Rheumatic Fever & Rheumatic Heart Disease.ppt

Comprehensive presentation to teach undergradute students about salient features of acute rheumatic fever and rheumatic heart disease in children.

1. Dr Amit Agrawal
MD,
Associate Professor,
Department of Pediatrics
RHEUMATIC FEVER

2. Etiology
Acute rheumatic fever is a systemic disease of childhood, often
recurrent that follows group A beta hemolytic streptococcal infection
It is a delayed non-suppurative sequelae to URTI with GABH
streptococci.
It is a diffuse inflammatory disease of connective tissue, primarily
involving heart, blood vessels, joints, subcutaneous tissue and CNS

4. Epidemiology
Ages 5-15 yrs are most susceptible
Rare <3 yrs
Girls >boys
Common in 3rd world countries
Environmental factors -- over crowding, poor
sanitation, poverty
Incidence more during fall ,winter & early spring

5. Pathogenesis
Basis is an autoimmune reaction to GABHS caused by molecular mimicry.
Epitopes of GABHS, especially repeat regions on M protein, resemble
human myosin, keratin and other molecules.
Two methods for autoimmune damage:
T cell, sensitized to human molecules following initial infection, are recalled after
subsequent GABHS infections.
Cardiac valve damage is caused by antibodies to GABHS N-acetylglucosamine that
cross react with laminin found on cardiac endothelium.
After a latent period of 1-3 weeks, antibody induced immunological
damage occur to heart valves, joints, subcutaneous tissue & basal ganglia.

6. Pathologic Lesions
Fibrinoid degeneration of connective tissue, inflammatory edema,
inflammatory cell infiltration & proliferation of specific cells resulting in
formation of Ashcoff nodules, resulting in-
-Pancarditis in the heart
-Arthritis in the joints
-Ashcoff nodules in the subcutaneous tissue
-Basal ganglia lesions resulting in chorea

7. Clinical Features
Flitting & fleeting migratory polyarthritis, involving major joints
Commonly involved joints-knee, ankle, elbow & wrist
Occur in 80%, involved joints are exquisitely tender
In children <5 yrs, arthritis is usually mild but carditis is more prominent
Responds dramatically to ASA (48-72 hrs)
Subsides 1-2 weeks with No sequelae
Arthritis do not progress to chronic disease
Low incidence of carditis and chorea
Arthritis

9. Manifest as pancarditis (endocarditis, myocarditis and pericarditis)
Occur in 40-50% of cases
Most serious manifestation and leaves sequelae & permanent damage
Latent period of 1-3 mo
Valvulitis occur in acute phase
Chronic phase- fibrosis, calcification & stenosis of heart valves (fish
mouth valves)
Carditis

10. Severe carditis may last for 2-6 mos
Mild:
Palpitation, shortness of breath, chest pain, murmur
Moderate to Severe:
Difficulty of breathing, edema of the legs, presence of
murmur or friction rub on auscultation, heart enlargement
on examination or in chest X-ray

11. Occur in 5-10% of cases
More common in prepubertal girls (8-12 yrs)
Latent period 1-6 mos. (May appear even 6-12 months after the attack)
Clinically manifest as - clumsiness, deterioration of handwriting,
emotional lability or grimacing of face
Clinical signs- pronator sign, jack in the box sign, milking sign of hands
Sydenham Chorea

12. Affects the basal ganglia and caudate nuclei
No neurological sequelae
Normal ASO titer & Acute phase reactants
TRIAD:
1.Involuntary movements
2.Muscular weakness
3.Emotional disturbance

15. Occur in <5% of the cases.
Unique, transient, serpiginous lesions of 1-2 inches in size
Pale center with red irregular margin
More on trunks & limbs & non-itchy
Worsens with application of heat
Often associated with chronic carditis
Erythema Marginatum

16. Erythema Marginatum

17. Hard, painless non-pruritic, pea-sized, 0.2-2 cm in diameter,
palpable, freely movable swelling
Occur in 2-10%
Delayed appearance & last for weeks
Mainly over extensor surfaces of joints, spine, scapulae & scalp
Associated with strong seropositivity
Always associated with severe carditis and higher mortality rate
Subcutaneous nodules

20. Other features (Minor features)
Fever-(upto 101 degree F)
Arthralgia
Pallor
Anorexia
Loss of weight

21. Laboratory Findings
High ESR, Elevated C-reactive protien
Anemia, leucocytosis
ASO titre >200 Todd units. (Peak value attained at 3 weeks, then
comes down to normal by 6 weeks)
 Positive Anti-DNAse B test
 Throat culture - GABH streptococci

22. ECG- prolonged PR interval, 2nd or 3rd degree blocks, ST
depression, T wave inversion
2D Echo cardiography- valve edema, mitral regurgitation, LA &
LV dilatation, pericardial effusion, decreased contractility

23. Diagnosis
Rheumatic fever is mainly a clinical diagnosis
No single diagnostic sign or specific laboratory test available for diagnosis
Diagnosis based on modified JONES criteria (Revised in 2015).
Separate criteria for Low-Risk populations (incidence ≤2 per
100,000 school-age children per year or all-age RHD prevalence of
≤1 per thousand population) and Moderate/High-Risk populations
(higher incidence or prevalence rates).
Carditis– subclinical evidence (i.e., in the absence of a murmur,
echocardiographic evidence of MR meeting specific criteria to
distinguish physiologic from pathologic MR)

24. Modified Jones Criteria for the Diagnosis of RF *
Major
Manifestation
Minor Manifestation Supporting Evidence
of Streptococcal
Infection
Clinical Laboratory
CARDITIS Arthralgia
POLYARTHRITIS Fever Elevated ESR Positive Throat culture
or Rapid Strept. Ag Test
CHOREA
ERYTHEMA
MARGINATUM
Positive CRP
Elevated or rising Strep
Ab titer of at least two
fold rise from baseline
SUBCUTANEOUS
NODULE
Prolonged PR
interval
* The presence of two major criteria, or one major and two minor
criteria, indicates a high probability of acute rheumatic fever, if supported
by evidence of Group A streptococcal infection

25. Echocardiographic Findings in
RheumaticValvulitis
Pathologic Mitral
Regurgitation (all 4 met)
Pathologic Aortic
Regurgitation (all 4 met)
1. Seen in at least 2 views1. Seen in at least 2 views
2. Jet length ≥2 cm in at least
1 View
2. Jet length ≥1 cm in at least 1
view
3. Peak velocity >3 meters/
second
3. Peak velocity >3 meters/
second
4. Pan-systolic jet in at least 1
envelope
4. Pan-systolic jet in at least 1
envelope

26. Exceptions to Jones Criteria
Chorea alone, if other causes have been excluded
Insidious or late-onset carditis with no other explanation
Patients with documented RHD or prior rheumatic fever, one
major criterion, with fever, arthralgia or high CRP suggests
recurrence

27. Differential Diagnosis
Juvenile rheumatiod arthritis
Septic arthritis
Sickle-cell arthropathy
Kawasaki disease
Myocarditis
Scarlet fever
Leukemia

28. Treatment
Step I - primary prevention (eradication of streptococci)
Step II - anti inflammatory treatment (aspirin, steroids)
Step III- supportive management & management of complications
Step IV- secondary prevention (prevention of recurrent attacks)

29. Treatment: Primary Prophylaxis
Agent Dose ModeDuration
Benzathaine Pen G
 
Pen V
600,000 U in < 27 kg (60lb)
1.2 M U > 27 kg
Children: 250 mg 3 X/day
Adolescents & Adults: 500 mg
3 X/day
IM
PO
Once
10 Days
Erythromycin
Estolate
Ethylsuccinate
 
Azithromycin
20-40 mg/kg/d
2-4 X daily (max 1 g/day)
40 mg/kg/day
2-4 X daily (max 1 g/day)
500 mg on the 1
st
day
250 mg for the next day
PO
PO
PO
10 Days
10 Days
5 Days

30. Bed rest
Treatment of congestive cardiac failure: digitalis, diuretics
Treatment of chorea - diazepam or haloperidol
Rest to joints & supportive splinting
Supportive management &
management of complications

31. Suggested Schedule of Anti Inflammatory Therapy
Clinical Severity Treatment
Arthralgia or mild arthritis;
no carditis
Analgesics only
Moderate or severe arthritis;
no carditis or carditis without
CCF
Aspirin 90 – 100 mg/kg/day for 2
weeks; longer if necessary at 60-70
mg/kg/day
Carditis with cardiac failure;
with or without joint
manifestation
Prednisone, 40-60 mg/day; after 2-3
weeks slow withdrawal to be
completed in 9 more weeks
Aspirin to overlap for 4-6 weeks after
discontinuation of Prednisone

32. Treatment: Secondary Prophylaxis
Agent Dose Mode
Benzathine Pen G
Pen V
Sulfadiazine
For Pen & Sulf
Allergy:
Eryhtromycin
1.2 MU >27 kg q 4 wks (q
3wks for pts with carditis)
0.6 MU <27 kg
250 mg BID
Or
0.5 g OD for < 27 kg
1.0 g for > 27 kg
250 mg BID
IM
PO
PO
PO

33. Duration of Secondary Prophylaxis
Category Duration
RF with carditis & residual heart
disease (persistent valvular disease)
RF with carditis but no valvular
disease
RF without carditis
10 yr since last episode & at least
until 40 yr, sometimes lifelong
10 yr or until adulthood
(whichever is longer)
5 yr or until age 21 yr
(whichever is longer)

34. Prognosis
Rheumatic fever can recur whenever the individual
experience new GABH streptococcal infection, if not on
prophylactic medicines
Good prognosis for older age group & if no carditis during
the initial attack
Bad prognosis for younger children & those with carditis
with valvar lesions

35. Valvular Heart DiseasesValvular Heart Diseases

36. Valvular Heart Disease
Is a disease caused by stenosis, or abnormal narrowing of the
cardiac valves, causing obstructed blood flow and blood
regurgitation.
Congenital or acquired.
Stenosis - failure of a valve to open completely, impedes
forward flow.
usually a chronic abnormality of the valve cusp that
becomes clinically evident after many years

37. Insufficiency - failure of a valve to close completely, allowing
reversed flow.
Valvular insufficiency may appear acutely
Single or multiple valve involvement
Functional regurgitation- incompetence of a valve from
abnormality in one of its support structures. E.g. dilation of the
right or left ventricle can pull the ventricular papillary muscles
down and outward, thereby preventing proper closure of
otherwise normal mitral or tricuspid leaflets.
Valvular Heart Disease

38. Spectrum of VHD
RegurgAcute
Aortic Valve Chronic
Stenosis
Chronic
RegurgAcute
Mitral Valve Chronic
Stenosis
Chronic

39. Cardiac Physiology

40. Cardiac Physiology
Regurgitation/ Insufficiency – leaking (backflow) of
blood across a closed valve
Stenosis – Obstruction of (forward) flow across an
opened valve
Systole AV/PV – opens-------Aortic Stenosis
S1-S2 MV/TV – closes------Mitral Regurg
DiastoleAV/PV – closes------Aortic Regurg
S2-S1 MV/TV – opens-------Mitral Stenosis

41. Clinical consequences
Clinical consequences vary depending on
valve involved
degree of impairment
how fast it develops
rate and quality of compensatory mechanisms
E.g. sudden destruction of aortic valve cusp by infection
(infective endocarditis) can cause acute, massive regurgitation
that can be rapidly fatal.
In contrast, rheumatic MS usually develops over years, and its
clinical effects are often remarkably well tolerated.

42. Clinical consequences
Valvular stenosis or insufficiency produces secondary
changes, both proximal and distal to the affected valve.
Valvular stenosis - pressure overload
Valvular insufficiency - volume overload
Injury to endocardium by high speed "jets" of blood ejected
through narrowed stenotic valves - IE

43. Rheumatic Heart Disease
RF - acute, immunologically mediated, inflam disease
Acute rheumatic carditis is common during active phase of RF and
may progress over time to chronic RHD.
RHD is characterized by deforming fibrotic valves eg MS
In chronic disease, MV is virtually always involved.
MV is affected alone in 65% to 70% cases, and along with the
AV in another 25% of cases.
TV involvement is infrequent, and PV is rarely affected.

44. Mitral Stenosis
MS – characterized by narrowing of the orifice of MV.
Almost caused by rheumatic heart disease.
Progressive obstruction of the mitral ostium causes increased
pressure in left atrium and pulmonary circulation.
Congestion may cause thromboembolism, and atrial hypertension
may cause atrial fibrillation.

45. Pathophysiology of MS
Obstruction to LA emptying
Increased
LA pressure
Increased
LA size
Atrial fibrillation
Increased pulmonary
artery pressure
Decreased LV filling
RV overload
Increased pulmonary
venous pressure
Pulmonary
edema

46. Symptoms of MS
CCF - such as dyspnea on exertion, orthopnea and
paroxysmal nocturnal dyspnea
Palpitations
Chest pain
Hemoptysis
Thromboembolism
Ascites and edema

47. Physical Examination
Prominent "a" wave in jugular venous pulsations: D/t
pulmonary hypertension and RVH.
Signs of right-sided heart failure: in advanced disease
Mitral facies: pinkish-purple patches on the cheeks ( d/t
vasoconstriction b/o diminished CO in severe MS.
Opening snap: heard at the apex when leaflets are still mobile.
(Due to abrupt halt in leaflet motion in early diastole, after initial
rapid opening)
A shorter S2 to opening snap interval - severe disease.

48. Loud S
1

Diastolic murmur: Low-pitched diastolic rumble
Most prominent at the apex,
Heard best with patient lying on left side in held expiration
Intensity does not correlate with severity of stenosis.
May be associated with:
MR or AS
Right Sided Murmurs
oPR – Graham Steel Murmur
oTR
Physical Examination

49. Evaluation of MS
ECG: Atrial fibrillation and LA enlargement
CXR: LAA and LA enlargement
Increased upper lobe vascularity (pulmonary congestion)
Kerley B and A lines, Dilated PA
Occasionally calcified MV
ECHO: The GOLD STANDARD for diagnosis.
Asses MV mobility, pressure gradient and MV area
Cardiac Catheterization – helpful, confirmatory, needed to
look at the coronaries

50. Management of MS
Mortality: Due to progressive pulmonary congestion, infection,
and thromboembolism.
Serial echocardiography:
Mild: 3-5 years
Moderate:1-2 years
Severe: yearly
Identify patient early who might benefit from percutaneous mitral
balloon valvotomy.

51. Medical Management: MS is a mechanical problem and
medical therapy does not prevent progression and it treats the
symptoms not the cause
-blockers, CCBs, Digoxin – to control heart rate and hence
prolong diastole for improved diastolic filling
Diuretics - for fluid overload
Anticoagulation – for AFib and LA clots
IE prophylaxis - Patients with prosthetic valves or a History
of IE for dental procedures
Management of MS

52. Surgical Therapy – it treats the cause
Percutaneous Ballon Valvulaoplasty – Non-calcified, pliable
valve
Open Commisurotomy – valve repair
Mitral Valve Replacement
Management of MS

53. Etiologies
Alterations of the Leaflets, Commissures, Annulus
Rheumatic
MVP
Endocarditis
Alterations of LV or LA size and Function
Papillary Muscle (Ischemic, MI, Myocarditis, DCM)
HOCM
LV Enlargement – Cardiomyopathies
LA Enlargement from MR
Mitral regurgitation

54. Pathophysiology of MR
Backward flow of blood from LV to LA (Systolic)
Increased
LA volume and
pressure
Increased LV filling
(Increased LVEDV)
Increased SV
Blood ejected into aorta
Left atrial enlargement
Increased
pulmonary
venous pressures
Pulmonary
edema

55. Mitral Regurgitation
Symptoms
Fatigue and weakness
Dyspnea and orthopnea
Right sided HF
Physical Exam
Holosystolic apical blowing murmur, Radiation to axilla
Intensity of murmur does correlate with the severity
Laterally displaced apical impulse, Soft S1
Split S2 (but is obscured by the murmur)
S3 Gallop (increased volume during diastole)

56. Workup in MR
ECG:
LA enlargement,
Atrial fibrillation
LV hypertrophy in severe MR
CXR:
LA enlargement,
Central pulmonary artery enlargement
ECHO:
Estimation of LA, LV size and function.
Valve structure assessment
TEE if transthoracic echo is inconclusive

57. Natural History of MR
Compensatory phase: 10-15 years
Patients with asymptomatic severe MR have a 5%/year mortality
rate
Once the patient’s EF becomes <60% and/or becomes
symptomatic, mortality rises sharply
Mortality: From progressive dyspnea and heart failure

58. Management of MR
Serial Echocardiography:
Mild: 2-3 years
Moderate: 1-2 years
Severe: 6-12 months
Medications
Vasodilator such as hydralazine
Diuretics for fluid overload
Rate control for AF with -blockers, CCB, digoxin
Anticoagulation in atrial fibrillation and flutter
IE prophylaxis: Patients with prosthetic valves or a history
of IE for dental procedures.

59. Surgical Management
Indications for Surgery
Asymptomatic patient with
LV dysfunction
Atrial fibrillation
Pulmonary hypertension
Symptomatic patient with LV dysfunction
Types of Surgery
MV Repair
MV Repalcement

60. Normal Aortic Valve Area: 3-4 cm
2
Symptoms: Occur when valve area is 1/4
th
of normal area.
Types:
Supravalvular
Subvalvular
Valvular
Most common surgical valve disease in developed world
Aortic Stenosis

61. Aortic Stenosis
• Rheumatic
- most common cause in the developing world
- almost always associated with MV disease
• Degenerative/calcification
- most common cause in industrialized world
- under 70 years of age ~ 70 % bicuspid and ~ 15 % tricuspid
- over 70 years of age, >50 % tricuspid and ~ 25 % bicuspid
• Other
- associated with other congenital cardiac abnormalities
(Co-arctation, VSD, Hypoplastic left heart, etc.,,)

62. Pathophysiology
Aortic Stenosis
Obstruction to LV Ejection
Chronic LV Pressure Overload
LV Hypertrophy
Pressure Gradient Created Across the Valve
Decreased LV Function

63. Symptoms
Chest pain/Angina (d/t increased myocardial oxygen
demand; demand/supply mismatch)
Syncope (exertional)
Dyspnea on exertion - due to heart failure (systolic and
diastolic)
Congestive Heart Failure (CHF)
Sudden death

64. Physical Examination
Harsh Ejection Systolic Murmur
Cresendo-decrescendo character
Late peaking - with increasing severity of stenosis
Loudness has no relation with severity
S4 gallop (from LVH)
Paradoxical split of 2
nd
HS in severe AS
Systolic thrill and typical heaving apical impulse
Pulsus Parvus et Tardus - Slow rising carotid pulse (pulsus tardus)
with decreased pulse amplitude (pulsus parvus)

65. ECG
•LVH with strain
CXR
•Dilated ascending aorta (post-stenotic dilatation)
•Valve calcification
Diagnosis

66. • Echo (primary diagnostic modality)
- AV anatomy (tricuspid, bicuspid, calcification)
- Mild Vs. Moderate Vs. Severe AS
- AVA and gradients can be calculated
- Progression of disease can be monitored
- Assessment of LV function and coexisting lesions
• Catheterization
- To assess coronaries prior to valve surgery
- Helpful to assess severity in complex situations
Diagnosis

67. Management
• Asymptomatic
- no specific therapy
- endocarditis prophylaxis
- if appropriate, rheumatic fever prophylaxis
• Mild and Mod AS ( AVA > 1.5 and 1.0 -1.4 sq cm)
- Normal physical activity
- No specific therapy, restoration of NSR in case of AF
- approx. progression is a decrease by 0.1 sq cm per year
- annual echo follow-up

68. General-
•IE prophylaxis
Medical M/M-
•limited role since AS is a mechanical problem.
•Vasodilators are relatively contraindicated in severe AS
Aortic Balloon Valvotomy -
•only a palliative treatment with little benefit,
Surgical Replacement -
•AV replacement
•Definitive treatment
Management

69. Aortic Regurgitation
Etiologies
Abnormalities of the Leaflets
Rheumatic, Bicuspid, Degenerative
Endocarditis
Dilation of the Aortic Annulus
Aortic Aneurysm / Dissection
Inflammatory (Syphyllis, Giant Cell Arteritis, Coll Vasc Dis-
Ankylosis Spondylitis, Reiters)
Inheritable (Marfans, Osteogensis Imperfecta)

70. Pathophysiology
Backward flow of blood from aorta into LV (Diastolic)
Increased
LV volume
and pressure
Increased SV
(Frank-Starling Mechanism)
Peak systolic pressure
increased because of
increased SV ejected into aorta
Increased diastolic
wall-tension produces
eccentric hypertrophy
Rapid fall of aortic
pressure during diastole
Increased pulse pressure
Increased
LA pressure
Increased
pulmonary
venous pressure
Pulmonary
edema

71. Asymptomatic until 4
th
or 5
th
decade
Rate of Progression: 4-6% per year
Progressive Symptoms include:
- Exertional dyspnea, orthopnea, and PND
-Nocturnal angina: due to slowing of heart rate and reduction of
diastolic blood pressure
-Palpitations: due to increased force of contraction
Symptoms

72. Physical Exam
Bounding Pulses
Wide pulse pressure - most sensitive indicator
Hyperdynamic and displaced LV apical impulse
Auscultation-
Diastolic Decrescendo blowing murmur at left sternal border
Austin flint murmur (apex) - Regurgitant jet impinges on anterior
MVL causing it to vibrate
Systolic ejection murmur - due to increased flow across AV
S4, S3 Gallop – in advanced AR

73. Peripheral Signs in AR
•Watson's water hammer pulse - bounding peripheral pulses
•Corrigan's pulse - rapid upstroke & collapse of carotid pulse
•de Musset's sign - head nodding in time with heart beat
•Mueller Sign – visible pulsations of uvula
•Quincke's sign - pulsations of capillary bed in the nail
•Traube's sign – Loud systolic 'pistol shots’ over femoral
artery
•Duroziez's sign - double sound heard over femoral artery
when compressed distally
•Hill’s sign – > 40 mm-Hg pressure difference between
popliteal & brachial arteries
•Mayne sign - > 15 mm-Hg drop in SBP on arm elevation

74. Diagnosis
CXR
enlarged cardiac silhouette
 aortic root enlargement
ECHO
Evaluation of the AV and aortic root
Measurements of LV dimensions and function
Aortography
Used to confirm the severity of disease

75. Treatment - Asymptomatic AR
Medical Therapy -
SBE Prophylaxis
Vasodialators (Nifedipine, ACE-I) - Nifedipine improve stroke volume
and reduce regurgitation only if pt symptomatic or HTN.
Diuretics
Follow up – To evaluate EF, Severity of AR
Mild/moderate cases - echo & cardiac stress test every 1–2 years
Severe moderate/severe cases - echo with cardiac stress test and/or
isotope perfusion imaging every 3–6 months.

76. Treatment - Symptomatic AR
Aortic Valve Replacement
Pulmonary Autograft (Ross)
Prosthetic Mechanical Valve
Xenograft valve (Bovine or Porcine)
Stented Pericardial Tissue valve
Homograft