Rheumatic Heart Disease

Chronic, progressive valvular damage resulting from acute rheumatic fever (ARF), which is an autoimmune, post-streptococcal inflammatory disease caused by group A β-hemolytic Streptococcus (GABHS) pharyngitis.

Epidemiology

  • Common in developing countries
  • Peak age: 5–15 years for ARF onset
  • Valvular complications appear years later (adolescence–adulthood)
  • Leading cause of acquired heart disease in young adults globally

Pathophysiology

  1. Streptococcal pharyngitis → untreated
  1. Immune response creates antibodies against M-protein
  1. Cross-reactivity with cardiac tissue (molecular mimicry)
  1. Pancarditis (myocarditis, pericarditis, endocarditis)
  1. Chronic scarring → valvular deformity

Most Affected Valve:

  • Mitral valve (65-70%) → stenosis > regurgitation
  • Aortic valve (25%)
  • Tricuspid/Pulmonary rarely involved

Clinical presentation

Acute Rheumatic Fever (Jones Criteria)

Occurs 2–3 weeks after sore throat
Major criteria:
  • Carditis (pancarditis)
  • Migratory polyarthritis
  • Chorea (Sydenham chorea)
  • Erythema marginatum
  • Subcutaneous nodules
Minor criteria:
  • Fever
  • Arthralgia
  • ↑ ESR/CRP
  • Prolonged PR interval
Diagnosis requires evidence of recent GAS infection + 2 major OR 1 major + 2 minor.

Chronic Rheumatic Heart Disease

Symptoms develop years later
Symptoms
  • Dyspnea on exertion (most common)
  • Orthopnea, PND
  • Fatigue, palpitations (AF)
  • Hemoptysis (pulmonary congestion)
  • Embolic events (AF + LA thrombus)
Signs
  • Mitral facies (pink-purple cheeks)
  • Loud S1, Opening snap (OS) in MS
  • Mid-diastolic rumbling murmur at apex (MS)
  • Systolic murmur in MR
  • Signs of heart failure in advanced stages

Investigations

  • Echocardiography: gold standard
    • Valve thickening, restricted mobility
    • LA enlargement, pressure gradients
  • ECG: AF, P-mitrale, PR prolongation
  • Chest X-ray: LA enlargement, pulmonary congestion
  • ASO titers / anti-DNase B for streptococcal evidence

Management

Acute Rheumatic Fever

  • Penicilline g Benzathine
    • ≥ 27 kg → 1.200.000 unit IM SD
    • < 27 kg → 600.000 unit IM SD
  • Aspirin/NSAIDs for arthritis
  • Steroids for severe carditis
  • Treat heart failure if needed

Chronic Rheumatic Heart Disease

  • Long-term secondary prophylaxis with penicillin to prevent recurrence
    • Given every 3 week
    • < 30 kg → 600.000 unit IM
    • ≥ 30 kg → 1.200.000 unit IM
  • Diuretics for congestion
  • β-blockers/diltiazem/digoxin for rate control in AF
  • Anticoagulation for AF, large LA, prior embolism

Secondary Prevention (Prophylaxis Schedule & Duration)

  • Rheumatic fever without cardiac involvement: 5 years or until age 18
  • Carditis without residual disease: 10 years or until age 25
  • Carditis with valvular disease: 10 years or until age 40 or lifelong