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• 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

Aschoff body- poorly formed granuloma

Giant Anitschkow cells Fibrinoid degeneration Anitschkow

edema

cells

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)Acute infective endocarditis- note perforation (blue area)

Pulmonary valvePulmonary valve Aortic valveAortic valve

Roth spotsRoth spots

Osler’s nodes &Osler’s nodes &Janaway spotsJanaway spotsIn SBE, (rare to seeIn SBE, (rare to seeToday)Today)

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

•Complications of IE

Cardiac• Valvular disease with CHF• Myocardial abscess• Suppurative pericarditis• Dehiscence of artificial valve

Embolic• To brain, spleen, kidney, etc.

Complications of IE

Metastatic infections• any organ or site

Renal• 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