Rheumatic Fever, Infectious Endocarditis & Misc. Heart Disease

Rheumatic Fever Systemic non-suppurative inflammatory disease Inflammation of connective tissue Edema, fibrinoid degeneration Hypersensitivity reaction to beta-hemolytic Group A Streptococci

RF 2-3 weeks after Group A Strep infection Age 5-15 years Sex - equal Social economic - poor, malnourished, crowded conditions, or rural isolated - Wy, Idaho, Utah

Major Manifestations Carditis Polyarthritis Chorea Erythema marginatum Subcutaneous nodules

Minor Manifestations Clinical findings Laboratory findings Arthralgia Fever Laboratory findings Elevated acute phase reactants Erythrocyte sedimentation rate C-reactive protein Prolonged PR Interval

Supporting Evidence of Antecedent Group A Streptococcal Infections Positive throat culture or rapid streptococcal antigen test Elevated or rising streptococcal antibody titer

Acute RF Pancarditis pericarditis - fibrinous myocarditis - Aschoff bodies and edema endocarditis - foci of inflammation and fibrinoid degeneration within the cusps or along the tendinous cords with small fibrinous vegetations - verrucae

Extra cardiac Lesions Subcutaneous nodules Polyarthritis CNS - Sydenham’s chorea

Sequelae of RF 1% die of acute carditis Progressive stenosis of valves Atrial fibrillation Mural thrombosis Congestive heart failure Bacterial endocarditis

Acute rheumatic fever– Acute fibrinous Pericarditis- Bread & Butter type

edema Giant Anitschkow cells Fibrinoid degeneration Anitschkow cells Aschoff body- poorly formed granuloma

Aschoff bodies Foci of fibrinoid degeneration Lymphocytes (T-cells) Macrophages-Anitschkow cells Large elongated nuclei - caterpillar cells May form giant cells

Acute rheumatic fever – fibrin vegetations along lines of Closure (verrucus endocarditis)

Chronic Rheumatic Disease Organization of the acute inflammatory process and subsequent deforming fibrosis Mitral stenosis alone: 65 -70% Mitral and aortic: 25 %

Normal mitral valve Chronic rheumatic endocarditis

Chronic rheumatic endocarditis – mitral stenosis

Chronic rheumatic stenosis- fibrous thickening & fusion of Chordae tendinae

Infective Endocarditis Every form of micro-organism has been implicated Acute form - highly virulent organisms (Staph, Strep, fungal - on normal valves Sub acute - less virulent organisms (Strep viridans)- damaged valves

Pathogenesis of IE Sterile platelet -fibrin deposits Agglutinating antibodies cause clumps of organisms likely to attach to fibrin Bacterial adhesion factors

Infective endocarditis with destruction of the aortic valve

Acute infective endocarditis- note perforation (blue area) Pulmonary valve Aortic valve Acute infective endocarditis- note perforation (blue area)

Roth spots

Osler’s nodes & Janaway spots In SBE, (rare to see Today)

Destruction of a damaged (rf) stenotic mitral valve by Stept. viridans

Complications of IE Cardiac Embolic Valvular disease with CHF Myocardial abscess Suppurative pericarditis Dehiscence of artificial valve Embolic To brain, spleen, kidney, etc.

Complications of IE Metastatic infections Renal any organ or site Focal glomerulonephritis (immune) Diffuse GN (immune) Multiple abscesses

Non-infectious Endocarditis Libman-Sacks (SLE) Non-bacterial thrombotic (marantic) (Terminal DIC syndrome) Hypercoagulable state - mucinous adenocarcinoma - pancreas

Complications of Artificial Valves Paravalvular leak Thromboembolism Infective endocarditis Structural deterioration Occlusion by tissue overgrowth

Pericardial Effusion Hemopericardium- blood Dissection Trauma Hydropericardium-- low protein Heart failure Nutrition-- low albumin

Pericarditis Serous pericarditis- SLE, Uremia etc Scant inflammatory cells Slow build up Fibrinous & serofibrinous most common Acute MI, uremia, SLE, RF Friction rub Purulent or suppurative- Pus, PNMs Bugs- if bloody think TB Hemorrhagic- tumor (breast or lung), TB, bleeding diathesis

Chronic Pericarditis Adhesive Mediastinopericarditis Follows supprative pericarditis, staph, TB, or cardiac surgery or radiation Leads to cardiac hypertrophy and dilatation -Mimics DCM Constrictive pericarditis- staph, TB Restricts cardiac output- mimics restrictive cardiomyopathy

Summary Mitral Stenosis : Rheumatic Heart Disease Mitral insufficiency: myxomatous degeneration (mitral valve prolapse), damaged papillary muscle due to infarct Aortic stenosis: calcification of normal and congenitally bicuspid aortic valves Aortic insufficiency: dilation of the ascending aorta, related to hypertension and aging