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Peak incidence: ages of 5-15 years. The incidence of rheumatic fever has declined over the past 30 years It affects large joints causing Arthritis. It affects the heart during its acute phase acute rheumatic carditis after many years may cause chronic valvular deformities
Endocardium
Common and may affect any valve, mostly mitral and aortic valves. Valves are edematous and thickened with foci of fibrinoid necrosis. (Aschoff nodules uncommon). Formation of small vegetations fibrinous clots along the lines of valve closure (Verrucous Endocarditis).
Pericardial involvement
Fibrinous pericarditis, sometime serosanguinous pericardial effusion. associated with serous or
Small vegetations (verrucae) are visible along the line of closure of the mitral valve leaflet (arrowheads). Previous episodes of rheumatic valvulitis have caused fibrous thickening and fusion of the tendinous cords.
Pathological changes:
Chronic scarring and calcification of the valve leaflets, which invert the valve into stiff and thickened structure which may lead to: Valve orifice becomes stenotic Improper closure (regurgitation). Shortening and fusion of the chordae tendineae.
Mitral stenosis with diffuse fibrous thickening and distortion of the valve leaflets, commissural fusion (arrow)
Surgically removed specimen of rheumatic aortic stenosis demonstrating thickening and distortion of the cusps with commissural fusion (rigid triangular channel)
The aortic valve leaflets are rigid and deformed by calcified masses, so fibrosis and calcification of the valve cusps lead to valve sclerosis.
The calcium deposits lie behind the valve cusps (at the bases of the cusps). The free edges of the cusps are usually not affected. Calcific stenosis does not fuse the cusps.
Symptom: severe cases may cause angina, syncope (fainting), congestive heart failure, L.V. hypertrophy, sudden death due to arrhythmia.
Degenerative calcific aortic stenosis of a normal valve having three cusps. Nodular masses of calcium are heaped up within the sinuses of Valsalva (arrow). Note that the commissures are not fused, as in post-rheumatic aortic valve stenosis
Long axis view of the left ventricle demonstrating ballooning with prolapse of the posterior mitral leaflet into the left atrium (arrow). The left ventricle is on the right.
Opened valve showing pronounced hooding of the posterior mitral leaflet with thrombotic plaques at sites of leafletleft atrium contact (arrows).