Rheumatic Fever & Rheumatic Heart Disease

Key Features

Essentials of Diagnosis

• More common (100 cases/100,000 population) in developing countries than in the United States (approximately 2 cases/100,000 population)

• Peak incidence ages 5–15 years

• Revision of Jones criteria in 2015 includes echocardiographic findings

• May involve mitral and other valves acutely, rarely leading to heart failure

General Considerations

• Rheumatic fever

  • A systemic immune process that is a sequela of a β-hemolytic streptococcal infection of the pharynx

  • Pyodermic infections are not associated with rheumatic fever

  • Rheumatic carditis and valvulitis

    • May be self-limited

    • May lead to slowly progressive valvular deformity

    • A perivascular granulomatous reaction with valvulitis is characteristic lesion

• Chronic rheumatic heart disease

  • Results from single or repeated attacks of rheumatic fever that produce

    • Rigidity and deformity of valve cusps

    • Fusion of the commissures

    • Shortening and fusion of the chordae tendineae

  • Valvular stenosis or regurgitation results and the two often coexist

  • Affects mitral, aortic, and tricuspid valves, but the pulmonary valve only very rarely

Demographics

• Rheumatic fever

  • Rare before age 4 or after age 40 years

  • Responsible for 320,000 deaths in young people worldwide each year

  • Over 15 million have evidence for rheumatic heart disease

• Rheumatic heart disease remains a major cardiovascular problem in the poorest regions of the world

Clinical Findings

Symptoms and Signs

• Signs of acute rheumatic fever

  • Usually commence 2–3 weeks after infection

  • May appear as early as 1 week or as late as 5 weeks

Major Criteria

• Carditis

  • Pericarditis

  • Cardiomegaly

  • Heart failure, with painful liver engorgement due to tricuspid regurgitation

  • Mitral or aortic regurgitation murmurs

• Carey–Coombs short mid-diastolic mitral murmur may be present due to inflammation of the mitral valve

  • Occurs most often in children and adolescents

  • When any of the above definitive signs are absent, the diagnosis of carditis depends on the following less specific abnormalities:

    • ECG changes, including changing contour of P waves or inversion of T waves

    • Changing quality of heart sounds

    • Sinus tachycardia, arrhythmia, or ectopic beats

• Erythema marginatum

  • Begins as rapidly enlarging macules that assume the shape of rings or crescents with clear centers; may be less notable on Black skin

  • May be raised, confluent, and either transient or persistent

• Subcutaneous nodules

  • Small (≤ 2 cm in diameter), firm, and nontender

  • Persist for days or weeks and are recurrent

  • Attached to fascia or tendon sheaths over bony prominences

  • Indistinguishable from rheumatoid nodules

  • Uncommon except in children

• Sydenham chorea

  • Involuntary choreoathetoid movements primarily of the face, tongue, and upper extremities

  • May be the sole manifestation of rheumatic fever; only half of cases have other overt signs

  • Girls are more frequently affected; occurrence in adults is rare

  • Least common (3% of cases) but most diagnostic manifestation of acute rheumatic fever

• Polyarthritis

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