Introduction to cardiomyopathy lec 1.pptx

Cardiomyopathy

1. Cardiomyopathies By : Rabia ehsan

2. Definition Cardiomyopathy is a progressive disease of the myocardium, or heart muscle. In most cases, the heart muscle weakens and is unable to pump blood to the rest of the body as well as it should.

3. WHO Classification Primary • Dilated
• Hypertrophic
• Restrictive
• Specific heart muscle disease (secondary) due to a known cause:
• Infective
• Metabolic
• Systemic disease
• Herediofamilial
• Sensitivity
• Toxic(alcohol)

4. Functional Classification Dilated (congestive, DCM, IDC) ⚫ ventricular enlargement and syst dysfunction
• Hypertrophic (IHSS, HCM, HOCM) ⚫ inappropriate myocardial hypertrophy in the absence of HTN or aortic stenosis
• Restrictive (infiltrative) ⚫ abnormal filling and diastolic function In addition to this We are going to cover • arrhythmogenic right ventricular dysplasia (ARVD)

5. Idiopathic Dilated Cardiomyopathy ⚫ a disease of unknown etiology that principally affects the myocardium
• LV dilatation and systolic dysfunction
• pathology ⚫ increased heart size and weight
⚫ ventricular dilatation, normal wall thickness
⚫ heart dysfunction out of portion to fibrosis Most common cause of clinical syndrome of Heart Failure

6. Pathophysiology of Dilated Cardiomyopathy

7. Causes ➤Cardiotoxic agents like alcohol or coccaine
➤Genetic
➤Hypertension
➤Ischemia (CAD)
➤Muscular dystrophy (Weakening and wasting of muscles)
➤Myocarditis
➤Valvular disease

8. Clinical Sign and symptoms Clinical signs • Systolic blood pressure normal or less than normal.
• Raised JVP
• Prominent a and v waves tricuspid regurgitation
• b/l basal crepts
• Pedal edema
• Hepatomegaly
• Gallop rhythm
• Systolic murmurs-Mitral, tricuspid reg. Symptoms of heart failure • Due to pulmonary congestion (left HF) dyspnea (rest, exertional, nocturnal), orthpnea
• Due to systemic congestion (right HF) edema, nausea, abdominal pain, nocturia
• Due to low cardiac output fatigue and weakness

9. Findings on ECG • Sinus tachycardia in presence of heart failure. • Atrial and ventricular tachyarrhythmia
• Poor r wave progression
• Anterior q waves Interventricular conduction defects –mostly LBBB
• Left atrial abnormality
• Hypertensive changes by voltage criteria not evident
• ST-T changes are seen

10. Findings on 2D Echo • Dilated chambers
• Left atrium is usually enlarged
• Left ventricle is enlarged. Normal 3.8-5.0cm
• Mitral and tricuspid regurgitation on doppler flow.
• LV or Global hypokinesia with an LVEF of <45%
• Stress testing –tachyarryhthmias
• Dobutamine stress echo helpful in assessing the clinical prognosis.

11. Treatment • ACE,
• B blockers in symptomatic,
• Diuretics- vol. Overload,
• Spironolactone –advanced,
• Digoxin is drug of choice, Treatment of arrhythmias. Biventricular pacing-plus ICDs-IVCDS,
Anticoagulation,
Amiodarone,

12. Hypertrophic Cardiomyopathy Hypertrophic cardiomyopathy (HCM) is a disease in which the heart muscle becomes thickened, also called hypertrophied. Walls of the left ventricle become thick and stiff. Over time, the heart can’t take in or pump out enough blood during each heartbeat to supply the body’s needs.

13. Clinical Features Symptoms.... SAD • Syncope on effort/pre-syncope
• Angina on effort
• Dyspnea on effort
• Sudden death (SCD)
⚫ (less frequent)...palpitations, PND, dizziness Signs • Jerky pulse
• Palpable LV heave
Double impulse at apex
mid-systolic murmur-sign of LV outflow obstruction
• Pansystolic murmur

14. Anatomical variants

15. Hypertrophic Cardiomyopathy Unexplained hypertrophy (no HTN, AS...) ■Any degree or pattern: diffuse, septal, apical
Abnormalities of diastolic function
Always present
■Not a consequence of hypertrophy
■Precedes hypertrophy!!
Correlates with symptoms
Early diagnosis in genetically at risk
■Increased risk of sudden death
Athletes
Marked septal hypertrophy
Family history of sudden death
LVOT obstruction with mild or no

16. Hypertrophic CMP Screening ■ Autosomal dominant
■ Phenotypic expression variable
■ 1/500chances
■ First degree relatives
Ages 12-18 yrs annual clinical, ecg, echo
> 18 yrs ASX every 5 years
<12 yrs evaluate if symptomatic or intense sports Affected family members need repeat screening every 12-18 months

17. Echocardiographic use in HCM Best screening test ■ Defines magnitude and location of hypertrophy
■Excludes other causes of hypertrophy (AS, VSD)
■LV wall thickness >13 Shows effect on other cardiac chambers
ASH-leads to earlier diagnosis---SAM-murmur and symptoms

18. Systolic Anterior Motion ■Abnormal independent anterior motion of the mitral leaflet
■ Requires
Narrow LVOT
Hyper contractile LV
■Severity of LVOT o is related to the duration and degree of SAM and to the reduction in LVOT size
■ Decreases stroke volume
■Raises ventricular pressures creating more hypertrophy

19. LVOT Obstruction Systolic Anterior Motion (SAM) most common cause of LVOT obstruction in HCM called Hocm
Only 25% of HCM at rest have LVOTO > 30mmHg
A gradient >50mmHg is considered significant
■ Dynamic Obstruction
Recent study found 70% of patients without LVOTo at rest developed LVOTo with exercise
Increases with increases in contractility and Decreases with increases in LV volume Independently predicts
More severe diastolic dysfunction and symptoms

20. Doppler Profiles in HCM ■LVOTo from SAM
Later-peaking than MR jet
Delayed onset Estimate LVOTO
Peak LVOTo = 4 (MR systolic jet velocity)2 +LA p – SB
■Mid-cavity obstruction
Steep, dagger shaped,
Latest peak Short duration
■Mitral Regurgitation
Posteriorly directed
Earlier onset
Longer duration
Greater magnitude than SAM jet
Earlier peak (first third of systole)

21. Pathophysiology of Hypertrophic Cardiomyopathy

22. Treatment Plainning •Medical management Beta adrenergic blockers: eg - atenolol
Calcium channel blocker: eg - verapamil Ant dysrhythmic drugs: eg - amiodarone

Surgical management
■ Septal myectomy : It is an open heart surgical procedure in which the surgeon removes the part of thickened, over grown septum between the ventricles.

■Septal ablation: In this procedure a small portion of the thickened heart muscle is destroyed by injecting alcohol through a long, thin tube into the artery supplying blood to

23. Restrictive Cardiomyopathy

24. Pathophysiology

25. Features Fatigue
Exercise intolerance
Dyspnea
Orthopnea(shortness of breath (dyspnea) which occurs when lying flat)
Syncope
Palpitations
Peripheral edema
Jugular venous distention

26. • Rare condition
• Ventricular filling is impaired because ventricles are stiff.
• Lead to high atrial pressure with atrial hypertrophy, dilatation and later atrial fibrillation.
• Amyloidosis is the most common cause.
• Other form:
• Idiopathic perimyocyte fibrosis
• Familial restrictive cardiomyopathy

27. Restrictive CMP Echo Findings ■ Hypertrophy
Enlarged atria
- Consequence of poor ventricular compliance
- Atrial fibrillation common
■Restricted ventricular filling
- Remember the myocardium is involved primarily
- Annular E’ is markedly reduced (always < 8)
Elevated filling pressures

28. Amyloid Most Common Normal to small LV cavity size
Thickend LV and often RV walls
■Speckling not specific
Infiltration into valve structures
■ Enlarged atria
Pericardial effusion in up to a third
High EF but low stroke volume
Elevated filling pressures
Restrictive filling

29. LV Non-Compaction Not yet in WHO as a form of CMP
■Abnormality of ventricular morphogenesis
Unique, rare cause of CHF, lethal
arryhthmias, and systemic embolization
■ Identifiable non-invasively

30. Echocardiographic diagnostic Criteria in LVNC Absence of other coexisting cardiac abnormalities
Thin, compacted, epicardial tissue layer and a thick spongy, endocardial, non compacted layer with extensive trabeculations and sinusoids
- End-systolic ratio of compacted to non-compacted layers of > 2 discrimates LVNC from HCM, HTN, etc
Deep intratrabecular recesses that communicate with LV cavity
Localization in the apex, mid lateral, or mid-inferior walls
Local or global LV dysfunction
RV can be involved but difficult to diagnose (normally trabeculated)

31. Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) M / F 1:3
Familial occurence 3
Pathologic fibrofatty infiltration of the RV free wall
Lethal arrhythmias in young patients
Clinically in this condition, patches of the right ventricular myocardium are replaced with fibrous and fatty tissue Inherited as autosomal dominant trait • Prevalence in India is 10 per 1 lakh population. • Dominant clinical problems are
⚫ ventricular arrhythmia
Sudden death
Right sided cardiac failure

32. Echocardiographic Findings ■ Regional RV dilatation (89%)
- PSLA view RVOT> 3.2cm
- PSAX RVOT > 3.6 cm
Morphologic RV abnormalities
- Trabecular derangement (54%)
- Moderator band hyperreflectivity (34%)
- Focal RV saculations or aneurysms (17%)
■ Abnormalities in Regional RV function (62%)
- 60% of patients with normal RV function had identifiable structural abnormalities Ecg shows inverted T waves in the right precordial leads.

• MRI useful diagnostic tool and used to screen 1 st degree

relatives from having the same pathology.