Rheumatic heart disease
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Rheumatic heart disease (RHD) is chronic valvular heart damage caused by prior acute rheumatic fever after untreated or undertreated group A streptococcal infection (usually pharyngitis).
High-yield summary
- Cause: autoimmune cross-reactivity after strep infection, not direct bacterial invasion of valves.
- Main valve affected: mitral valve (nearly always), then aortic valve.
- Typical lesion progression:
- early: valvular regurgitation
- late: fibrosis, commissural fusion, chordal thickening/shortening -> stenosis (classic mitral stenosis)
- Classic pathology:
- acute RF carditis: Aschoff bodies, verrucae along valve closure lines
- chronic RHD: thickened fibrotic leaflets, fused commissures, "fish-mouth" mitral stenosis
- Burden: now uncommon in many high-income settings, but still a major cause of cardiovascular morbidity/mortality in many low-resource regions.
Clinical picture
- Acute rheumatic fever usually appears about 2 to 3 weeks after strep pharyngitis.
- Jones-based manifestations include carditis, arthritis, chorea, erythema marginatum, subcutaneous nodules.
- RHD often presents years later with progressive valvular disease:
- dyspnea, reduced exercise tolerance
- palpitations/atrial fibrillation
- heart failure symptoms
- embolic risk (especially with enlarged left atrium/thrombus in mitral stenosis)
Diagnosis
- Echocardiography is central for detecting and grading valvular involvement (including subclinical carditis in acute RF and latent RHD in endemic areas).
- ARF diagnosis is clinical (Jones criteria with evidence of recent streptococcal infection); there is no single definitive test.
Management principles
- Secondary prophylaxis is essential to prevent recurrent ARF and worsening valve injury.
- Common regimen: intramuscular benzathine penicillin G every 4 weeks, long duration (often at least 10 years; sometimes longer depending on severity/risk).
- Treat valvular complications per severity:
- medical therapy for heart failure/rhythm control
- anticoagulation when indicated (for example AF with mitral stenosis)
- intervention for advanced disease: balloon mitral valvotomy (selected cases), valve repair/replacement when needed.
Pathology image (RHD)
This figure shows acute and chronic rheumatic valvular pathology (verrucae, Aschoff-body histology, commissural/chordal fibrosis, stenotic deformity):
Recent evidence check (last 2 years)
Recent systematic reviews/meta-analyses emphasize that prevention and adherence to secondary penicillin prophylaxis remain decisive for outcomes:
- PMID: 39542478
- PMID: 39312290
- PMID: 39960383
No major contradiction to core textbook principles, but these reviews reinforce that implementation quality (especially long-term prophylaxis adherence and timely valve intervention) strongly affects progression and outcomes.
Sources:
- Robbins, Cotran & Kumar Pathologic Basis of Disease, p. 525-527
- Goldman-Cecil Medicine, p. 3043-3044
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