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Pathology of Valvular Diseases Peter B. Baker, M.D. Clinical Professor, Pathology

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Cardiopulmonary Block Objectives Describe the etiologies and pathologic changes causing stenosis and/or regurgitation of the cardiac valves Describe the changes in the cardiovascular system that occur with aging

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Objective for Part 1 List the etiologies and describe the pathologic features for aortic valve stenosis, aortic valve regurgitation, mitral valve stenosis and mitral valve regurgitation.

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Aortic Valve Stenosis Handout VIII Left ventricular pressure overload lead to concentric hypertrophy as the compensatory mechanism Etiologies 1. Congenital stenosis (often unicuspid valve) 2. Post-inflammatory valve disease (usually due to chronic rheumatic valvular disease) 3. Degenerative calcification on a previously normal valve 4. Congenital valve malformation with acquired stenosis (usually congenital bicuspid valve)

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Unicuspid Aortic Valve

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Congenital Bicuspid Aortic Valve Handout VIII.B.4 Incidence 1% M:F ratio 3:1 Hemodynamics 1. normal function for many years 2. stenosis usually acquired starting at about age 40 years 3. insufficiency (less frequent) myxomatous degeneration

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Congenital Bicuspid Aortic Valve

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Degenerative (senile) Calcification Handout VIII.B.3 Calcification on a previously normal 3-leaflet valve Stenosis usually does not occur until after age 65 years

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Degenerative Calcification in an Aortic Valve Aortic Valve with Degenerative Calcification

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Postinflammatory (chronic rheumatic) valvular disease Handout VIII.B.2 Fibrosis, commissural fusion and calcification Always associated with anatomic pathology in the mitral valve

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Aortic Valve with Chronic Rheumatic Valvular Disease

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Aortic Valve Regurgitation Handout IX Left Ventricular volume overload leads to eccentric hypertrophy as the compensatory mechanism Mechanisms of Aortic Valve Regurgitation 1. Valve cusps do not cover the orifice Infectious endocarditis Collagen disorders (ie Marfans syndrome) Chronic rheumatic valvular disease 2. Aortic root dilation enlarges the orifice Rheumatoid arthritis Syphilis

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Aortic Valve with Myxomatous Degeneration

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Aortic Valve Leaflet with Myxomatous Degeneration Myxomatous Degeneration

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Mitral Valve Regurgitation Handout X Left Ventricular volume overload leads to eccentric hypertrophy as the compensatory mechanism Causes of Mitral Valve Regurgitation 1. Cusp destruction 2. Leaflet prolapse 3. Rupture of chordae 4. Ischemic necrosis/fibrosis of papillary muscles 5. Severe ventricular dilation

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Normal Mitral Valve AL PL PM Normal Mitral Valve

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Floppy Mitral Valve (Severe Mitral Valve Prolapse) Handout X.B.2 Gross Pathology Large cusps Ballooning or hooding Elongated chordae tendineae Fibrous thickening of endocardial surface

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Floppy Mitral Valve with Ballooning of Leaflets Floppy Mitral Valve with Ballooning of Leaflets

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Normal Mitral Valve Histology

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Floppy Mitral Valve Histology

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Comparison of Normal and Floppy Mitral Valves Comparison of Normal (Bottom) and Floppy (Top) Mitral Valves

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Floppy Mitral Valve Complications Handout X.B.2.b Rupture of chordae tendineae Infectious endocarditis Thromboembolism Arrhythmia Sudden death

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Mitral Valve Stenosis Postinflammatory Valvular Disease Handout XI Gross Pathology Diffuse fibrosis Commissural and scallop fusion Fusion of chordae tendineae Shortening of chordae tendineae Calcification Funnel-shaped valve apparatus

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Mitral Valve with Chronic Rheumatic Valvular Disease AL PL Mitral Valve with Chronic Rheumatic Valvular Disease

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Chronic Rheumatic Mitral Valve Disease with Severe Left Atrial Dilation LA

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Objectives for Part 2 Describe the pathologic features and list a few complications of mitral valve annular calcification List the pathologic conditions that cause stenosis and/or regurgitation of the tricuspid and pulmonary valves Name the major 2 types of prosthetic valves and list the complications of prosthetic valves.

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Calcification of Mitral Valve Annulus Handout XII.A

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Tricuspid Valve Tricuspid Valve Regurgitation 1. Annular dilation due to right ventricular dilation 2. Infectious endocarditis 3. Chronic rheumatic valvular disease 4. Carcinoid tumors Tricuspid Valve Stenosis 1. Chronic rheumatic valvular disease

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Pulmonary Valve Pulmonary valve regurgitation 1. Infectious endocarditis 2. Carcinoid tumors Pulmonary valve stenosis 1. Congenital stenosis

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Types of Prosthetic Valves Prosthetic Valves

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Complications of Prosthetic Valves Mechanical Bioprosthetic Thromboembolism+++ Paravalvular leak++ Infection++ Deterioration+++ Fibrosis++ Hemolysis+rare

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Thank you for completing this module I hope that I was able to teach the subject clearly. If you have any questions or feedback, write to me. Peter.Baker@ChildLab.com

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