Rheumatic Heart Disease Types of Heart Diseases IHD Hypertensive Heart Disease Valvular Heart Disease y Degeneration y Rheumatic

Heart Disease y Infective Endocarditis y Non-Infective Vegetations Carcinoid Heart Disease Myocardial Disease Pericardial Disease Neoplastic Heart Disease Congenital Heart Disease Rheumatic Heart Disease Heart disease developing in Rheumatic Fever y Collagen-vascular disease y Connective tissue disease Acute condition characterized by y Fever (1-5 weeks after an episode of group A ( haemolytic) streptococcal pharyngitis) y M types 1, 3, 5, 6, 14, 18, 19, 27, 29 y Multi system involvement y Immunologically mediated 5-15 y/o Only 3% develop rheumatic fever (genetic predisposition) Antibiotic therapy (given within 9d of infection prevents rheumatic fever) Risk factors y Crowding y Poor hygiene Under developed countries ( prevalent) Pathophysiology Jones Criteria Rheumatic Fever Major y Carditis y Polyarthritis y Sydenhams Chorea y Erythema marginatum, subcutaneous nodules

Minor Clinical Previous rheumatic fever, RHD, arthralgia, fever Laboratory C reactive protein, leukocytosis, prolonged PR interval

Supporting Evidence ASO, +ve throat culture for group A streptococci Jones Criteria 2 Major OR 1 Major & 2 Minor criteria Pathological Lesion Rheumatic Fever

Morphology Aschoff bodies Foci of fibrinoid necrosis Surrounded by T lymphocytes (primarily) Ocassionally plasma cells Anitschkow cells (pathognomonic) Macrophages Abundant amphophilic cytoplasm Round to ovoid nucleus Central slender wavy chromatin (caterpillar cells) Aschoff giant cells

Site of Involvement Many sites (heart, skin, CNS, synovium) More distinct in heart (Pancarditis) y Pericarditis y Myocarditis y Endocarditis Pericarditis

Fibrinous/ serofibrinous exudate

Bread-and-butter appearance

Clinical Features Carditis y Murmur due to dysfunction of MV, AV y Chest pain, pericardial rub (due to pericarditis, effusion) y Heart block, atrial fibrillation, prolonged PR interval Polyarthritis y Usually large joints y 2 (synchronous, metachronous) y Features of acute inflammation y No permanent disability Sydenham chorea/ St. Vitus dance y Definite sign of rheumatic fever y Sudden, aimless, irregular, involuntary movement y Girls > Boys y Several months after streptococcal infection y Runs weeks to months y No permanent neural sequelae Erythema marginatum (< 10%) y Non pruritic, pain, erythematous macules y Typically occurs in trunk (never on face, hands), rounded borders y Transitory, changes its appearance within minutes hours Subcutaneous nodules y Extensor surface of joints y Over bony prominence (elbow) st y Appear in 1 week (disappear within 1 month) y Asymptomatic

Myocarditis

Aschoff bodies (scattered within interstitium perivascularly) Endocarditis Vegetations (Verrucae) Small (1-2 mm) Located on y Cusps y Leaflets along line of closure y Tendinous cords Due to deposition of fibrin (at sites of erosion related to underlying inflammation, fibrinoid degeneration)

MacCallum plaques Whitish patch Left atrium Endocardial thickening Due to mitral valve regurgitation

Vegetations Vegetations

Chronic Rheumatic Heart Disease Characterized by y Organization of acute inflammation Neovascularisation of valve leaflets y Subsequent fibrosis & deformity of valves Pure mitral (65-70%) Mitral + Aortic (25%) Tricuspid + Pulmonary (rare, less severe) Replacement of Aschoff bodies (by fibrous tissue) y Morphology Fish mouth/ button hole mitral valve y Thickening of valve leaflets y Commissural fusion y Shortening, thickening, fusion of tendinous cords y Fibrous bridging Progressive dilatation of left atrium Atrial thrombus CVC of lung (chronic venous congestion of lung) Right ventricular hypertrophy (Left ventricle is normal in isolated mitral stenosis)

Other Valvular Lesions

Mitral incompetence Aortic stenosis Aortic incompetence Tricuspid stenosis +/- incompetence Pulmonary stenosis +/- incompetence

Clinical Features Manifest years/ decades after initial episode of rheumatic fever Depends on which heart valve(s) involved y Cardiac murmur y Cardiac hypertrophy, dilatation y Cardiac failure Arrhythmia (atrial fibrillation) Thromboembolic manifestation Superadded infective endocarditis Vegetations Infective Endocarditis Non Bacterial Thrombotic Endocarditis (Marantic endocarditis) Libman-Sacks Endocarditis

Bulky Friable Destructive to underlying structures Contains Fibrin, Inflammatory cells, Bacteria Single/ multiple valves MV; AV > TV; PV

Small (1-5 mm) Fibrin + Platelets + Other blood components Single/ multiple Both sides of the heart Sterile (No destruction) Loosely attached (embolism to brain, spleen, kidney) Debilitated patients (cancer; sepsis)

SLE (typically occur in) Small (1-4 mm) Sterile Single/ multiple MV & TV Granular pink Frequent on under surface of valves Also on y Chordae y Mural endocardium y Atrium y Ventricle Haemotoxyphilic bodies (under microscopy) Leads to scarring, deformity