient or oufpatient (Options B, D).
t(tY PotxIs
o First-line therapy for acute idiopathic pericarditis is
aspirin or NSAIDs and colchicine.
o Patients with acute pericarditis and high-risk features,
including temperature higher than 38.0 'C (100.4'F),
subacute onset, a large pericardial effrrsion or tampon-
ade at presentation, oral anticoagulation therapy, or lack
of response to treatrnent, may require hospitalization.
Bibliography
Adler Y, Charron B Imazio M, et al; ESC Scientific Document Group. 2O1S
ESC guidelines for the diagnosis and management ofpericardial diseases:
the Task Force for the Diagnosis and Management ofPericardial Diseases
ofthe European Society of Cardiolory (ESC) Endorsed by: The European
Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J. 2015;36:
2921-2964. IPMID' 26320112] doi:10.1093/eurheartj/ehv318
Item 95 Answer: B
Educational Objective: Diagnose coronary vasospasm.
This patient's findings of transient dif fuse ST-segment elevation
with associated hemodynamic instability are most consistent
rvith serere multivessel coronary vasospasm (Option B). Coro
nary vasospasm occurs either spontaneousll' from \asomo
tor dy'sfunction or after exposure to dmgs (such as cocaine
or chemotherapeutic irgents) and is olten a diagnosis of
exclusion. Invasive testing n,ith coronary angiographl' ma1'
not reveal spontaneous vasoconstriction. and additional
pro\,ocative testing u,ith acetylcholine or ergonovine infu
sion may be necessary to confirtn irn abnormal vasoconstric
tive response. With the use ol provocative testing. coronary
vasospasm has been identified in as many as 46'X, of patients
with myocardial infarction in the absence of obstructive
coronary artery disease. ECG patterns ma1'be nonspecific
or suggest ST elevation myocardial infarction. Serere cases
o1'multivessel spasrr may be ass<lciated *'ith n'idespread
ST segment elevation and possibll' r'entricular d1'srh1'th
mia and cardiac arrest. Treatment with calcium channel
blockade and,or nitrates can rapidly reverse coronary'\aso
constriction. with improventent in my'ocardial perfusion.
symptoms, ECG abnormalities, and hernodynamics.
Myocardial ischemia and injury may be the result of
coronary embolism liom atrial fibrillation or left
"entric
ular or valvular thrombotic or infbctious processes. Unlike
coronary spasm, embolism (Option A) is identified as fixed
('orolrdry arten obstruction.
Although pulmonary embolism (Option C) has been
associated rvith syncope and cardiac arrest. marked revers
ible ST segment elevation is generally not encountered.
Stress (takotsubo) cardiomyopath), (Option D) m:r1
result in ST segment elevation and or deep symmetric
T wave inl'ersion on ECC associated with chest pain, hemo
dynamic compromise, and elevated cardiac enzymes. These
findings, however are generally in u,clmen and are persistent
rather than transient. with slort'resolution over the course
ofdays to \eeks.
rIT POIf,Is
. ECG pattems in patients with coronary artery spasm
may suggest ST-elevation myocardial infarction; severe
cases of multivessel spasm may be associated with
widespread ST-segment elevation.
. Treatment of coronary artery vasospasm with calcium
channel blockade and/or nitrates can rapidly reverse
coronary vasoconstriction, with improvement in
myocardial perfusion, symptoms, ECG abnormalities,
and hemodlmamics.
Bibliography
Tamis-Holland JE, Jneid H, Reynolds HR, et al; American Heart Association
Interventional Cardiovascular Care Committee ofthe Council on Clinical
Cardiologr; Council on Cardiovascular and Stroke Nursing; Council on
Epidemiologi and Prevention; and Council on Quality of Care and
Outcomes Research. Contemporary diagnosis and management of
patients with myocardial infarction in the absence ofobstructive coronary
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217. Answers and Critiques
anery disease: a scientific statement from the American Heart Association.
Circulation. 2019;139:e891 e908. IPMID: 30913893] doi:10.1161/CIR.
0000000000000670
Item 95 Answer: C
Educational Objective: Manage nitrate tolerance by
reducing the dose frequency.
Once-daily isosorbide mononitrate dosing (Option C) is
the most appropriate management. Nitrates improve myo-
cardial oxygen delivery through coronary vasodilation and
decrease oxygen consumption by reducing preload. They
are an important component of anginal symptom control.
Long acting nitrates, such as isosorbide mononitrate and
patch formulations, provide a constant level of vasodilation
and symptom relief throughout the day. However, a nitrate
fiee interval of B to 12 hours daily is required to avoid nitrate
tolerance, the clinically apparent effect ofprolonged nitrate
exposure reducing nitrate efficacy. This patient has experi-
enced an increase in anginal symptoms with an increase of
isosorbide mononitrate to twice daily dosing, and the most
reasonable next step is to decrease the frequency of admin-
istration to once daily to restore a nitrate-free interval and
nitrate's beneflcial eflects on blood pressure and symptoms.
Calcium channel blockade may be useful in patients
with angina who are symptomatic despite B blocker ther
apy or have intolerance to p blockers. This patient is already
taking the nondihydropyridine calcium channel blocker
diltiazem for blood pressure and rate control, and given
his persistent hypertension (goal systolic blood pressure
<130 mm Hg), it would be reasonable to increase diltiazem
before adding a dihydropyridine calcium channel blocker,
such as amlodipine (Option A), as long as the resting heart
rate remains above 55/min. However, the patient's blood
pressure may reach target by reducing isosorbide mono
nitrate to restore the eflectiveness of the drug on his blood
pressure and anginal symptoms.
Treatment for hypertension should include drug classes
demonstrated to reduce cardiovascular events in patients
with diabetes mellitus. ACE inhibitors or angiotensin recep
tor blockers are recommended as flrst-line therapy for
hypertension in patients with diabetes and coronary artery
disease. A reduction in the lisinopril dosage (Option B) is
not indicated; an increase in dosage may be indicated to
attain the desired systolic blood pressure goal of less than
130 mm Hg if the patient does not respond to isosorbide
mononitrate dosage reduction.
Coronary angiography is an invasive procedure that
carries risk for contrast nephropathy or reactions, vascu-
lar complications (1% to 2"1,), myocardial infarction (0.1'1,),
stroke (<0.1%), or death (0.1'1,). It should be reserved for
patients with unstable or medically refractory symptoms
and uncertain anatomy. Given this patient's recently docu-
mented nonrevascularizable coronary artery disease with
out clinical flndings or features suggesting acute coronary
syndrome, repeat coronary angiography (Option D) is not
indicated.
rEY POIXI
. Long-acting nitrates provide a constant level ofvasodi-
lation and q.,rnptom relief throughout the day, but a
daily nitrate-free interval is required to avoid tolerance.
Bibliography
Katz D, Gavin MC. Stable ischemic heart disease. Ann Intern Med. 2019:
l71tlTc17 ITC32. IPMID: 31382288] doi:10.7326lAlTC2Ol9O8060
Item 97 Answer: B
Ed ucationa I Objective: Diagnose ibrutinib-related atrial
fibrillation.
'lhe
most likely contributor k) the patient's atrial fibrilla
tion is ibrutinib (Option B). Ibrutinib, a tyrosine kinase
inhibitor, is an efi'ective trcatlncnt lbr chronic lympho
cytic leukemia and various B cell lymphomas. It has becr.t
rcportecl to cause atrial fibrill:rtion in up to 16')i, of patie uts.
anri this can be a tl-rerapy linriting rtclverse efl'ect. In one
nreta anall,sis. the incidence ol ibmtinib associatecl atri:ll
librillatior.r r,vas 5.77 per 100 persolr years. much highcr
than in tl.re general population. Ne\,\, onset atrial fibrillrt
tion in patients r.t,ith cancer is associated
"vith
increased
risk fbr heart failure anci thronrboenrbolistn. p Blockers
lre usually used saf'ely fbr rate control. Although diltiazenr
and verapamil are effective in blocl<ing the atrioventricular
node, they are moderate cytochrornc P450 3A4 inhibi
tors. and they may signilicirntly iltcrease plasma lcvcls
of ibrutinib through this nrechanism. Ibrutinib irtcrertscs
cligoxin plasma levels. r'r,hich mav increase the potential
firr digoxin toricity:
Myocardial inlarction (Option A) is unlikelli
'lhis
patient's symptoms have been present fbr 1 to 2 days. Given
the nonnal troponin level. irbsence ol chest pain, nortnltl
lcft ventricular systolic lirnctitlt.t without a regittnal wall
lurotion abnormality, and altsetrce rif specific liCG changes, it
is r-rnlikely that ml,ocardial infar-ction is causing the paticllt's
:rtrial fibrillation.
'[his patient's \Vells score is 2.5 points based on
the presence of malignancy ar.rd heart rate gre.ltcr thilll
100,'rnin. placing him into a motlerate risk group (pulnlrr
nary embolisn.r ur.rlikell. b-y ntodifiecl \Vells criteriir).
'lhc
nomal D climer le."'el ancl ttort.t-tal right heart function ot-t
cchocirrcliography rech-rce t hc cl i n ical probabi lity of' th rrltn
bocmbolisnr (Option C).
'lhe patient has normirl thyroicl stimulating hormot.tc
ar.rcl fiee tl-ryroxine levels. making thyrotoxicosis (Option I))
uulike[1r
XEY POII{T
. Ibrutinib, a
BT osine kinase inhibitor, is associated with
increased rates of atrial fibrillation.
Bibliography
Canatra S, Sharma A, Shah S, et al. Ibrutinib associated atrial fibrillation.
JACC Clin Electrophysiol. 2018;4:1491 500. IPMID: 30573111] doi:10.1016/
i.iacep.2018.06.004
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218. Answers and Critiques
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Item 98 Answer: B
Educational Objective: Treat cardiogenic shock in the
setting of non- ST-elevation myocardial infarction.
This patient is in cardiogenic shock and requires placement ol
an intra aortic balloon pump (Option B). Cardirtgenic shock is
characterizecl by signs ancl syr-nptoms of low cardiac output r,r'itl.r
systolic blood pressure less than 90 mm Hg (or suppon to main
tain blood pressure) and evidence ot end organ hypopefiusion.
This paticnt has already been revascularized: tl.rus. the fbcus ot
therapy should be on supporting the cardiac outpul. Appropri
ate therapics include in<"rtropes, vasopressors, and mechani
cal support (such as balloon pumps, percutaneous ventricular
assist devices, and extracoqrure:rl membrane og,genation).
I)igoxin (Option A) is a r,r,eak inotrope that has bee'n shou,n
to reduce hospitalizations fbr patients $dth chronic hearl fai]ure.
It would not be helpfui fbr this patient in the acutc sctting.
In thc outpatient sctting. B blockers have been shown
to improve survival in patients with heart failure who have
no signs of volume overload. ln the acute setting. cspecialll'
fbr palients receiving norepinephrine. r,r{rich is a potent
vasopressor and has inotropic properties mediated through
the cardiirc B receptor. addirrg a p blocker, sucl.r irs metopro
lol (Option C), would not be ilppropriate.
ln the short term, thc fbcr-rs should be or.r ltenrodl,'nanric
support irr the hopes of imltrovir-rg clrrdiac lirnctior-r ur,er the
next ,18 hours r,r'ith revlrsculurization rirther thin urgent
transler to a transplant ceuter (Option D).
Vasopressin (Option [) may be addcd to norepinepl-r
rine to lurther raise bloocl prcssure or rccluce the close ot
norepinephrine in patients with r,,asodilatory shock (cold
and dry). ln general. r'asoprcssir.r shcluld n<tt ltc used in car
diogenic (r,r'urm and net) or hypovolemic shock.
rEY PO I lIIS
. Treatment of cardiogenic shock initially focuses on
reversing the cause of shock, such as reperfusion in
the setting of acute coronary syndrome.
. In cardiogenic shock, appropriate therapies to support
cardiac output include inotropes, vasopressors, and
mechanical support (such as balloon pumps, percuta-
neous ventricular assist devices, and extracorporeal
membrane oxygenation).
Bibliography
van Diepen S, Katz JN, Albert NM. et al; American Heart Association Council
on Clinical Cardiolory; Council on Cardiovascular and Stroke Nursing;
Council on Quality ofCare and Outcomes Research; and Mission: LifelinE.
Contemporary management ofcardkrgenic shock: a scientific statement
from the American Heart Association. Circulation. 2017;136:e222 e26g.
IPMID: 289239881 doi:10.1161 /CI R.000000000000052s
Item 99 Answer: C
Educational Objective: Identiff the cause of embolic
stroke of undetermined source.
The most reasonable management is loop recorder implan_
tation (Option C). Among patients aged 55 years or older
with an ischemic stroke or transient ischemic attack of
undetermined source. occult intermittent atrial flbrillation
(AF) is thought to be present in up to 25"/,, of cases, and
prolonged ambulatory ECG monitoring may be indicated
for detection. According to the 2019 American College of
Cardiologi/American Heart Association/Heart Rhythm
Socieg AF focused update, in a patient with an ischemic
stroke of unknown cause in whom external ambulatory
ECG monitoring is inconclusive, implantation of a cardiac
monitor (loop recorder) is reasonable to optimize detection
of silent AF (class 2a recommendation). The 2019 guide-
line on the management of acute stroke from the Amer-
ican Heart Association/American Academy of Neurologr
notes that the effectiveness of prolonged cardiac monitoring
during hospitalization after acute ischemic stroke to guide
treatment selection for prevention of recurrent stroke is
uncertain. However, there is some practice variation regard-
ing the duration of "proionged" monitoring and the burden
of detected, asymptomatic AF that warrants anticoagula
tion. Also, the sequence of evaluation varies for example,
extended rhythm monitoring flrst versus testing for intracar
diac shunt with transesophageal echocardiography.
It would be premature to switch this patient's antiplate
let therapy to anticoagulation (Option A) in the absence of
conflrmed AF.
Similarly, consideration of left atrial appendage occlusion
(Option B) would be premature. This procedure is reserved
primarily for patients with known AF who are at significant
risk for stroke and have complicating medical comorbid con-
ditions that make oral anticoagulation high risk or ineflective.
This patient does not have conflrmed AF, nor has oral antico
agulation failed to prevent stroke associated with AE
Thrombophilia evaluation (Option D) is not routinely
indicated among older patients with embolic stroke of unde-
termined source.
l(tv PotltI
. In patients with an ischemic stroke of unknown cause
in whom external ambulatory monitoring is incon-
clusive, implantation of a cardiac monitor (loop
recorder) is reasonable to optimize detection of silent
atrial fibrillation.
Bibliography
January Cl. \{hnn LS. Calkins H, et al. 2019 AHATACC,,HRS focused update
ol the 201.1 AHATACC,IIRS guideline for the management of patients
with atrial fibrillation: a report of the American College of Cardiologr/
American Heart Association Task Force on Clinical
practice
Guidelines
and the Heart Rhythm Society in Collaboration With the Society of
'l'horacic
Surgeons. Circulation. 2019;140:e125-51. [pMID: SOOAObat]
doi: 10.1161 /CIR.0000000OO000066S
Item 100 Answer: A
Educational Objective: Treat a patient with a mechanical
mitral valve with warfarin.
The most appropriate antithrombotic therapy is to continue
the current dosage of warfarin (Option A). This patient,s
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206

219. history and examination flndings are consistent with a nor
mally functioning mechanical mitral valve. patients with a
mechanical cardiac valve require antithrombotic therapy for
thromboembolic prophylaxis. Given the increased throm
bogenicity associated with mechanical versus bioprosthetic
cardiac valves, antithrombotic management requires anti-
coagulant therapy rather than antiplatelet monotherapy.
With the advent of direct oral anticoagulants (dabigatran,
apixaban, rivaroxaban, edoxaban), there has been interest in
using these agents in patients with mechanical and biopros-
thetic cardiac valves. However, given the data from the ran
domized RE ALIGN trial, which showed that dabigatran was
associated with excess bleeding and thromboembolic events
compared with warfarin, dabigatran (Option D) should
be avoided. The safety of other direct oral anticoagulants,
including rivaroxaban (Option E), has not been assessed,
and these agents should not be used. lnstead, warfarin and/
or aspirin should be used, depending on valve type (bio-
prosthetic or mechanical) and position (aortic, mitral, pul
monary or tricuspid). With mechanical valves in the mitral
position, guidelines recommend anticoagulation with war
farin, with a goal INR of 3.0. A range ot 2.5 to 3.5 is accept-
able to balance the risks ofunder and over anticoagulation
in a patient with a mechanical mitral valve.
Although this patient has ecchymoses, he has a normal
hemoglobin level, no clinical evidence of overt bleeding,
and a therapeutic INR. As a result, no change should be
made to his warfarin dosing or his goal INR (Option B). In
practice, it is diflicult to control the INR exactly, and in the
absence of clinically significant overt bleeding, the risk fbr
under-anticoagulation precludes a change in INR range for
ecchymoses.
Aspirin monotherapy (Option C) should not be used for
thromboembolic prophylaxis in patients with a mechanical
valve. Adding aspirin (75 100 mg/d) to warfarin may be con-
sidered in patients who have an indicaticin for antiplatelet
therapy (cardiovascular or cerebrovascular disease) if their
bleeding risk is low Aspirin (75-100 mg/d) monotherapy is
also reasonable in patients with a bioprosthetic valve and no
additional indication for anticoagulation.
I(EY POIT{TS
o Patients with a mechanical cardiac valve require war-
farin anticoagulation for thromboembolic prophy
laxis; dabigatran is associated with excess bleeding
and thrombosis, and the safety of other direct oral
anticoagulants has not been assessed.
. In patients with a prosthetic cardiac valve, the appro-
priate INR goal varies by type ofvalve and valve posi
tion, with a goal INR of 3.0 (range 2.5 3.5) for a
mechanical valve in the mitral position.
Bibliography
Otto CM, Nishimurx RA. Bonow RO. et al. 2020 ACC/AUA guideline lbr the
mrnagement ot patients $,ith valvular heart disease: a report of the
Anrerican College of Carcliolog American Ilcart Associiltion Joint
Committee on Clinical Prirctice Guidelines. Circulation. 2021 :113:e7 )
e227. IPMID: 333321 50] doi:10. I I 61,'CtR.0000oo0000000923
Answers and Critiques
Item 101 Answer: B
Educational Objective: Evaluate a patient with a bicuspid
aortic valve for aortopathy.
'[he
most appropriate management is to perform CT angiog-
raphy (CTA) (Option B) or magnetic resonance angiography
(MRA) of the thoracic aorta. The patient's symptoms and ECG
are consistent with aortic stenosis, and an echocardiogram
reveals a bicuspid aortic valve associated with severe aortic
stenosis. Bicuspid aortic valve is frequently associated with
aortopathy resulting in aneurysm, dissection, and coarctation.
Because these flndings may be associated with the need for
surgical intervention on the aorta itself, appropriate prepro
cedural planning for aortic valve replacement requires careful
evaluation of the thoracic aorta. The initial recommended
imaging test is transthoracic echocardiography However, MRA
or CIA is indicated when morphologr of the aortic sinuses,
sinotubular junction, or ascending aorta cannot be assessed
accurately with echocardiography, as is the case for this patient.
In patients undergoing valve intervention with low pre
test probability for coronary artery disease, CT angiography
of the coronary arteries is recommended and can be per
lbrmed at the same time as CT angiography of the aorta.
ln those u,ith intermediate or high pretest probability of
coronary disease, invasive coronary angiography is recom
mended to assess coronary anatomy and guide the need for
and type of revascularization. However, assessment of the
aorta should occur first to rule out aortic pathologr, which
may complicate cardiac catheterization (Option A).
Exercise stress testing (Option C) is not the appropri
ate next step in management. Exercise stress testing is con
traindicated in symptomatic severe aortic stenosis, given the
increased risk fbr severe adverse events (sudden cardiac death).
In patients with bicuspid aortic valve and symptomatic
severe aortic stenosis, transcatheter aortic valve implanta-
tion (TAVI) (Option D) may be considered as an alternative
to surgical aortic valve replacement if the procedure is per
fbrmed at a Comprehensive Valve Center. However, TAVI
may not be a viable therapy in patients who require surgery
because of concomitant aortopathy.
rEY POIl{I5
. Bicuspid aortic valve is frequently associated with
aortopathy resulting in aneurysm, dissection, and
coarctation and requires initial assessment with
transthoracic echocardiography.
o In patients with a bicuspid aortic valve, CT angiography
or magnetic resonance angiogfaphy ofthe thoracic aorta
is indicated when morphologz olthe aortic sinuses, sino-
tubularjunction, or ascending aorta cannot be assessed
accurately with transthoracic echocardiography.
Bibliography
Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for thc
management of patients with valvular heart disease: a report of the
American College of Clrtiiolory American Heart Association Joint
Committee on Clinical l)ractice Guidelines. Circulation. 2o2l:143:e7)
e227. LPMII): 333321501 doir10.1161 iCIR.0000000000000923
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Item 1O2 Answer: C
Ed ucati o na I O bjective : Treat chronic limb-threatening
ischemia (critical limb ischemia).
The most appropriate next step in the management of this
patient with chronic limb-threatening ischemia (CLTI), also
called critical limb ischemia, is to perform invasive angiog-
raphy of the affected limb (Option C) with subsequent revas
cularization. CLII is characterized by more than 2 weeks of
ischemic rest pain, nonhealing wound/ulcers, or gangrene
in one or both legs that is attributable to objectively proven
peripheral artery disease (PAD). PAD can be conflrmed
with ankle-brachial index testing (often <0.4 in CLTI), toe-
brachial index testing, and other measures. Unlike patients
with other symptoms of PAD (e.g., intermittent claudica-
tion), patients with CLTI often cannot exercise because of
skin ulceration and/or ischemic rest pain. The primary goal
for patients with CLTI is to revascularize the limb to reduce
the incidence of major amputation, and invasive angiog-
raphy expedites the diagnosis and allows for concurrent
endovascular revascularization. In patients who undergo
successful limb revascularization, the 1 year risk for major
amputation is signiflcantly lower than in patients who do
not undergo revascularization.
Owing to the high morbidity and mortality associated
with CLTI, immediate referral for invasive angiography with
endovascular revascularization is often the most eflective
strateS/ to preserve tissue viability. Noninvasive imaging stud-
ies, such as CT angiography (Option A), would result in treat
ment time delays in this patient with CLTI and a viable limb.
Clinical trials of hyperbaric oxygen therapy (Option B)
for the treatment ofarterial ulcers have focused on patients
without severe PAD and have not demonstrated a long term
beneflt on wound healing or an improvement in amputation-
free survival compared with sham treatment. Hyperbaric
oxygen therapy is not a viable treatment option for this
patient who needs urgent revascularization.
Primary amputation (Option D) refers to amputation
without attempting to salvage the limb. In patients with
CLTI and limb viability, invasive angiography and revascu
larization as an initial strates/ is always preferred to primary
major amputation of the lower extremity. Patients older than
65 years who undergo major amputation have a 1-year mor
tality rate of nearly 50'7, and a 3-year mortality rate higher
thanTO"/,,.
XEY POIilTS
r Chronic limb-threatening ischemia is characterized by
more than 2 weeks of ischemic rest pain, nonhealing
wound/ulcers, or gangrene in one or both legs that is
attributable to objectively proven peripheral artery
disease.
. In patients with chronic limb-threatening ischemia,
immediate invasive angiography with endovascular
revascularization is often the most effective strateg/ to
preserve tissue viability.
Bibliography
I.arber A. Chronic limb threatening ischemia. N Engl J Med. 2018:379:l7l
180. IP\'1lD: 2999608s] doi:10.1056/NEJMcp1709326
Item 1O3 Answer: B
Educational Objective: Prevent sudden cardiac death in
a patient with hypertrophic cardiomyopathy.
Implantable cardioverter-deflbrillator (lCD) therapy (Option B)
is the most appropriate treatment. Any unexplained episode
of syncope in a patient with hypertrophic cardiomyopathy
(HCM) represents a potential arrhythmic event and requires
further risk stratiflcation, including echocardiography and
ambulatory ECG monitoring for arrhythmia. The 2020
American College of Cardiologz/American Heart Association
Guideline for the Diagnosis and Treatment of Patients With
Hypertrophic Cardiomyopathy recommends ICD placement
for patients with HCM and previous documented cardiac
arrest or sustained ventricular tachycardia (class 1 recom
mendation). For adult patients with HCM and one or more
major risk factors for sudden cardiac death (SCD), offering
an ICD is reasonable (class 2a recommendation). Risk factors
include SCD judged definitively or likely attributable to HCI\4
in one or more first-degree or close relatives aged 50 years
or younger, left ventricular (LV) hypertrophy of 30 mm or
greater in any LV segment, one or more recent episodes of
syncope suspected by clinical history to be arrhy,thmic in
nature, LV apical aneurysm, and LV ejection fraction less than
50'7,. Based on the patient's recent episode of unexplained
syncope and LV wall thickness of 30 mm, it is reasonable to
refer her for consideration ofan ICD to prevent SCD.
For patients with HCM and symptoms attributable to
LV outflow tract obstruction, nonvasodilating p blockers
are recommended. Such symptoms typically include effort-
related dyspnea or chest pain. If p-blockers are ineffective or
not tolerated, a nondihydropyridine calcium channel blocker
(verapamil or diltiazem) may be substituted. If symptoms
persist despite p blocker or nondihydropyridine calcium
channel blocker therapy, adding disopyramide in combi-
nation with one of the other drugs or treatment w'ith sep-
tal reduction therapy (SRT) is recommended. This patient's
obstructive symptoms generally have been well managed on
metoprolol, and her outflow tract gradient on echocardiog
raphy does not indicate severe LV outflow tract obstruction;
thus, changing this patient's medications (Options A, D) is
unnecessary.
A peak LV outflow tract gradient (resting or provoked)
of 50 mm Hg or greater is generally considered the threshold
for SRT (Option C) in patients with drug refractory symp-
toms. Referring this patient for SRT is not indicated.
I( EY PO I XTS
o An implantable cardioverter-defibrillator is recom
mended for patients with hypertrophic cardiomyopathy
and previous documented cardiac arrest or sustained
ventricular tachycardia.
(Continued)
208

221. Answers and Critiquesi
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. In patients with hypertrophic cardiomyopathy (HCM)
and risk factors for sudden cardiac death (SCD), such
as SCD attributable to HCM in a first-degree or close
relative aged 50 years or younger, left ventricular (LV)
hypertrophy of 30 mm or greater in any LV segment,
syncope suspected to be arrhythmic in nature, LV api
cal aneurysm, and LV ejection fraction less than 50%,
it is reasonable to offer an implantable cardioverter-
defibrillator.
Bibliography
Ommen SR, Mital S, Burke MA, et al. 2020 AHA/ACC guideline for the diag
nosis and treatment of patients with hypertrophic cardiomyopathy: exec
utive summary: a report of the American College of Cardiolos//American
Heart Association loint Committee on Clinical Practice Cuidelines.
Circulation. 2020;142:e533 e557. IPMID: 33215938] doi:10.1161/CIR.
0000000000000938
Item 104 Answer: A
Educational Objective: Treat a patient with non-ST-
elevation acute coronary syndrome with dual antiplatelet
therapy.
All patients u,ith non ST-elevation acute coK)nary synclrome
(NSTE-ACS) sl-rould be treated with aspirin and a P2Y,, inlrib-
itor. such as clopidogrel (Option A). regarclless of reperfusion
strateS/. Cun-ent Anrerican Hearl Association,'American Col
lege of Cardiologz guidelines recommend aclnrinistration of
mpirin (162 :125 mg at presentation. fbllowecl by 81 162 rng/d)
lnci P2\',, inl.ribitior.r cornbined n'ith earl1, inrasive irngiog
raphy rtithin 2,1 hours of presentation in hemodynamically
stable paticnts who have evidence of NSI'U ACS and elevated
risk as determined by prognostic irssessmer.rt with risk scores.
such as'flMI and GRACE risk moclels. Clopidogrel or ticagre
lor is indicated at presentation in patients with acute coronary
syndroure managed with an early inrrasivc strates/ (class
1
recommendation). Ticagrelor (lB0 mg at presentation lol
lor,ved by 90 n1g twice daily) is more e{Iective than cktpidogrel
(300-600 mg at prcsentation followed by 75 mg/d) and pre-
f'crred in patients rtithout increased bleeding risk. Horterer.
ticagrelor is associated rtith higher rates ot'bleeding than clcr
pidogel and is associatcd with an increasecl incidence of dys-
pnea and bradycardia.
'l
his patient has oxygen dependent COPD,
r,r,hich may limit her ability to tderutc ticagelor therapy:
'lhere
fbre. clopidogrcl is the rnost reasonable option fbr this paticnt.
Prasugrel (Option ts) is an cftective antiplatelet agent in
patients with ACS but is resenred ltlr use after coronary stent
placement and not at presentation. Prasugrel is also contra
indicatecl in p:rtients rged 75 years or older and those with
previous transient ischemic attack or stroke; it also must be
rusecl rvitl-t crution in underr.teight patients.
Thronrbolytic therapyi such as reteplase (Option C),
has not been shown 1o be benelicial in patients without
ST ele'uation myocardial infarction (STEMI) or STEMI cquiv
alcnts (e.g., ner,r,, bundle branch block) and is r.rot indicated
lbr this patient.
Glycoprotein llb/llla inhibitors, such as tirofiban
(Option D) and eptifibatide, are potent iutravenous anti
platelet agents that are generally reserved for use at the
time of coronary angiography and coronary intervention for
treatment of significant intracoronary thrombus burden.
Tirofibar.r rvould have no benefit ir-r tl.ris clinically stable
patient and has the potential to increase bleeding.
TEY POTTI
. All patients with non-ST-elevation acute coronary
syndrome should be treated with aspirin and a P2Y12
inhibitor regardless of reperfusion strates/.
Bibliography
Anderson Jt., Morrow DA. Acute myocardial infarction. N Engl J Med.
2077 ;376:2053 64. IPMID: 28538121] doi:10.1056/NEJMral606915
Item 105 Answer: C
EducationaI Objective: Evaluate refractory ischemic chest
pain with invasive coronary angiography.
lnvasive coronary angiography (Option C) is the rnost
appropriate imaging test to evaluate this patient's chest
pain. This procedure is indicated for patients with acute
chest pain who arc highly synptomatic with abnormal
findings on stress testing or lbr selected patients with an
acute coronary synclrome. Coronary angiographl, should
also be considered fbr patients with ischemic symptoms
that are relractory to medication, such as tl-ris patient with
unstable angina refractory to rnedical therapy. If culprit
lesions arnenable to intervention are identilied cluring
diagnostic coronary angiography, percutaneous revascu
larization can be perfbrmed.
Calcification of the coronary arteries indicates athero-
sclerosis and may be quantified with CT. Although coronary
ar1ery calcium (CAC) scoring (Option A) provides inlirrnration
regarding the burden of disease. it cannot determine the
clegree o1'obstruction. In addition, CAC scoring has no use in
patients already receiving statin therapy CAC scor:ing will pro
vide no benefit to this patient with acute, refiactory ischemia.
This patient's chest pain w"rrants inraging of the cor
onary arteries, rvhich could be accomplisl.red by coronary
CT angiography (Option B). Ilowever, the acuity of this pre-
sentation indicates a high likelil-rood ol requiring coronary
inten€ntion, '"r,hich nrould be delayed by pursuing another
imaging test beforehand. In addition, coK)nary CT angiogra
phy assessnrent of atherosclercltic occlusions may be limited
b1r the presence of accompanying calcification.
Transthoracic echocardiography (T'lE) (Option D) can
evaluate right ancl lctt chamber size, thickness, and func
tion, including wall motion. TTE also provides infbrnration
on valr,ular pathology, diastolic function. hernodynam
ics, and tl.re pericarclium. New wa1l rnotion abnonr.ralities
might suggest the presence ol ischemic disease, but cor-
onary ar.rgiography can establish a precise anatomic diag-
nosis and has the potential b be fbllowecl immediately by
rcvasculari zation.
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Answers and Critiques
XEY POIlIT
. Coronary angiography is indicated for patients with
acute chest pain who are highly symptomatic with
abnormal findings on stress testing, selected patients
with acute coronary syndrome, and patients with
ischemic symptoms that are refractory to medication.
Bibliography
Amsterdam EA, Wenger NK, Brindis RG, et al. 201,1 AHA/ ACC guideline fbr
the management of prtients with non ST elevation acute coronary syn
dromes, a report of the American College of Cardiolog, American Heart
Association Task Force on Practice Cuidelines. I Am Coll Cardiol.20l1:
64:e139 e228. IPMID: 25260718] doi:10.1016 j.jacc.20l.1.09.O17
Item 106 Answer: C
Educational Objective: Manage advanced heart failure
with a left ventricular assist device.
The most appropriate management is left ventricular assist
device (LVAD) placement (Option C). LVADs are reasonable
either as a bridge to heart transplant or as primary therapy
in patients with advanced heart failure. Survival at 1 year is
near the survival after heart transplant, and an LVAD would
be worth considering in patients who have several markers
of increased mortality. This patient has advanced heart failure
with several risk factors for an increase in mofiality over the
next 2 years. These risk factors include several heart fail
ure hospitalizations, worsening kidney function, low semm
sodium level, high diuretic dosage, and inability to toler
ate usual heart failure medications because of symptomatic
hypotension. Based on these risk factors, a discussion with
the patient about his $'ishes fbr end ofllife care and thoughts
about advanced heart failure therapies would be appropriate.
This patient is not a candidate for heart transplant
(Option A) based on his age and recent colon cancer. Most
centers have an age cutoff of 65 years, although some may
extend the cutoff past that age. With the exception of skin
cancer, most programs require that a patient be cancer lree
for at least 5 years before being considered for heart transplant.
The decision to hospitalize a patient with heart failure
(Option B) is usually based on evidence of acute decompen
sation with elevated neck veins and symptomatic heart lail
ure. This patient has New York Heart Association functional
class III to IV symptoms but does not appear to have volume
overload on examination. Therefore, his symptoms are most
likely unrelated to acute volume overload and instead are a
sign of his progressive heart failure.
This patient's p blocker (carvedilol) was stopped because of
slmptomatic hypotension. There is no absolute blood pressure
cutofffor initiating a p blocker, but starting a p blocker (Option D)
in a patient who has not tolerated one in the past, especially with
this patient's low blood pressure, is not appropriate.
I(EY POITI
o Left ventricular assist devices, either as a bridge to
heart transplant or as primary therapy, are reasonable
in patients with advanced heart failure.
Bibliography
Guglin N{. Zucker l\{J. Borlaug BA, et al: ACC Hean l,ailure and'l'ransplant
l\'lember Section and Leadership Council. E\€luation tbr heart transplanta
tion and L\AD implantation: JACC Council Perspectives. J Ant Coll Cardiol.
2020r75:1-171 1487. IPMII): 32216916) doi:10.1016,'j.jlcc.2020.01.03.1
Item 107 Answer: A
Educational Objective: Treat a patient with atrial fibril-
lation and coronary artery disease.
The most appropriate management is to discontinue aspirin
(Option A). This patient is currently treated with triple ther
apy with an oral anticoagulant and dual antiplatelet therapy
to prevent stroke in atrial fibrillation (AF) and recurrent
acute coronary syndrome, respectively. Each of these drugs is
the cornerstone of preventive therapy lor the respective dis
ease processes; however, together they significantly increase
the risk for bleeding. This patient is being evaluated 7 months
after coronary stent placement, and she has a history ofgas
trointestinal bleeding. lt is reasonable to discontinue aspirin
to reduce the risk for bleeding associated lt,ith triple therapy,
as several trials have demonstrated the safety and efficacy of
double therapy with an oral anticoagulant and a second- or
third generation P2Y,, inhibitor. such as clopidogrel.
This patient's CHATDS2 VASc score is 4 (1 point each
for age 65 74 years, female sex, hypertension, and coronary
artery disease), putting her at substantial risk for AF'related
stroke. Although dual antiplatelet therapy may be sufficient
to prevent recurrent acute coronary syndrome, it is insuffi
cient therapy to prevent stroke. lherefore, discontinuation of
rivaroxaban (Option B) is inappropriate.
Although switching from rivaroxaban to reduced dose
apixaban (Option C) may reduce this patient's risk for bleed
ing, this strates/ has two drawbacks. First, triple therapy
with reduced dose apixaban may not be associated with a
lower bleeding risk than dual therapy with rivaroxaban plus
clopidogrel. Second, the reduced dose ofapixaban fbr stroke
prevention in AF is indicated among patients with at least
two of the fbllowing three criteria: age B0 years or older,
weight less than 60 kg (132.3 lb), and serum creatinine level
of 1 .5 mgldl or higher (>-tZZ.6 pmol/L). She does not fulflll
these criteria and will not be adequately treated to prevent
AF related stroke with reduced dose apixaban.
Switching rivaroxaban to warfarin (Option D) is more
likely to increase her bleeding risk, not decrease it,
"t,ithout
signiflcant improvement in clinical benefit in terms of stroke
prevention. Of more importance, switching from one anti
coagulant to another does not address the burden oF triple
therapy in this patient.
XEY POIilT
. Among patients with atrial fibrillation who have under-
gone percutaneous coronary intervention for acute coro-
nary syndrome, double therapy with clopidogrel or
ticagrelor plus a direct oral anticoagulant is recommended
over triple therapy with an oral anticoagulant, aspirin, and
P2Y,, inhibitor to reduce the risk for bleeding.
210

223. Bibliography
January CT, Wann LS. Calkins II. et al. 2019 AHA'ACC/ IIRS focused update
of the 201.1 AHA,ACC,'llRS guideline tbr the management ol patients
h,ith atrial fibrillation: 0 report of the American College of Cardiolog, r
American I leart Association Task Firrce on Clinical Practice Guidelines
and the llcart Rhythm Sociery J Am Coll Cardiol.2019;74:104 132.
IPMID: 307031311 doi:10. l0l6/j.jacc.2019.01.011
Item 108 Answer: E
Ed u cati ona I O bj ective : Avoid inappropriate endocarditis
prophylaxis.
This patient requires no endocarditis prophylaxis (Option E).
Endocarditis prophylaris is recommended for a specific group
of patients before dental procedures that involve manipula
tion ofgingival tissue or the periapical region ofthe teeth, or
perforation of the oral mucosa. The highest risk for infective
endocarditis (lE) is in patients with a prosthetic val'ue, previous
IE, or congenital heart disease with residual flow disturbances.
Current indications for endocarditis prophylaxis include (1) a
history of IE; (2) cardiac transplantation with valve regurgita
tion due to a structurally abnormal valve; (3) a prosthetic valve;
(4) prosthetic material used for cardiac valve repair, including
annuloplasty rings and chords; (5) unrepaired cyanotic congen
ital heart disease; (6) repaired congenital cyanotic heart disease
with residual defects at the site or adjacent to the site of a pros
thetic patch or device; and (7) a defect that has been repaired
(surgical or catheter based) with prosthetic material within the
previous 6 months. None of these indications is present in this
patient, and antibiotic prophylaxis is unnecessary
In patients with valvular heart disease who are at high risk
fbr IE, antibiotic prophylaris (Options A-D) is not recommended
for nondental procedures, such as transesophageal echocardiog
raphy, esophagogastroduodenoscopy, colonoscopy, or cystoscopy,
in the absence of active infection. Drug resistant organisms,
Clostridioides dfficile colitis, unnecessary expense, and drug
toxici$/ may result from indiscriminate antibiotic prescribing.
If the patient had an indication for endocarditis pro
phylaxis, amoxicillin (2 g once orally 60 minutes befbre the
cleaning) would be the most appropriate therapy. Azithro
mycin and clindamycin should be reserved for patients able
to take oral antibiotics who have a penicillin allergz. lntra
venous ceftriaxone should be used for endocarditis prophy-
laxis only in patients unable to take oral antibiotics.
I(EY POITTS
. Endocarditis prophylaxis is indicated only for patients
at high risk for infective endocarditis who are about to
undergo gingival manipulation or other procedures
that break the oral mucosa.
. The highest risk for infective endocarditis (lE) is in
patients with a prosthetic valve, previous IE, or congenital
heart disease (repaired or unrepaired) with residual flow
disturbances; other indications include cardiac transplan
tation vah.ulopathy, prosthetic material used for cardiac
lalve repair, and a cardiac defect that has been repaired
with prosthetic material within the previous 6 months.
Answers and Critiques
Bibliography
Otto CM, Nishimura RA. Bonorv RO. et al. 2020 AC(irAHA guideline tbr the
management of patients u'ith v;rlvular heart disease: a report of the
American College of Cirrdiolopy,'American IIeart Association Joint
Committec on Clinical Practice (iuidelines. CircuLltion. 2021 ;l4lj:e7'2
e227. IPl\4 Il): 333321s01 doi:10.1161 1cl R.00000000o0000923
Item 109 Answer: A
Educational Objective: Monitor an asymptomatic patient
with severe aortic regurgitation.
'lhe
most appropriate next step in management is reevalua-
tion with clinical examination and echocardiography in 6 to
12 months (Option A), or earlier as dictated by a change in
clinical status or examination findings concerning for heart
failure. This patient's examination flndings (widened pulse
pressure, murmur, and bounding pulses) are consistent with
severe aortic regurgitation (AR). With all regurgitant lesions,
indications for invasive intervention are based on either the
negative impact of valvular regurgitation on ventricular size/
contractility or the emergence of symptoms due to valvular
regurgitation or heart failure. ln symptomatic patients with
severe AR, aorlic valve surgery is indicated regardless of left
ventricular (LV) systolic function. In asymptomatic patients
with chronic severe AR and LV systolic dysfunction (LV ejec
tion fraction <557,), aortic valve surgery is indicated ifl no
other cause for systolic dysfunction is identifled. In asymp
tomatic patients with severe AR and normal LV systolic
function (LV ejection fraction >55'/"), aortic valve surgery
is reasonable if the left ventricle is severely enlarged (LV
end systolic dimension >50 mm).
This patient does not require surgical aortic valve
replacement now (Option B). He is asymptomatic, and
quantitative echocardiographic evaluation conflrms severe
AR with a normal LV ejection fraction and without signif
icant ventricular dilation. He should be followed closely
with clinical examination and echocardiography every 6 to
12 months.
Current guidelines recommend that patients with iso
lated severe AR who have indications for surgical aortic valve
replacement and are candidates for surgery not undergo
transcatheter aortic valre implantation (Option C), because
it is rarely feasible and has potential harms.
Transesophageal echocardiography (Option D) may be
used to assess AR. However, in native valve AR, it rarely
provides incremental diagnostic information beyond what
is obtained by transthoracic echocardiography with good
image quality.
rEY POIl{TS
. In symptomatic patients with severe aortic regurgitation,
aortic valve surgery is indicated regardless ofleft ventric
ular systolic function.
r In asymptomatic patients with chronic severe aortic
regurgitation and left ventricular systolic dysfunction
(ejection fraction <55%), aortic valve surgery is indi-
cated.
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224. Answers and Critiques
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Bibliography
Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the
management of patients with valvular heart disease: a report of the
American College of Cardiolo$//American Heart Association Joint
Committee on Clinical Practice Guidelines. Circulation. 2021 143:e72
e227. IPMID: 33332150] doi:10.1161/CIR.O00000OO0oooo923
Item 11O Answer: B
Educational Objective: Discontinue flecainide in a
patient with ischemic heart disease.
The most appropriate management is to discontinue fle
cainide (Option B). This patient has ischenric l.reart disease
and previously diagnosed paroxysmal atrial fibrillation (AF).
Amioclarone. dof'etilide. flecainide. propafenone. sotalol.
and dronedaK)ne may be used to maintain sir.rus rhythrn in
patients
'-t ith Ali Antiarrhyhmic drug selectiou is guided by
the patient's comorbid conditions and safet-v considerirtior-rs.
Flecainide is a class IC antiarrhyhmic agent and, along \,\,ith
propafblrone. is absolutely contraindicated in patients rvith
ischemic heart disease, gir€n the increased risk for ventricular
arrhythmias in this population. This patient also has evidence
of left r,entricular (LV) dysfirnction on a post m),"ocardia}
infarction echocardiograrr.r. r,r,l.rich n.ray be due to infarcted (as
opposed to stunned) myocardium and nlay lead to increased
long term risk fbr ventricular arrhl.thmias. Furlhermore. his
ECG shows widening of the QRS intenal, $rhich may reflect
adverse ellects of flecainide.
'lhis
drug, thereflore, is not safe in
tl-ris patient; it should be discontinued ar-rd not restarted.
Although ambulatory ECG morritoring (Option A) at
discharge nray be helpful to assess AF burden, rates. and
symptoms, it should not be used to guide flecainide theripl'
in this patient, given his contraindications, which arnbula-
tory ECG mollitoring r,r,ill not address.
There is no reason to stop metoprolol (Option C). This
patient's heaft rate is only mildly bradycardic. does not appear
to be causing adrerse effects, and is unlikely to be aft'ecting
his right bundle branch block. Aggressive p blockade is r,tar
ranted, given his recent myocardial infarction, coronary artery
disease, and LV dysfunction. Furlhermore. he will likell'need
metoprolol for rate control, if or when he relerls to AE
In the revascularized patient nith stable or absent
ischemic symptoms. there is no role lor predischarge stress
testing (Option D). Although exercise stress testing is some
tinres used to assess the QRS duration during exercise in
patients treated with class IC antiarrhythmic drugs, it will
not change management in this patier.rt. This patient already
has QRS
prolongation at rest and an absolute contraindica
tion to flecainide because of his coronary artery disease.
XEY POIXI
r Flecainide and other class IC antiarrhythmic agents
are contraindicated in patients with ischemic heart
disease.
Bibliography
Echt DS. Ruskin JN. Use of flecainide for the treatment of atrial fibrillation.
Am J Cardiol. 2020;125:1123 33. [PMID: 320440371 doi:10.1016/j.ami
card.2019.l 2.041
Item 111 Answer: A
Educational Objective: Treat heart failure with cardiac
resynchronization therapy.
Cardiac resynchronization therapy (CRT) (Option A) is the
most appropriate treatment. This patient with heart fail-
ure with reduced ejection fraction is on optimal guideline-
directed medical therapy. CRT is indicated in patients with
a left ventricular ejection fraction (LVEF) of 35% or less,
New York Heart Association (NYHA) functional class II to IV
symptoms despite guideline-directed medical therapy. sinus
rhythm, and left bundle branch block with a QRS complex of
150 ms or longer (class 1 recommendation). In such patients,
CRT is associated with improved LVEF, reduced symptoms,
and improved survival rates. Many patients who meet the
indication for CRT also meet indications for implantable
cardioverter-deflbrillator (lCD) therapy. ICD therapy reduces
mortality in patients with NYHA class I to III heart failure
symptoms and LVEF less than 35'/o on optimal medical ther-
apy. This patient meets the criteria for ICD placement, but
she may experience substantial improvement in ejection
fraction with CRT and may not require or beneflt from ICD
insertion.
In patients with LVEF of 35% or less who are in sinus
rhythm with a heart rate of at least 7Olmin and taking
maximally tolerated doses of a p-blocker, the sinoatrial
node modulator ivabradine (Option B) reduces heart
failure-associated hospitalizations and the combined
end point of mortality and heart failure hospitalization.
Ivabradine is not indicated in this patient with a heart
rate of 58/min.
The mitral valve clip (Option C) is designed to approx
imate mitral valve leaflets and reduce mitral regurgitation.
Mitral valve clip placement is reasonable for patients with
severe secondary mitral regurgitation with heart failure
symptoms and a left ventricular end systolic dimension
Iess than 70 mm and pulmonary artery pressure less than
70 mm Hg. CRT is more likely to improve symptoms and is
the most appropriate step for this patient before considering
a mitral valve clip.
Transcatheter aortic valve implantation (16y1) (aption D)
is usually reserved for patients with symptomatic severe
aortic stenosis. Factors that increase procedural risk include
reduced ejection fraction, another reason not to proceed
with TAVI in this patient.
t(lY POrXr
o Cardiac resynchronization therapy is indicated for
patients with ejection fraction of 35% or less with left
bundle branch block, QRS duration of 150 ms or
greater, and New York Heart Association functional
class II to IV symptoms despite guideline-directed
medical therapy.
Bibliography
Wu A. Heart failure. Ann Intern Med. 2o18;168:lTC8l ITC96. [PMID:
2986881 6l doi:10.7326lAITC201806os0
212

225. Item 112 Answer: B
Educational Objective: Assess the benefit ofcoronary
artery bypass grafting in a patient with chronic stable
angina.
The anticipated benefit of coronary artery bypass grafting
(CABG) in this patient with stable coronary artery disease is
an improvement in symptoms (Option B). The relative ben-
eflt of revascularization depends on a patient's underlying
clinical and anatomic features. The goal of revasculariza
tion in stable syndromes is generally to relieve angina and
improve quality of life. This patient has stable but persistent
symptoms in the setting of documented moderate ischemia
and two vessel coronary artery disease with preserved left
ventricular function. Revascularization with CABG (or per
cutaneous coronary intervention) and continued medical
therapy have the potential to improve symptoms and would
be the primary reason to proceed with revascularization in
this patient with persistent symptoms despite maximally
tolerated medical therapy. CABG with optimal medical ther
apy is generally recommended for patients with multivessel
coronary artery disease, particularly in the setting of left
ventricular dysfunction and/or diabetes mellitus, because it
results in decreased recurrence ofangina and lower rates of
major cardiovascular events compared with percutaneous
intervention or medical therapy alone.
Antiplatelet therapy and other cardioprotective medi
cations, such as statins, are required in all patients with cor
onary heart disease, including those who have undergone
CABG. Discontinuation of cardioprotective medications
(Option A) is not a beneflt of coronary artery revascular
ization.
CABG may be considered an option to increase survival
(Option C) in patients with signiflcant (>70'1,) stenosis of two
or more major coronary arteries in the setting of extensive
myocardial ischemia, especially with involvement of the
proximal left anterior descending coronary artery and/or
marked left ventricular dysfunction. This patient does not
meet these criteria.
In this patient with a stable coronary syndrome receiv
ing optimal medical therapy and secondary prevention strat
egies, there is no expectation for reduced risk for myocardial
infarction (Option D) or death following surgical or percu
taneous revascularization. Early symptomatic improvement
would be the primary goal of revascularization.
XEY POIIITS
. Coronary artery bypass grafting can reduce angina in
patients with chronic stable angina.
. Coronary artery bypass grafting may be considered as
an option to increase survival in patients with signifi
cant (>7O%) stenosis of two or more major coronary
arteries in the setting ofextensive myocardial
ischemia, especially with involvement of the proximal
left anterior descending coronary artery and/or
marked left ventricular dysfunction.
Answers and Critiques
Bibliography
PATEI MR, CiTIhOON JH, DChMCT GJ, Et AI. ACC/AATS/AHA/ASI]/ASNC/SCAI/
SCCT/STS 2017 appropriate use criteria fbr coronary revascularization in
patients with stable ischemic heaft disease: a report of the American
College ol Cardiolory Appropriate Use Criteria Task Force, American
Association tbr Thoracic Surgery. American Heart Association. American
Society of llchocardiography, American Society of Nuclear Cardiologl,
Society frrr Cardiovascular Angiography and Interventions, Society of
Cardiovascular Cr)mputed n)mography, and Society of Thoracic Surgeons.
J Am Coll Cardiol. 2017;69:2212 41. [PMID: 282916631 doi:10.1016/j.
iacc.20U.02.00l
Item 1 13 Answer: A
Educational Objective: Diagnose atrial septal defect.
An atrial septal defect (ASD) (Option A) is the most likely
diagnosis in this patient. An ASD is a defect in the atrial
septum resulting in a left to right shunt with eventual
right-sided cardiac chamber dilatation in most patients.
Adults with an ASD most often present with dyspnea, atrial
arrhythmias, and/or right heart enlargement detected on
cardiac imaging. Characteristic clinical flndings include ele
vated central venous pressure, a right ventricular lift, and
flxed splitting ofthe Sr. A large left to right shunt causes a
pulmonary midsystolic flow murmur and a tricuspid dia-
stolic flow rumble owing to increased flow This patient's
ECG shows right axis deviation, incomplete right bundle
branch block, and right ventricular hypertrophy, flndings
consistent with secundum ASD.
Aortic regurgitation due to a bicuspid aortic valve
(Option B) causes a diastolic murmur at the left sternal bor
der. The central venous pressure is generally normal, and a
right ventricular impulse would not be expected. A systolic
ejection click is often heard in patients with bicuspid aor
tic valve, but flxed splifiing of the S, is not heard. The ECG
typically demonstrates a normal axis and features of left
ventricular hypertrophy.
Patients with mitral stenosis (Option C) often present
with dyspnea and atrial arrhythmias. The central venous
pressure is often elevated, with associated pulmonary
hypertension or tricuspid regurgitation. A right ventricu
lar impulse may occur. An opening snap might be heard,
loilowed by a diastolic murmur; this is generally best heard
at the apex. Fixed splitting of the S, is not heard. The ECG
typically demonstrates left atrial enlargement.
Patients with a small (restrictive) ventricular septal defect
(VSD) (Option D) are usually asymptomatic and demonstrate
a loud holosystolic murmur along the left sternal border that
often obliterates the Sr. A thrill may be noted at the left stemal
border. A right ventricular impulse would not be present. The
ECG in patients with a small VSD is typically normal.
rtY P0l1{Tt
o Characteristic clinical findings of an atrial septal defect
include a parasternal impulse, fixed splitting of the 52,
and a pulmonary outflow murmur.
. In patients with an ostium secundum atrial septal
defect, the ECG demonstrates right axis deviation and
incomplete right bundle branch block.
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213

226. D
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Answers and Critiques
Bibliography
Stout KK. Daniels CJ. Aboulhosn JA. et al. 2018 AHA ACC guideline fbr the
management of adults \'ith congenital heart disease: a report of the
American College ofCardiolopS American Heart Association Task Rrrce
on Clinical Practice Guidelines. J Am Coll Cardiol. 2019:73:e81 e192.
IPMID: 301212391 doi:10.1016 j.iacc.2018.08.1029
Item 114 Answer: D
Educational Objective: Treat parorysmal atrial fibrilla-
tion with heart failure with rhythm control.
The most appropriate treatment is rhyhm control (Option D).
Atrial flbrilation (AF) complicates nearly 40'l. of heart failure
cases, and the combination of AF and heart failure dramatically
yorsens outcomes. Several clinical trials have demonstrated that
aggressive efforts to achiere rhlthm control in patients with AF
and concomitant heart failure decrease morbidity and mortality.
In patients with heart failure with reduced ejection fraction,
recent clinical trials have shown that catheter ablation of AF is
associated with a favorable eflect on morbidity and mortality
compared with medical therapy. Amiodarone is another option
for rhythm control. Although amiodarone carries a risk for
toxicity, this patient's lifetime exposure to the drug is likely to be
relatively low given her age, comorbid conditions, and probable
amiodarone dosage, which is generally lower for AF than lor
ventricular arrhyhmias. Monitored appropriately, it can be used
safely and eflectively in patients her age.
Atrioventricular node ablation with permanent pacemaker
implantation (Option A) is an option for patients with AF who
have continued symptomatic tachycardia despite rate and
rhlthm control therapy. Therapeutic ablation of the atrioven
tricular node requires implantation of a permanent pacemaker.
These patients remain in AF and still require anticoagulation.
Management with rh1'thm control should be attempted flrst'
An implantable cardiorerter deflbrillator (lCD) (Option B)
improves sun'ir,al lvhen used for both primary and second
ary pre\€ntion of sudden cardiac death (SCD). Patients with
sustained ventricular arrhythmias (>30 seconds) or cardiac
arrest',t,ithout a reversible cause have a class 1 recommen-
dation for secondary prevention ICD placement. ICD place
ment is recommended for the primary prevention of SCD
in patients with ischemic or nonischemic cardiomyopathy,
ejection fraction less than 35'7,, and New York Heart Associ
ation functional class II or Ill heart failure. This patient with
AF and an ejection fraction of 45')(, does not ha\e an indica
tion for ICD theraPY.
In patients with AF who are at high risk for stroke
and have contraindications to anticoagulation therapy' left
atrial appendage occlusion (Option C) to prevent stroke and
systemic thromboembolism may be considered' Left atrial
appendage occlusion will not help control her arrhl'thmia
and is not indicated.
fEY POITI
. Aggfessive efforts to achieve rhythm control in
patients with atrial fibrillation and concomitant heart
failure decrease morbidity and mortality'
Bibliography
Januar} CL U'ann LS. Calkins I I. ct al. 2019 AHA ACC I IRS tbcused update
of the 201,1 AHA ACC llRS guicleline firr the management ol pirtients
u'ith atrial fibrillation: a report ot the American College of Cardiolop5,
American Heart Association Task I.brce on Clinical Practice Guidelines
and the Heart Rh1'thm Societ-r' in Collaboration With the Socielv ot'
Thoracic Surgeons. Circulation. 2019;l{O:el25-e151. [PMID: 306860{ll
doi:10.1161, CIR.0000000000000665
Item 115 Answer: D
Educational Objective: Diagnose acute aortic regurgita
tion complicating ascending aortic dissection.
Thc rnost appropriate cliiignostic tests are transthoracic
cchocardiographl, ancl CT angiographl' of the aorta
(Option D). This patient's presenting symptom ol acute
anterior chest pain suggests acute coronary syndromc,
pulnronary embolisnr. or aortic clissection. In this patient,
aortic dissection is most likely because of the significant
difierences in systolic bloocl pressures between the right
and left arms. In acldition, thc grade 3/6 decrescenclo
diastolic murmur at the left stcrnal border indicates the
presence ofaortic regurgitation (AR). In the setting ol sus
pccted aortic dissection. t he prcscnce of new or lr,orsenecl
AR suggests aortic root involvemcnt, rt'hich constitutes a
snrgical emergency. Transthoracic echocardiogruphy or
transesophageal echocarcliographl' ('fEE) is indicatecl lo
confirtt the presence. scvcrit),, and etiology of acute AR.
C'l angiography is indicrrled in patients with acute aor
tic dissection because it is highly accurate and rapidly
irvailable. TEE n.ray be used wlren CT imaging is unavail
iible and is helpful in asscssntent of aortic valve f'unction
befbre and after the surgicirl intcrvention.
i\cute coronarl' slnclronte, particularly ST elevtrtiorl
m1'ocardial infarction lnd high risk non ST-elevation
rn1'ocardial inf'arction, ntav be managed lvith cardiac
catheterization (Option A). Hor'r'ever, aortic dissection
is a nruch more likell' diagnosis based on the presencc
ol blood pressure discrepancl'' in the arms, AR murmur.
nondiagnostic ECG finclings. ancl indeterminate tropc)
nin measurement. Carcliac cirtheterization is relatively
contraindicated in acrtte ltortic dissection because <ll'the
possibilitl' of propagatitlg the dissection. Furthernlorc.
coronar\' :lrtery catheterization rvill not address aortic
clissection. Thus. if aortic clissection is being considercd,
O'l angiography is prelcrred to coronary artery catheter
ization.
In patients in lr'hom aortic dissection is suspected. C1'
ar.rgiographf is pref.erred to inr"asive aortography (Option B).
Aortograph!'is less sensitive than either TEE or CT angiog
rapl.rf in the diagr-rosis o1'aortic dissection. Invasive aortog
raphy should be considerecl only when the diagnosis cannot
be determined by noninvasivc imaging.
N,{agnetic resonance angiography (Option C) is rarely
usecl in the acute settillg t<l diagnose aortic dissection
because of patient instabilitl irnd the need for prolongecl
sclnnit.tg times. I-imitecl availabilitl' on an emergencl' basis
also r.nakes it less desirable thirn CT angiograph)l
tr
214

227. Answers and Critiques
o Echocardiography and CT angiography ofthe aorta are
indicated in patients with suspected aortic dissection
associated with acute aortic regurgitation.
o Aortic dissection with aortic root involvement,
including acute aortic regurgitation, constitutes a
surgical emergency.
Bibtiography
otto cM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the
management of patients with valvular heart disease: a report of the
American College of Cardiologl/American Heart Association Joint
Committee on Clinical Practice Guidelines. Circulation. 2o2l:743 rc7 2
e227. IPMID: 33332150] doi:10.1161/CIR.0000000000000923
Item 116 Answer: E
Ed ucatio na I O bjective: Diagnose pericardial effusion
with tamponade.
Transthoracic echocardiography (TTE) (Option E) is the
most appropriate diagnostic test. Symptoms of progressive
dyspnea may be caused by underlying pulmonary or car-
diac disorders, but this patient's physical flndings, including
a signiflcant pulsus paradoxus (24 mm Hg), tachycardia,
distant heart sounds. and crackles. as well as an enlarged
cardiac silhouette on chest radiograph, suggest the
life-threatening diagnosis of cardiac tamponade. Pulsus
paradoxus represents exaggerated ventricular interdepen
dence and is a key clinical feature of cardiac tamponade. It
is characterized by a fall in systolic pressure of greater than
10 mm Hg during inspiration. Pulsus paradoxus is not spe
cific for tamponade and must be interpreted in conjunction
with other clinical and echocardiographic features. If the
fluid has accumulated slowly, the cardiac silhouette is typ
ically enlarged on chest radiograph. This patient's findings
should prompt urgent evaluation for cardiac tamponade
and treatment if present. Echocardiography to evaluate fbr
the presence, distribution, and relative volume of pericardial
fluid is important to establish the diagnosis.
Right heart catheterization (Option A) might conflrm
hemodynamic findings consistent with tamponade, and peri
cardiocentesis could be performed within the cardiac cathe
terization laboratory. TTE to evaluate for a pericardial effusion
and tamponade should precede any invasive evaluation or
therapeutic procedure because it is safe and readily available.
'Ihis
patient has a lung mass that is likely malignant. with
associated malignant pericardial effusion. CI directed needle
biopsy (Option B), fiberoptic bronchoscopy (Option C), and/
or PET/CT (Option D) may all be indicated to refine the diag
nosis and guide treatment after the patient is stabilized, but
echocardiographic evaluation is needed immediately.
o Findings suggesting cardiac tamponade include pulsus
paradoxus, tachycardia, distant heart sounds, and occa-
sionally an enlarged silhouette on chest radiograph.
(Continued)
o Echocardiography is an essential tool in the diagnosis
of cardiac tamponade because it defines the presence,
distribution, and relative volume of pericardial fluid.
Bibliography
Imazio M, De Ferrari CM. Cardiac tamponade: an educational review. Eur
Heart J Acute Cardiovasc Care. 2020:2048872620939341. [PMID:
326280381 doi:LO.1177 I 2O48872620939341
Item 117 Answer: A
Educational Objective: Treat a patient with heart failure
and diabetes mellitus with a sodium-glucose cotransporter
2 inhibitor.
The most appropriate treatment is to add dapagliflozin
(Option A). This patient has heart failure, tlpe 2 diabe-
tes mellitus, and kidney disease. Evidence shows that the
sodium-glucose cotransporter 2 (SGLI2) inhibitors dapagli-
flozin, empagliflozin, canagliflozin, and ertugliflozin are
associated with a reduction in cardiovascular death or hos-
pitalization for heart failure in patients with type 2 diabetes,
and dapagliflozin and empagliflozin are effective in patients
without diabetes. In addition, for patients with type 2 diabe
tes, an SGLI2 inhibitor reduces progression ofdiabetic kid-
ney disease. SGLI2 inhibitors should not be used in patients
with type 1 diabetes, increased risk for type 2 diabetic keto-
acidosis, or rapidly declining or changing kidney function.
Glimepiride (Option B) is a second-generation sulfonylurea.
Although the rezults of many studies have been inconclusive, it
seems that the second-generation agents most likely do not have
any adverse cardiac eflects but also have no cardiac beneflt.
Among patientswith tlpe 2 diabeteswho have established
atherosclerotic cardiovascular disease (ASCVD) or established
kidney disease, the American Diabetes Association and the
American College of Cardiologz recommend an SGLT2 inhib-
itor or glucagonJike peptide 1 receptor agonist with demon
strated cardiovascular beneflt. In patients with type 2 diabetes
and ASCVD, Iiraglutide, semaglutide (injectable), and dula-
glutide have been shown to decrease cardiovascular death.
However, liraglutide (Option C) has no effect on heart failure
outcomes in patients with established heart failure.
Saxagliptin (Option D) is a dipeptidyl peptidase-4 inhib-
itor, a class of drug that has been shown to have no diflerence
in cardiovascular outcomes compared with placebo. However,
depending on the study, there was either a trend toward more
heart failure hospitalizations (saxagliptin) or an increased inci-
dence of heart failure (alogliptin). Because of this increased
incidence of heart failure hospitalizations, adding saxagliptin
as a second line agent would not be appropriate.
o Sodium-glucose cotransporter 2 inhibitors reduce risk
for worsening heart failure and cardiovascular death
in patients with heart failure with reduced ejection
fraction with or without type 2 diabetes mellitus.
(Continued)
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215

228. Answers and Critiques
Ut
E
lD
t^
o,
EL
l.l
.a
.D
UI
IEI POIXIS (orfiilrcd)
. Among patients with type 2 diabetes mellitus who
have established atherosclerotic cardiovascular dis-
ease or established kidney disease, a sodium-glucose
cotransporter 2 inhibitor or glucagon-like peptide 1
receptor agonist with demonstrated cardiovascular
disease benefit is recommended.
Bibliography
Wilcox T, De Block C, Schwartzbard AZ. et al. Diabetic agents, fiom met
formin to SCLI2 inhibitors and GLP1 receptor agonists: JACC focus semi
nar. J Am Coll Cardiol. 2020;75:1956 1974. [PMID: 32327107] doi:10.1016,'
j.jacc.2020.02.056
Item 118 Answer: B
Ed ucati ona I O bjeAive : Treat intermittent claudication
with revascularization.
The most appropriate treatment is revascularization (Option B).
This patient with peripheral artery disease (PAD) has quit
smoking, been adherent to a supervised exercise program,
and been treated with aspirin, cilostazol, and atorvasta
tin; despite these interventions, she remains symptom
atic, with lifestyle-limiting claudication. She is therefore
a candidate for revascularization, and because she has
worse symptoms and a lower ankle-brachial index on the
left, revascularization should be targeted to the left leg.
Experienced vascular specialists can accomplish recanal-
ization with angioplasty, atherectomy, stenting, and/or a
combination of techniques. Aortobifemoral bypass surgery
is reserved for patients with severe aortoiliac stenoses.
Aortoiliac disease most typically manifests as buttock, hip,
and, in some cases, thigh claudication.
Historically, pentoxifylline (Option A) was used in
patients with intermittent claudication; however, this med
ication has a high adverse-effect proflle, is not effective, and
is no longer recommended by guidelines.
In patients with tlpe 2 diabetes mellitus, sodium glucose
cotransporter 2 (SGLT2) inhibitors and glucagon-like peptide 1
receptor agonists reduce the rates of acute myocardial infarc-
tion, stroke, cardiovascular death, and, in the case ofSGLI2
inhibitors, heart lailure hospitalizations. These classes of
drugs are recommended as part of the glucose lowering reg
imen in patients with atherosclerotic cardiovascular disease
(ASCVD) or at high risk for ASCVD. The American College
of Cardiologr recommends that canagliflozin be used with
caution in patients with prior amputation, severe periph
eral neuropathy, severe peripheral vascular disease, or active
diabetic foot infections. In this patient, revascularization
will improve her symptoms, whereas stopping canagliflozin
(Option C) is neither necessary nor helpful.
A small study showed that patients with intermittent
claudication treated with ramipril had an improvement in
pain free walking time and maximal walking time com-
pared with patients treated with usual care. However, there
have not been additional studies that have shown ramipril
or other ACE inhibitors to be associated with improvements
in functional capacity or symptoms in patients with PAD.
ACE inhibitors and angiotensin receptor blockers may be
effective in reducing cardiac events in patients with PAD,
and there are no data showing preference for one over the
others. Switching from olmesartan to ramipril (Option D)
is not indicated.
XEY POIXT'
. Revascularization is indicated in patients with inter
mittent claudication and severe disability who have
not improved with optimal medical therapy and
structured exercise programs.
r Canagliflozin and ertugliflozin should be used with
caution in patients with severe peripheral artery disease
because of an increased risk for amputation.
Bibliography
Gerhard Herman MD, Gornik tJL, Barrett C, et al. 2016 AHA/ACC guideline
on the management of patients with lower extremity peripheral artery
disease: executive summary: a report of the American College of
Cardiobs//American Heart Association Task Force on Clinical Practice
Guidelines. J Am Coll Cardiol. 2Ol7:69:1465 1508. [PMID: 27851991]
doi:10.1016/j.jacc.2016.11.0O8
Item 1 19 Answer: D
Educational Objective: Evaluate a patient with chest
pain and left ventricular hypertrophy with exercise
myocardial perfusion imaging.
Thc most appropriate cliagnostic test is exercise ntyocardial
perfusion in,aging (Option D).
'Ihis
patient presents with
cl.rest pain that is concerning ftrr ischemic hcart disease. and
carcliac stress testing is indicated. Stress testing to evaluate
f<rr con-rnary artery disease should aln,:rys be perforn'red u'ith
exercise, unless exercisc is contraindicated or the paticnt
is unable. Exercise capacit-v is a polterlul predictor of out
comes: individuals unable to achicve 5 nretabolic equiv
alents. or the first stage of a Bruce protocol. have higher
all cause nrortalitl,. Stress testing with imaging is indicated
in patients u,ith an inability to exercise, baseline ECG abnor
malities that limit intcrpretation of the exercise ECG, or
indeterminate findings ot-t the exercise EC(1. Exercise myo
cardial perfusion imaging provides a visual assessment of
relatir,e my'ticardial blood flow (using a radionuclide tracer)
bl,comparing images in the resting and stressed states. Jhis
test is thc most appropriate option lbr this patient !\.ith lell
ventricular hypertrophy r,r'ith a strain pattern on baseline
E(.G that u'ill impair the interpretation of an exercisc ECG.
Exercise is preferred over pharmacologic stressors.
Dobutanrine. like exercise. increascs ml,ocardial oxygen
dernand and elicits ischemia because of insulficient perlu
sion to the aff'ected myocardium. Exercise rnvocardial per
firsion imaging is preferred to a d<tbutamine based strcss
tcst u.ith either echocardiography or tnyocardial perfusion
imaging (Options A, B).
Exercise ECG (Option C) is the test of choice fbr most
patients ur-rdergoing ir.ritial evaluation for chest pain thought
to be of cardiac origin, unless there are c<lntraindicatiot.ts.
tr
216

229. Answers andg-']-t!gy9:
FFI
(lt.rntrainclications include an inability to exercise ancl
lll 1',rr"1i,'," ECG abnornralities (e.g., S'f segment clepression
c0NT
>l nrnr, lelt bunclle brarrch block. left ventricular hypertro
ph1,. paced rhythnr. or preexcitation).
'lhis
patient's chesl
plin in lhe presence of left ventricular hypertrophy will be
belter ilssessed r,r,ith exercise myocardial perfusion inragir.rg
becuuse the tiCG chilrges associaterl with left vetrtricular
hypertrophy r,vill obscurc the presence ol llCG ischcnric
chunges.
TEY POI]IIS
o Stress testing to evaluate for coronary artery disease
should always be performed with exercise, unless
exercise is contraindicated or the patient is unable.
. Stress testing with imaging is indicated in patients with
an inability to exercise, baseline ECG abnormalities
that limit interpretation of the exercise ECG, or inde
terminate findings on the exercise ECG.
Bibliography
Fihn SD, Blankenship JC, Alexander KB et al. 2014 ACC/AHA/AATS/PCNA/
SCAI/STS focused update ofthe guideline Ibr the diagnosis and manage
ment of patients with stable ischemic hearl disease: a report of the
American College of Cardiologr/American Heart Association Task Force
on Practice Cuidelines. and the American Association for Thoracic
Surgery Preventive Cardiovascular Nurses Association, Society fbr
Cardiovascular Angiography and Interventions, and Society of Thoracic
Surgeons. Circulation. 2014;1301749-67. [PMID: 25070666] doi:1o.1161/
cl R.0000000000000095
Item 120 Answer: A
Educational Objective: Diagnose atypical chest pain.
This patient's symptoms are most consistent with atypical
angina (Option A). The structured evaluation of patients
with chest pain includes a focused history regarding the
duration, type, and associated characteristics of discomfort.
Stable angina pectoris, deflned as reproducible discomfort
of the chest, neck, or arms of at least 2 months' duration,
can be further classifled according to three components:
1) the quality and duration of discomfort, 2) provocation
by exertion or emotional stress, and 3) relief with rest or
nitroglycerin. Typical qualities of angina include squeezing,
tightness, pressure, constriction, fullness, heaviness, and
weight. Angina typically resolves when the inciting factor
is removed. Most episodes of stable angina last 2 to 5 min
utes, not seconds or 20 to 30 minutes. The degree to which
a patient's symptoms match these components reflects the
pretest likelihood of coronary artery disease (CAD) as the
underlying cause of the symptoms. Although she meets
two criteria for typical angina (quality and duration of dis-
comfort, provocation by exertional or emotional stress), her
symptoms resolve without rest; therefore, she has atypical
chest pain, with an intermediate pretest probability of CAD
as the cause of her symptoms.
Patients with only one or none of the features of typical
stable angina pectoris are classified as having nonanginal
or noncardiac chest pain (Option B). Such patients have a
corresponding low likelihood of symptomatic CAD.
Patients are classifled as having tlpical angina (Option C),
with an associated high likelihood of CAD, if all three com-
ponents of stable angina are present.
This patient's ongoing and unvarying exertional symp-
toms are not consistent with a classiflcation of unstable
angina (Option D), which is characterized by angina that
is new in onset, occurs at rest or with little exertion, or is
worsening in severig or frequency. Unstable angina is asso-
ciated with increased short-term risk for acute myocardial
infarction.
XEY POIXI
o Stable angina pectoris can be classified according to
three components: 1) the quality and duration of dis
comfort, 2) provocation by exertion or emotional
stress, and 3) reliefwith rest or nitroglycerin, and the
degree to which symptoms match these components
reflects the likelihood ofcoronary artery disease being
the underlying cause.
Bibliography
Katz D, Gavin MC. Stable ischemic heart disease. Ann Intern Med. 2019:
17t:lTCt7 ITC32. IPMID: stlAzZes] doi:10.7326lAITC2ol908o60
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217

231. t
t
Index
Note: Page numbers followed by fand t denote figure and table, respectively.
Test questions are indicated by Q.
A
Abdominal aortic aneurysm (AAA),1O1 1O2, i01t, Q42
ACE inhibitors
for ACS, 24, 25t, 27
angioedema and, 32
cough induced by, 32
for heart failure, 32-34, Q70
during pregnancy, 71 6, 777 t
for stable angina, 16
Aclarubicin, cardiotoxicity of, 1131
Acute aortic syndromes, 102 106, 102f
Acute coronary syndrome (ACS), 17-28
care after, 28
diagnosis of, 18f
general considerations, 17-18
medical therapy for, 24-26, 25t
non ST elevation, S, Q20
without obstructive CAD. 27 28
Acute decompensated heart failure, 38-40
Acute limb ischemia, 111, Q46
Acutemyocarditis,41 42
Acute pericarditis, 84-86, 851 111-112
Adenosine, 47t, 116, l17t
Advanced refractory heart faiure,40 41
Alcohol septal ablation, 80
Aldosterone antagonists
for ACS, 25t. 27
contraindications to. 116
for heart failure, 34, Q38
for HFpEE, 38
side effects of. 34
Aliskiren during pregnancy, 117t
Alteplase in STEMI, 21
Amiodarone, 47t, 57 , llTt
AmJodipine for hean failure.35
Ampicillin in infective endocarditis, 76t
Andexanet alfa. 53
Angina. See olso Stable angina pectoris
coronary revascularization for, 16-17
evaluation of, 13, Q88
Angioedema, 32,41
Angiography
coronary 5t,9, Q24, Q105
cT, 3, st,100t, Q4, Q42, Q101
with FFR,9
magnetic resonance, 1001
radionucleotide. 11t
Angiosarcomas, S3 84,841 Ql4
Angiotensin receptor neprilysin inhibitor (ARNI), 32 33, Q1
Angiotensin receptor blockers, 251, 27 ,32-35,116, llTl
Ankle brachial index, 108, 108t, 1091 Q28
Anthracycline toxicity, 112 113
Antianginal medications, 16, 26 27 ,26t
Antiarrhythmic drugs, 46-48, 47t
Anticoagulation therapy. See olso Specific Anticoagulants
for ACS, 26
in acute limb ischemia, Q46
after coronary revascularization, 17
for atrial fibrillation, 17, 52-54, 53t
mechanical mitral valves and, Ql00
for peripheral artery disease, Q33
during pregnanry, 116-118, 1181
Antiplatelet agents for ACS, 24,25t,26
Anxiery cardiovascular disease and, 1
Aorta
coarctation, 931, 96-97, 96f
diseases of. 99-106
dissection,103,105f,116, Q16, Q62, Q78, Q115
Aortic atheromas. l02
Aortic regurgitation, 62t, 64t,67-68, Q78, Q109, Q115
Aortic root dilation, Q87
Aortic stenosis, 62t, 63 67 , 64t, 651
aortic valve replacement for, 66, Q8
diagnosis of, 63-66. Q53
low flow, low gradient,
Q90
radiation related. 112
Aortic valve replacement, 66, Q72
Aortogmphy, l00t
Apixaban,53,53t, l17t
Arrhythmias, 43- 61. See olso Atrial fibrillation; Bradycardia; Palpitations;
Vent ricu lar arrhythmias
diagnostic testing for, 10 13
implantable electronic devices, 45t
Arrhlthmogenic ri ght ventricular cardiomyopathy/dysplasia (ARVC/D),
57. 58t
Arsenic, cardiotoxicity of. 1131
Arterial insufficiency ulcers, 107f
Ascites, 38
Aspirin
for ACS, 24, 25t
after coronary revascularization, 17
after percutaneous coronary intervention, Q36
in diabetes, 29
for PAD, 110, Q33
during pregnancy, 1l7t
for stable angina pectoris, 14
Atenolol, 25t, 47t, 116, 177 t
Atherosclerotic cardiovascular disease (ASCVD)
diabetes and, Q79, Q93
risk of, 2, Q19
testing for, 3 l0
Atherosclerotic ulcers, lo3, l04f
Athlete heart, diagnosis of. 78, 78t, Q75
Atorvastatin. 14
Atrial fibrillation, 50 55,51f
anticoagulation therapy for, 17, 52 54, 53t
chemotherapy related. ll3l
embolic stroke and, Q99
hypertrophic cardiomyopathy and, 77
ibrutinib-related, Q97
management of, 51 55,
Q11, Q107
nonpharmacologic approaches, 54
paroxysmal,
Ql14
in restrictive cardiomyopathy, 82 83
stroke prevention in. Q83
stroke rates and. 52t
suDcllnlcat.54 55
Atrial flutter, 55, 551, Q32
Atrial myxomas, 83, 831; Q80
Atrial septal aneurysm, 9l
Atrial septal defects (ASDs), 63t,91-94,92f, Q56, Q1i3
Atrioventricular block,22.43,43f, 441 45, 80, 113t
Atrioventricular nodal reentrant tachycardia (AVNRT), 49, Q25
Atrioventricular reciprocating tachycardia (AVRT), 49-50
Atropine,45, Q4.1
Azithromycin, 76t
B
B-type natriuretic peptide (BNP), 30-31, 37-39
Belinostat, cardiok)xicity ol 1131
Bendopnea,3l
Benign flow murmur,63t
p-Blockers
forACS,24,25t,26 28
contraindications to, l6
dosages, 34t
function of. 16
heart rate control and,42
for HFTEF. 33- 34, 34t
219

232. lndex
p-Blockers (Continuedl
in peripheral artery disease, 109 110
during pregnancy, U6, 1l7t
for premature ventricular contractions, Q55
for stable angina, 16
titration in HFTEF, Q45
Betrixaban during pregnancy, lut
Bevacizumab,114
Bicuspid aortic valve disease, 68, 99, Q34, Q101
Biguanides,3St
Bisoprolol, 16, 25t,34,34t, 47t
Black patients. See olso Ethnicity, cardiovascular disease and
amyloidosis in, 81
heart failure in,41
isosorbide dinitrate-hydralazine in, 34, Q31
peripartum cardiomyopathy in, 115-116
Bortezomib, cardiotoxicity of, u3t
Bradycardia,43 45,113t, Q2, Q44
Breastfeeding, 116
Bruce protocol, 6
Brugada syndrome, 57, 58t,591
Q71
c
CAD. See Coronary artery disease (CAD)
Calcium channel blockers, 16, 261, 35, 68
Canagliflozin for heart failure, 35
Capecitabine, cardiotoxicity of, l13t
Captopril, 22, 1171
Cardiac amyloidosis, 10, 81-82, 81f, 81t
Cardiac catheterization, 111, 23, 89, Q85
Cardiac magnetic resonance (CMR) imaging, 5t,8, 111, 31
in infiltrative cardiac disease, 81, Q7
mitral regurgitation evaluation with, Q84
in structural cardiac disease, 10
ventricular tachycardia evaluation with, Q89
Cardiac rehabilitation, 28, 37, Q86
Cardiac resynchronization therapy (CRT), 36,45t, Q77, Q111
Cardiac syndrome X, 27
Cardiac tamponade, 85t, 86-88, 87f
Cardiac tumors. 83 84
Cardiogenic shock, 22, 39-40, 39t, Q98
Cardiomyopathies, 41 42
peripartum, 115-116
Cardiorenal syndrome, 38
Cardiovascular disease (CVD)
calculating the risk of 2
cost of 1
epidemiologl of, 1-3
risk factors for, I 2
Cardioversion, 51-52
Carfi lzomib, cardiotoxicity ol 113t
Carvedilol, 16, 251, 34t, 47t, 80, Q45
Catecholaminergic polymorphic VT, 58t
Catheter ablation, 49,54, 55, Q32
Cefazolin. T6t
Cephalexin, T6t
Cerebral aneurysm, 96
Chemotherapy, cardiotoxicity ol 112-114, 1l3t
Chest CT with contrast. l1t
Chest pain
atypical, Ql20
coronary cT angiography in, Q4
evaluation ol 13, Q57
ischemic, Ql05
microvascular dysfunction and, Q24
myocardial perfusion imaging in, Q1l9
Chest radiogaphy, 31, 311 85t, 871 89, 89f
Cholesterol levels, 1, 14, 15
Chronic limb threatening ischemia (CLil), 106-107,
Q1O2
Cilostazol,110, Q3
Cisplatin, cardiotoxicity of, 113t
Clarithromycin, 16, 76t
Clopidogrel
for ACS, 251, 26
after percutaneous intervention, Q36
in aspirin intolerance,
Q12
characteristics of, 21t
discontinuing, Q65
for PAD, u0, Q33
during pregnancy, lut
for stable angina pectoris, 14,
Q12
in STEMI.2I
Cloxacillin. T6t
Clubbing,95,98
Complete heart block, 22,43
Congenital heart disease
adult,90-99
c,"notic, 98-99, Q73
Connective tissue disorders. 99t
constrictive pericarditis, 851, 88-90, 90f, Q58
Continuous positive airway pressure (CPAP), 36
Contraceptives, 91
Contrast media, risks ol 9
Coronary angiography, 5t. See olso Angiography
in ischemic chest pain,
Q1O5
microvascular dysfunction on, Q24
risks of. 9
in stable angina pectoris, 16, Q41
Coronary arteries, visualization ol 8 9
Coronary artery bypass graft (CABC) surgery 17, Q6, Ql12
Coronary artery calcium (CAC) scoring,3, 5t, 9
Coronary artery disease (CAD), 13-30, 14f
atherosclerotic, 3-10, Q19
alrial nbrillation and. Ql07
diagnosis ol 3 9,13,14f, Q67
evaluation of, Q85
multivessel, Q6
premafure,2
pretest likelihood of, 13, 13t
radiation associated, 112, Q76
risk ofdiagnostic testing, 9 10
stress testing for, 3-9, 31-32
Coronary revascularization, 16-17 , Q29
Coronary vasospasm, 27, 29, Q95
Crackles, 38
CT angiography, 5t, llt, Q4.
See olso Angiogaphy
AAA localization using, Q42
bicuspid valve examination using,
Q101
thoracic aortic imaging, l00t
qanotic congenital heart disease, 98-99, Q73
Clclophosphamide, 1131
Cyclosporine,4l
Qrtomegalovirus
(CMV), 41
D
Dabigatran, 53, 531, 1171
Dapagliflozin for heart failure, 35, Q117
Decompensated heart failure, 38 40, Q15
Delta wave, 49f
Depression, CVD and, 1
Dexrazoxane. 114
Diabetes mellitus
atherosclerotic cardiovascular disease and, Q79
CAD in.29-30
cardiovascular disease risk and, 2
heart failure prevention and, 37, 41
in peripheral artery disease, 109
risk reduction, 1
Diastolic dysfunction, 30
Diastolic heart sounds, 89t
Digoxin, 35, 471, 117t
Diltiazem, 16, 47t, 117 t
Dipyridamole, T, 117t
Direct oral anticoagulant (DOAC), 17, 52 53, 53t,
117t,118
Direct renin inhibitors, u6
Disopyramide,80, lut
Diuretic therapy
clinical traiectories, 39f
for heart failure, 34, 37-38
during pregnancy, 1171
for pulmonary vascular congestion, 22
Dobutamine.39t
Dobutamine cardiac magnetic resonance imaging, 5t
Dobutamine echocardiography, 4t, 10
Dobutamine myocardial perfusion imaging, 4t
Docetaxel, cardiotoxicity of, 1131
Dofetilide, 471, 48
Dopamine,39t
Doxorubicin. ll3t
DPP-4 receptor antagonists, 35t
Dronedarone, 47t, 48
Dual antiplatelet rherapy (DAPT). 17, 26, Q36, Ql04
Duke Treadmill Score. 6
220

233. lndex
t
i
t
t
t
i
t
t
\
\
j
L
i
I
L
t
\
I
I
I
i
t
I
t
I
\
i
\
;
I
I
)
i
I
h
I
t
i
i
I
I
i
i
:
I
Dyslipidemia, 1, 109
Dyspnea, causes ol 31
t
Echocardiography, 1O, 11t
in aortic stenosis. 661
evaluation of palpitations using,
Q30
in heart failure, 31, 36
pericardial effusion on, 84
screening for myocardial dysfunction, e40
Edoxaban,53t
Ehlers Danlos syndrome, 99
Eisenmenger syndrome, 93t, 94, 9g 99
Elderly patients, CAD in, 28 29
Electrical alternans. 85r, 87, 87f
Electrocardiography, 4t, 6, 10, 121. See olso Specilic conditions
Electrophysiolory study, 12t
Empagliflozin, 35, Q10, Q93
Enalapril,25t, 33, 117t, Q70
Endocarditis
antibiotic therapy, 76t
infective, 74-76, Q18, Q72
prophylaxis, 75-76, 761, Q108
Endovascular revascularization, 110 111
Enoxaparin, 21, 26
Epirubicin, cardiotoxicity of , ll3t
Eplerenone,2T, 34
Erlotinib, cardiotoxicity ot, 113t
Esmolol during pregnancy, 117t
Etaracizumab, cardiotoxicity ol 113t
Ethnicity, cardiovascular disease and, 2-3
Exercise electrocardiography, 4t, 6, 6t, 72I, Q43, Q67
Extracorporeal membrane oxygenation (ECMO), 22
F
4-factor prothrombin complex concentrate, 53
Felodipine for heart failure, 35
Figure 3 sign, 96, 96f
Flecainide, 46-4a,47t
discontinuing, Ql10
during pregnancy, llTt
toxicity,46f
Fluid overload, 30, 38-40
Fluid retention, 30
5 Fluorouracil. 113t
Fondaparinux, 21, 26
Fractional flow reserve (FFR), angiography with, 3, 5t, 9
Frailty, definition of, 61
Furosemide,34, Q15
G
Cadolinium contrast, 9, 86f
Gemcitabine, cardiotoxicity ol 1131
Gentamicin in infective endocarditis, 76t
Giant cell myocarditis, 42
Glucagon-like peptide I (GLP 1) receptor antagonists, 29, 35t, Q79
Glucocorticoids, for recurrent pericarditis, 86, Q35
Glycemic control, 29
Glycoprotein IIb/llla inhibitors, 26
GRACE risk score, 23
Guideline-directed medical therapy (GDMT),
Q26, Q33
for heart failure, 331 35-36, 57, 66
for stable angina, 14, l5l l7
for ventricular dyslunction, 72
H
H2FPEF risk score, 3l
Heart block, transient, Q81
Heart failure (HF), 30 42
ACE inhibitors for, Q70
aldosterone antagonists fbr, Q38
atrial fibrillation treatment in,
Q114
diagnosis of, 30 32
heart transplantation for, Q22
management of,32-41, Q52, Q1o6
new-onset, Q85
pathophysiolos/ of, 30
in pregnancy, 116
right sided,88
screening tbr, 30
severity ol 32, 32t
SGLT2 inhibitor for, Q10, Q117
treatment with CRT, Q77, Q111
Heart failure with preservecl ejection fraction (HFpEF), 37-38, e3t, e82
Heart failure with reduced €jection tiaction (HFTEF). 30,32 36,33f, el, e4S
Heart rate control. 52
Heart sounds, 89t
Heart transplant, Q22, Q66
for cardiac amyloidosis, 81, 8l t
tbr heart failure, 32t, 36-37. 40 41. 116
infections and. 74
for m),ocarditis, 42
for restrictive cardiomyopathy. 82, Q66
Heparin for ACS, 26
HIV CAD risk in, 3
Holter monitors, 10, 12t
Hydralazine during pregnancy, 1171
Hypercalcemia, 19
Hyperkalemia,34
Hypertension
cardiovascular risk and, I -2
chemotherapy related. lt3t
heart failure prevention and, 37
Hypertrophic cardiomyopathy (HCM),57,77 81. Q50. QI03
diagnosis of, 4t, 10, 77 78, Q23
ECG findings in, 77, 781'
evaluation of, 63t, 77 78
imaging of, 79f
presentation, 77
risk stratification. 78 79
sudden cardiac death and, 58t, 78, 79t
I
Ibrutinib, 1131, Q97
Idarubicin, cardiotoxicity ol, I l3t
ldarucizumab. S3
ldiopathic pericarditis, 86
Ifosfamide, cardiotoxicity ol; I I 3t
lmplantable cardioverter delibrillators (lCDs), 22, 36, 45t, 7tt 79, Q9, Q103
Inappropriate sinus tachycardia (lST), 48
Infections
endocarditis, 7 4 -7 6, 7 6t, Ql 8, Q72
implantable cardiac devices. 61, Q64
lnfective endocarditis (l E), 74-76
Duke criteria. 75t
evaluation of, Q18
treatment ol 76t, Q72
Insulin in heart failure, 35(
Intermittent claudicatkrn. 106-107, 110, Q3, Q86, QtlS
lntra-aortic balloon pump (IABP), 22, 7l
lntramural hematoma, 103, 103f, 104
lodinated contrast material. 9
Iron deficiency, 35, Q73
Iron therapy for hearl lrrilure. 35
lschemia
chemotherapy related. ll3t
evaluation for. 31 32
on exercise ECG. 6 7
limb threatening, 106 108, Ql02
lschemic cardiomyopathy, 57
Isosorbide dinitrate hydralazine, ll4,
Q31
Isosorbide mononitrate. 34
lvabradine for heart failure. il4
I
J
Jugular venous distention. 38
x
Kerley B lines, 31
Kidney disease, risks ofcontrast media in, 9
Kussmaul sign, 82, 89
L
hbetalol during pregnancy, llTt
lactation, drugs during, ll6. llTt
Leadless pacemakers, 45t, 46f'
Left bundle branch block. 6,7, 36. 56f. Q57
Left ventricular assist devices (LVADs), 22,
.10,
Ql06
Left ventricular ejection fiaction (t.VEF). 30
Left ventricular hypertrophlr .5t. 6. .531, 81,
Q119
Left ventricular outflow tract obstructlon, 77. 77t
Left ventricular free wall rupture, 22
Left ventricular wall thickness, 78t
Leucovorin, cardiotoxicity of, ll3t
Lidocaine,4Tt, u7t
221

234. lndex
Lifestyle, cardiovascular risk and, 1
Limb ischemia, 106, 110, 111, Q46
Lipid lowering ther^py, 14, 27
Lisinopril, 25t, 117t
Long QT syndrome, 57, 581 58t,113t
Loop diuretics for heart failure, 34, 38, 39f
Looping event recorders, 10, 12t, 13
Low cardiac output, 31
m
Magnetic resonance angiography, 100t, l08t
Marfan syndrome, 99-101, 106, 116,
Q87
Mechanical circulatory support, 40
Metabolic syndrome, 2
Metformin in heart failure. 35t
Metoprolol, 16, 34t. 47 I, 117 I
Mexiletine.4Tt
Microvascular dysfunction, 27, 29, Q24
Milrinone,39t
MINOCA (MI in the absence of obstructive CAD). 27
Mitoxantrone, cardiotoxicity ol, 1131
Mitral inflow velocity, 88f
Mitral regurgitation. 62t, 641. 7 O 72, 7 2t
CMR imaging in, Q84
mitral valve repair in, Q63
treatment ol Q26
Mitral stenosis, 62t, 64t, 68-7O, 69f. Q43
Mitral valve clipping, 73f
Mitral valve prolapse, 70, 71f
Mitral valve repair, 72, Q63
Mitral valves, mechanical. 73, Q100
Mobile cardiac outpatient telemetry 12t
Multi-Ethnic Study of Atherosclerosis, 2
Multifocal atrial tachycardia, 50, 50f
Myocardial disease, 77 8,1
Myocardial fibrosis, radiation and, 112
Myocardial infarction (MI), 17 28
heart block after, Q81
hereditary factors in, 2
non ST elevation,23 24, Q98
in pregnancy, 116
with right ventricular involvement. Q51
ST elevation. 18 23
Myocardial injury interpretation ol 18f
Myocardial interstitial librosis, 63
Myocardial perlusion imaging (l\{PI).7,
Q88, Q119
Myocardial viability study, T 8,8t
Myocarditis,4l ,12
il
Nadolol.4Tt
Neprilysin, inhibition of, 32-33
Nitrates, 16, 24, 26-27, Q96
Nitroglycerin, 16, 26t, 39t
Nivolumab, cardiotoxicity of, 113t
Nocturnal bradycardia, 43
Nondihydropyridine calcium channel blockers, 26t, 35, 47t, 48
Non ST elevation acute coronary syndrome (NSTE ACS).17 l8
23 24,23t,Q2O
management of, Q59
risk stratiflcation in, 23
treatment of, 23 24,24f,Q1O4
Non ST elevation myocardial infarction,23 24, Q98
Nonsteroidal antiinflammatory drugs, 86, Q35
Noonan syndrome,96
Norepinephrine. 39t
0
Obesity, CVD risk and, 2
Ostium primum ASD,91, 93t
Ostium secundum ASD, 91, 93t, Q56
Oxacillin. T6t
Oxiplatin, cardiotoxicity ol, 1l3t
Oxygen. supplem ental, 24, 26
P
P2Yu inhibitors, 2lt, Q36
Pacemakers.45t
infections, Q64
leadless, 45t,46f
for symptomatic bradycardia, Q2
transvenous. 45t
Paclitaxel, cardiotoxicity oi 113t
PAD. See Peripheral artery disease (PAD)
Palpitations, 1o. Q30, Q60
Papillary fibroelastomas, 83
PARADIGM HF trial,33, Ql
Patent ductus arteriosus (PDA), 95, Q27
Patent lbramen ovale (PFO), 91, 9lf, 93t, Q48
Patient triggered event recorders, l2t
Percutaneous coronary intervention (PCI), 17, 20-21
Pericardial calcifi cation, 891
Pericardial disease, 84 90, 84t, 85t
Pericardial efTusion, 84. 86-88, 88f, Q116
Pericardial friction rub. 84
Pericardial thickening, 90
Pericardiocentesis. 86
Pericarditis,84 86
acute,84 86,851 1ll 112, Q94
constrictive, 851, 88-90, 90f, Q58
idiopathic, 86
recurrent, Q35
restrictrive, 89f
Peripartum cardiomyopathy, ll5 l16, Q13, Q70
Peripheral artery disease (PAD), 106 ll1
asymptomatic. Q69
cardiovascular risk reduction in, Q21
cilostazol fbr intermittent claudication, Q3
clinical presentation, 106-107
diagnosis of. Q28
imaging, loSt
interventional therapy, 110 lll
medicaltherapy, 109 110
physical examination in, 107t
revascularization in,
Q118
statin therapy in, Q54
treatment with GDMT, Q33
Peripheral edema, 38
PET/CT,5t,9
Phenytoin during pregnancy, 117t
Platypnea-orthodeoxia syndrome. 91
Plebotomy,98
Pooled Cohort Equations (PCE), 2
Postural orthostatic tachycardia syndrome (POTS). 48 49
Prasugrel, 21t.25t,26
Pregnancy
anticoagulation therapy during, 116-118
p blockers during. l16
cardiovascular disease and. 114 118
drugs during, 1171
Marfan syndrome and, Q87
normal signs in, 1151
pathobgic signs in, l15t
peripartum cardiomyopathy, Qi3, Q70
pulmonary stenosis and. 96
warfarin during, Q68
Premature atrial contractions (PACs), 50
Premature ventricular contractions (PVCs), 55 57, Ql7, Q55
Presyncope, diagnostic testing in, 10
Procainamide during pregnancy, i17t
Propafenone, 16 48, 47t, 48f , 117t
Propranol,4Tt, 117t, Q55.
See olso p-Blockers
Prosthetic valves, 73 74, Q68
Pseudoclaudication. 107t
Pulmonary balloon valvuloplasty, 95-96
Pulmonary regurgitation, 621, 97, Q92
Pulmonary stenosis (PS). 62t, 95 96
Pulmonary vascular congestion, 22
Quinidine during pregnancy, 1171
R
Radiation therapy, 111, 111t, Q76
Radionucleotide angiography (MUCA), 11t
Ranolazine. 16
Renin-angiotensin aldosterone system (RAAS), 30, 32
Restrictive cardiomyopathy. 82 83. 9Of, Q66
Reteplase in STEMI, 21
Retroperitoneal hemorrhage, 9
Reynolds risk score, 2
Rheumatoid arthritis, 3
Rhythm control,42, 54-55, 80, 82,
Q11, Q114
Rifampin, T6t
t
222

235. lndex
Right ventricular infarction, 22
Rivaroxaban, 53, 531, 54, 110, 1171,
Q21
Rosuvastatin, 14, 251, 35
s
Sarcoidosis.42
Septal myectomy, 80
Septal reduction therapy (SRT), 80, Q50
Sexual activity after ACS, 28
Shone syndrome, 96
Silent myocardial ischemia, 28
Sinus bradycardia, 43, Q2
Sinus tachycardia, 48-49
Sinus venosus ASD, 91, 93t
Sleep disordered breathing, 36
Smoking cessation, 37, 109, Q39
Sodium nitroprusside, 391, 117t
Sodium-glucose cotransporter 2 (SGL12) inhibitors
for ASCVD, 29, Q93
for heart failure, 35, 351, Qlo, Ql17
Sorafenib, cardiotoxicity of, l13t
Sotalol,47t,48, 1ut
SPECT imaging, Tf
myocardial perfusion, 4t, 7t
risks of, 9
Spironolactone for heart failure, 34, 38
Spontaneous coronary artery dissection (SCAD), 27,116, Q47
Stable angina pectoris, 13-17, 13t
CABG beneflts in, Q112
diagnosis ol 13 14
treatment ol 14-16, 15f, Q12, Q41
Statin therapy, 74,24, 25t,27 ,28, 35,109, Q54
ST elevation myocardial infarction (STEMI), u-23
complications of, 21 23
ECGfindingsin,fi la,2of
management of, 19t, 20 21, Q29
recognition of, 18-20
thrombolytic therapy, 21, Q7 4
Stress echocardiography, 41, 7, 7t, Q90
Stress testing, 3, 4t, 5 8, 31-32
in aortic stenosis. 65
contraindications to. 9
interpretation of, 7t
risks of, 9
Stroke
anticoagulation for prevention ol 52 54, Q83
atrial fibrillation and. 50 51
embolic, Q99
patent formen ovale and, Q48
risk stratiflcation, 52
Structural heart disease, 10, Q49
Sudden cardiac arrest, 59, 60f, 61
Sudden cardiac death, 28, 36, 57 -61,7a 79,79t, Q9, Q103
Sulfonylureas in heart failure. 35t
Sunitinib, 1131,114
Supraventricular tachycardias (SVTs), 19-20. 49-50, 116, Q25
Syncope, diagnostic testing in, 10
Systemic inflammation, CAD risk in,3
Systemic lupus erythematosus, 3
Systolic dysfunction, 7, 30, 115
Systolic murmur, 10, 621, Q5
T
Tachycardia mediated cardiomyopathy, 42
Tachycardias, 45-50
Tacrolimus,4l
Takotsubo cardiomyopath]: 27 28 , 41 , 42f, Q67
Tamponade, cardiac, 85t, 86-88, 871 Q1l6
99m-Technetium pyrophosphate scintigraphy, 10, 11t, 81, 81f
Tenecteplase in STEMI, 21
Tetralos/ of Fallot,93t,97 98,97f, Q92
Thalidomide, cardiotoxicity of, 113t
Thiazide diuretics. 34
Thiazolidinediones, 35t
Thoracic aortic aneurysm (TAA), 99-101, 991, 1001, Q91
Thoracic endovascular aortic repair (TEVAR), 101-102, 104
Thoracic irradiation, U1, l1lt, 112f
Three-dimensional echocardiography, l1t
Thrombolytic therapy. lgf , 21, 22t
Ticagrelor, 211, 251, 26, 53, Q36
Tobacco use, CVD and, 1
Torsades de pointes, 1131
Transcatheter aortic valve implantation (TAVI). 66, 67f, Q8
Transesophageal echocardiography (TEE), 10, lof, llt, 67,74,91,
100t, Q18
Transthoracic echocardiography (TTE), 10, 11t
in aortic regurgitation, 67-68
in bicuspid aortic valve disease, 69
in cardiac tamponade, 87, Ql16
in hypertrophic cardiomyopathy, 77 78
in infective endocarditis, T4 75
in mitral regurgitation,To 72
murmur evaluated using, Q49
in thoracic aortic disease, 10ot
Transthyretin amyloid (ATTR) amyloidosis, 10, 81, 811: Q7
Tranwenous pacemaker, 45t
Trastuzumab, 113t, 114, Q40
Tricuspid regurgiralion, 62t, 72 7 3
Tricuspid stenosis, 621, 73
Tricuspid valve disease, T2 73
Troponin levels, 8, 17, 181 39
Tuberculosis.85 86
Tumor plop, 83
Turner syndrome, 96
Unfractionated heparin, 21, 26, 111, Q46
U
Valsartan sacubitril,32 33,38, Ql
Vahular heart disease (VHD), 61 76, 62t,63r,641
Vancomycin, T6t
Vandetanib, ll3t
Vasculitis. 99t
Vasodilator myocardial perfusion imaging, 4t, 9, Q57
Vasodilator single-photon emission CT, Q57
Vasopressin, 39t
Venous I h romboembolism. l13t
Ventricular arrhlthmias, 55 57
Ventricular fibrillation, 22, 60f
Ventricular septal defect (VSD), 23, 63t, 931, 94, 94f, Q5
Ventricular tachycardia, 46, 56-57
after reperfusion, 22
chemotherapy related. I l3t
diagnosis of, Q37
evaluation of, Q89
during MI, 22
Verapamil, 16, 26t, 35, 47t, 57, 80, 1171
Volume overload, signs ol 31, 38
Volume status, assessment ol 38-40
w
Warfarin, 52, 53, 53t
in atrial fibrillation, 17, 52, 53, 53t
mechanical mitral valves and, 74, Qi00
in peripheral artery disease, 110
during pregnancy, 116-118, 118t
Water boftle heart, 85t, 87f
Weight loss, heart failure prevention and, 37
Wenckebach heart block, 22.43,43f
Women
CAD in,29
CAD risk in, 3
congenital heart disease in,90 91
223