Alfrin Antony

Lecturer
Rheumatic Heart Disease Department of pathology
+919738286092

Definition:- Rheumatic heart disease is a chronic condition

characterized by scaring and fibrosis of valves and layers of the
heart secondary to rheumatic fever

Pathogenesis:-
Autoimmune mechanism has been proposed

Attack of group ‘A’ beta hemolytic streptococci

↓
Pharyngitis
↓
Antibodies directed against the M proteins of certain strains of streptococci cross-react
with tissue glycoprotein in the heart, joints and other tissues.
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(Heart)
Edema of connective tissues and increased acid mucopolysaccharide in the ground
substance.
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Accumulation of ground substance.
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Separation of collagen fibers.
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Collagen fibers become fragmented and disintegrated.
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Proliferation of cells (lymphocytes, plasma cells, a few neutrophils, cardiac histocytes
(anitschkow cells) at the margin of the lesion
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Aschoff nodules (12 to 16 weeks)

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Anitschkow cells nodule becomes spindle shaped with diminished cytoplasm.
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AFTER YEARS
Aschoff body becomes less cellular and collagenous tissue is increased
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Fibro collagenous scar
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Formation of chronic sequelae (endocardium , myocardium, pericardium)
Rheumatic pancardiditis
1. Rheumatic endocarditis
(a).Rheumatic valvulitis
(b).Rheumatic mural endocarditis
2. Rheumatic myocarditis
3. Rheumatic pericarditis

1(a).Rheumatic Valvulitis
Grossly Microscopically

Acute
1.Thickening and loss of translucency
of the valve leaflets
2.Gray brown, watery vegetations
Chronic
1.Permanent deformity of on one or
more valves (mitral or aortic)
2.”Fish mouth” or “button hole”
3.Thickening, shortening and fusion of
chordae tendinae
Acute
1.Edema
2.Cellular infiltration
3.Vegetations of fibrin
Chronic
1.Thicken by fibrous tissue with
hyalinization (Calcification rarely)
2.Thickened blood vessels with
narrowed lumina

1(b).Rheumatic mural endocarditis

Grossly Microscopically
MacCallum’s patch:- MacCallum’s patch:-
Lesions of endocardial surface in the a. Edema
posterior wall of the left atrium just above b. Fibrinoid changes in collagen
posterior leaflet of the mitral valve c. Cellular infiltrate of lymphocytes
d. Plasma cells
e. Macrophages
f. Anitschkow cells*

2. Rheumatic Myocarditis
Grossly Microscopically
Acute Acute
Left ventricular myocardium soft and Aschoff nodules are scattered (inter
flabby venticular septum, left ventricle and left
atrium)
Intermediate stage Intermediate stage
Interstitial tissue of the myocardium shows In Aschoff:- Granuloma with central
small foci of necrosis fibrinoid necrosis and surrounded by
palisade of anitschkow cells
Late stage Late stage
Foci of aschoff bodies are visible Aschoffs bodies are replaced by small
fibrous scars
3. Rheumatic Pericarditis
Grossly Microscopically
chronic chronic
a). Deposition of fibrous exudates (Loss of a). Fibrosis aschoff bodies on the surfaces
normal shiny pericardial surface)
b). Accumulation of fibrous exudates in the
pericardial sac b). Infiltrated sub serosal connective tissue
(“Bread and butter” appearance)
c). Chronic adhesive pericarditis

c). Adhesions between visceral and parietal

surfaces

Extracardiac Lesions
 Polyarthritis
 Subcutaneous nodules
 Erythema marginatum
 Rheumatic arteritis
 Chorea minor
 Rheumatic pneumonitis pleuritis

ANTOBODIES AGAINST

Beta-Haemolytic streptococci group A

 Anti-streptolysin O (ASO)
 Anti-streptokinase
 Anti-streptohyaluronidase
 Anti DNA ase B

Diagnosing criteria
Diagnosed by Jones Criteria:
Either two of the major manifestations or one major and two minor manifestations.

JONES CRITERIA

MAJOR CRITERIA MINOR CRITEIA

1.Carditis 1.Fever
2.Poly arthritis 2.Arthralgia
3.Chorea:- a neurologic disorder with 3.Previous History of RF
involuntary purposeless rapid movements. 4.Increased
4.Erythema Marginatum a. E.S.R
5.Subcutaneous Nodules b. C-Reactive Protein
c. Leucocytosis
5.Prolonged PR interval

Complications

• Severe mitral stenosis progresses to left atrial

hypertrophy and dilatation,
• Mural thrombosis,
• Pulmonary congestion,
• Pulmonary vascular sclerosis and then right
ventricular hypertrophy.
• The left ventricle is normal is isolated pure
mitral stenosis.

Other complications of chronic RHD include

• heart failure,
• Arrhythmias particularly AF in case of M.S,
• Thrombo embolic complications and infective
endocarditis.
• The long term prognosis is highly variable
• Rx- Surgical replacement of diseased valves
with prosthetic device

*anitschkow cell: are the cardiac histocytes present in small numbers in the normal heart.
The nuclei are vesicular and contain prominent central chromatin mass which in
longitudinal section appears “serrated” or “caterpillar” like and in cross section it look
like an “owl’s eye”
**Aschoff bodies: are spheroidal or fusiform distinct microscopic structures occurring in
the intestitium of the heart in RHD.(it contains almost four anitschkow cells )