4- marboe valvular heart disease - columbia university€¦ · mv 65-70% mv & av 25% mitral...
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Aortic stenosis
Aortic valve structure
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Calcification
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Aortic insufficiency- aortic dissection
• Hypertension• Hypertension• Connective tissue disorders
Marfan’s: 1 in 5000.70-85% familial - Autosomal dominantFib illi 1 (15 21) 500 di ti tFibrillin-1 (15q21) ~ 500 distinct
mutations
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Medial degeneration without atherosclerosis
Mitral stenosis
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Acute Rheumatic Fever
Immunologically mediated following 1 6Immunologically mediated, following 1-6 weeks after pharyngitis with Group A Streptococcus in 3% of those infected.
Antibodies against streptococcal M proteins g p pcross-react with similar antigenic determinants in joints, heart, skin, CNS.
Acute Rheumatic Fever: Major manifestations (need 2 for diagnosis)1 Migratory polyarthritis in 75%1. Migratory polyarthritis in 75%2. Carditis in 50%3. Sydenham’s chorea in 10%(involuntary,
rapid, purposeless movements – caudate)4. Erythema marginatum of skin in 10%
(brown/pink with pale center)5. Subcutaneous nodules (0.5 – 2 cm;
extensor surfaces, occur late)
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Acute Rheumatic Fever
Minor Manifestations (need 2 plus 1 Major): Previous rheumatic fever, arthralgia, fever, , g , ,increased ESR or CRP, prolonged P-R interval.
With 2 Major or Major/2MinorSupportive evidence of streptococcal
infection: ASO Ab, anti-DNAase B, positive throat culture
Excellent prognosis: 1% mortality - cardiac
Acute Rheumatic Fever
PancarditisPericarditisPericarditisMyocarditis (Aschoff body)Endocarditis with sterile vegetations
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Aschoff body of acute rheumatic carditis: a cardiac granuloma
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Chronic Rheumatic Heart Disease
Follows Acute Rheumatic Heart Disease by decadesFollows Acute Rheumatic Heart Disease by decades.MV 65-70%MV & AV 25%
Mitral stenosis, aortic stenosis.Arrhythmias (A. fib. with left atrial enlargement).Heart failure.Predisposed to infective endocarditis.
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Chronic Rheumatic Heart Disease: “Fishmouth” deformity of the mitral valve
Mitral Valve Prolapse
• Most are asymptomaticMost are asymptomatic• Mid-systolic click• Rarely: chest pain, dyspnea, fatigue, or psychiatric
manifestations• ~3% have complications:
Infective endocarditisInfective endocarditisMitral insufficiencyThrombus formation with embolizationArrhythmia/sudden death (unknown mechanism)
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Aortic valve structure
Myxomatous, prolapsing mitral valve
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“Myxomatous” mitral valve
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Endocarditis
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Non-bacterial thrombotic endocarditis (NBTE)
• Small (1-5 mm) sterile masses of fibrin on• Small (1-5 mm) sterile masses of fibrin on lines of closure.
• Hypercoagulable state: patients are often debilitated (“marantic” endocarditis) or may have malignancy (mucinous adenocarcinoma) renal failure sepsisadenocarcinoma), renal failure, sepsis.
• May embolize to brain, kidney, heart.• With bacteremia, may be converted to
infectious endocarditis.
Non-bacterial thrombotic endocarditis (NBTE)
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NBTE: No inflammation or organism in thrombus
Rupture of mitral valve papillary muscle causing MR
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Heart Valve Prostheses: Complications
• Mechanical: component failure• Mechanical: component failure• Bioprostheses: degeneration with
calcification• Both: Paravalvular leak, endocarditis.
Thrombosis and tissue overgrowthThrombosis and tissue overgrowth are less common in bioprostheses than in mechanical valves.
Bioprosthetic (Hancock)valve: Degeneration and tissue overgrowth
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AV prosthesis with thrombus (from aortic side)
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Tissue overgrowth of MV prosthesis (from atrial side
Endocarditis of ball valve prosthesis (Starr-Edwards)