Aortic Stenosis “Senile” calcific aortic stenosis. Degeneration and calcification of bicuspid aortic valve. Chronic Rheumatic Heart Disease Endocardium Fibrosa Spongiosa Elastic fibers in black Aortic valve structure Ventricular side 1 Calcification 2 3 Aortic insufficiency due to aortic dissection • Hypertension • Connective tissue disorders Marfan’s: 1 in 5000. 70-85% familial (Autosomal dominant) Fibrillin-1 (15q21) ~ 500 distinct mutations 4 Medial degeneration without atherosclerosis Mitral Stenosis • Chronic Rheumatic Heart Disease • (Rare) Mitral annular calcification Myxoma 5 Acute Rheumatic Fever Immunologically mediated, following 1-6 weeks after pharyngitis with Group A Streptococcus in 3% of those infected. ?Antibodies ? Hypersensitivity reaction induced by streptococcal M proteins with cross-reaction with similar antigenic determinants in joints, heart, skin, CNS. Clinical Diagnosis: revised Jones criteria Acute Rheumatic Fever Pancarditis Pericarditis Myocarditis (Aschoff body) Endocarditis with sterile vegetations Death in acute rheumatic fever is very rare (<1%) and due to cardiac involvement 6 Aschoff body of acute rheumatic carditis: a cardiac granuloma Acute rheumatic fever: involvement of mitral chordae tendineae 7 Native chordae bound together by fibrous tissue in chronic rheumatic mitral valve disease Chronic Rheumatic Heart Disease Follows Acute Rheumatic Heart Disease by decades. MV involved in 65-70% MV & AV in 25% Mitral stenosis, aortic stenosis. Arrhythmias (A. fib. with left atrial enlargement). Heart failure. Predisposed to infective endocarditis. 8 9 Chronic Rheumatic Heart Disease: “Fishmouth” deformity of the mitral valve Mitral Valve Prolapse • 2 populations: women in 20s; men in 40-50s; the gross and microscopic appearances of the valves are the same. • Most are asymptomatic • Mid-systolic click • Rarely: chest pain, dyspnea, fatigue, or psychiatric manifestations • ~3% have complications: Infective endocarditis Mitral insufficiency Thrombus formation with embolization Arrhythmia/sudden death (unknown mechanism) 10 Normal (aortic) valve structure Myxomatous, prolapsing mitral valve 11 “Myxomatous” mitral valve 12 Acute endocarditis of mitral valve 13 Rupture of MV papillary muscle with inferior wall MI The following 6 slides on valve prostheses were not presented in the lecture on 1-4-06 14 Heart Valve Prostheses: Complications • Mechanical: component failure • Bioprostheses: degeneration with calcification • Both: Paravalvular leak, endocarditis. Thrombosis and tissue overgrowth are less common in bioprostheses than in mechanical valves. Bioprosthetic (Hancock)valve: Degeneration and tissue overgrowth 15 Thrombus occluding AV prosthesis 16 Thrombus covering mitral valve prosthesis (viewed from opened left atrium Endocarditis (arrows on vegetations) of ball valve prosthesis (Starr-Edwards) 17.