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Rheumatic Heart Disease

Chronic valvular damage (especially mitral stenosis) resulting from acute rheumatic fever — an autoimmune sequela of Group A Streptococcal pharyngitis

Review date
reviewed 2026-05-04
Reading time
5 min read
Author
Dr Kola Tytler MBBS CertHE MBA MSt MRCGP
Cardiovascularless-commonlong-term-condition
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Condition details
Cardiovascular
less-common
5 min read
reviewed 2026-05-04
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About This Page

This is a clinician-written, evidence-based summary aligned to the USMLE Step 2 CK Content Outline. It is intended for medical students preparing for USMLE Step 2 CK. Management reflects current ACC/AHA, USPSTF, and APA guidelines. Always cross-reference with UpToDate, institutional protocols, and clinical judgment.

Key points

  • Acute rheumatic fever: autoimmune inflammatory response 2-4 weeks after GAS pharyngitis (molecular mimicry with cardiac tissue)
  • Jones criteria for initial episode: 2 major or 1 major + 2 minor manifestations + evidence of preceding GAS infection
  • Major criteria: carditis (pancarditis), polyarthritis (migratory, large joints), chorea (Sydenham), erythema marginatum, subcutaneous nodules
  • Mitral valve is most commonly affected (MS > MR). Aortic valve second most common
  • Secondary prophylaxis: IM benzathine penicillin G q4 weeks (or PO penicillin V BID) — duration depends on severity and age

Overview

Acute rheumatic fever (ARF) is an autoimmune inflammatory disease triggered by Group A Streptococcal (GAS) pharyngitis through molecular mimicry — antibodies against streptococcal M protein cross-react with cardiac tissue (myosin, laminin, valve glycoproteins). Rheumatic heart disease (RHD) refers to the chronic valvular damage resulting from recurrent or severe ARF episodes. The mitral valve is most commonly and severely affected, typically causing mitral stenosis (from commissural fusion and leaflet thickening). ARF primarily affects children aged 5-15 in resource-limited settings.

Epidemiology

RHD remains a major global health burden, affecting ~33 million people worldwide, predominantly in sub-Saharan Africa, South/Southeast Asia, and Pacific Islands. In the US, ARF has become rare due to improved living conditions and antibiotic treatment of GAS pharyngitis, but cases still occur in underserved populations. RHD is the most common acquired heart disease in children and young adults in developing countries. ARF occurs 2-4 weeks after untreated or inadequately treated GAS pharyngitis — it does NOT follow GAS skin infections (impetigo).

Clinical Features

Symptoms
Preceding pharyngitis 2-4 weeks before onset (sore throat, fever — may be mild or subclinical)
Fever, malaise, arthralgia
Migratory polyarthritis (large joints — knees, ankles, elbows, wrists; most common manifestation, ~75%)
Chest pain, dyspnea (carditis — new murmur, pericarditis, myocarditis, HF)
Involuntary purposeless movements (Sydenham chorea — may appear months after pharyngitis, more common in girls)
Signs
New murmur of MR (apical holosystolic) or MS (diastolic rumble) — carditis is the only manifestation that causes permanent damage
Erythema marginatum: pink/red non-pruritic serpiginous rash on trunk and proximal extremities, sparing the face. Evanescent (comes and goes)
Subcutaneous nodules: painless, firm, pea-sized nodules over bony prominences and extensor surfaces. Associated with severe carditis
Sydenham chorea: involuntary movements, emotional lability, muscle weakness. May occur in isolation months after pharyngitis
Arthritis: swollen, red, tender large joints in a migratory pattern (one joint improves as another flares). Dramatically responsive to aspirin/NSAIDs

Investigations

First-line
Evidence of preceding GAS infectionRequired for Jones criteria: elevated or rising ASO titer (most commonly tested), anti-DNase B, positive throat culture, positive rapid antigen detection test. Note: throat culture may be negative by the time ARF presents
Acute phase reactantsElevated ESR and CRP (minor criteria). CRP rises and falls faster than ESR
ECGProlonged PR interval (first-degree AV block — minor criterion). Does not correlate with severity of carditis
EchocardiogramSubclinical carditis: mitral/aortic regurgitation, pericardial effusion. Doppler findings of valvular regurgitation count as carditis even without auscultatory murmur (in 2015 revised Jones criteria)
Second-line
CBCLeukocytosis, elevated ESR, anemia of chronic disease
Blood culturesIf endocarditis is in the differential (especially if high fever + new murmur)

Management

AHA 2015 Revised Jones Criteria + WHO Guidelines on Rheumatic Fever and RHD
1
Acute rheumatic fever
  • Eradicate GAS: benzathine penicillin G 1.2 million units IM x 1 dose (or penicillin V PO x 10 days, or amoxicillin PO x 10 days). Azithromycin if penicillin allergy
  • Anti-inflammatory for arthritis: aspirin high-dose (80-100 mg/kg/day in children) or naproxen. Dramatic response within 24-48h
  • Carditis without HF: aspirin or naproxen
  • Carditis with HF: corticosteroids (prednisone 1-2 mg/kg/day) in addition to standard HF management (diuretics, ACEi). Taper over 2-3 weeks, overlap with aspirin/NSAID to prevent rebound
  • Sydenham chorea: usually self-limited (2-6 months). Carbamazepine or valproic acid if severe. Haloperidol alternative
  • Bed rest during acute phase (especially if carditis)
2
Secondary prophylaxis (prevention of recurrence)
  • Benzathine penicillin G 1.2 million units IM every 4 weeks (every 3 weeks in high-risk areas) — most effective
  • Alternative: penicillin V 250 mg PO BID, or sulfadiazine 1 g PO daily
  • Duration: ARF without carditis = 5 years or until age 21 (whichever is longer). ARF with carditis but no residual valvular disease = 10 years or until age 21. ARF with persistent valvular disease (RHD) = 10 years or until age 40 (some recommend lifelong)
  • This is one of the longest antibiotic prophylaxis regimens in medicine — compliance is critical
3
Chronic RHD management
  • Same as management of the specific valvular lesion: see Mitral Stenosis, MR, AR pages
  • Anticoagulation (warfarin) if AF + MS
  • Endocarditis prophylaxis: only if prosthetic valve or prior endocarditis (not for native RHD per AHA)
  • Surgical valve repair/replacement when indicated by severity and symptoms
USMLE Step 2 CK Exam Tips
  • 1Jones criteria: 2 major OR 1 major + 2 minor + evidence of prior GAS infection. Memorize the 5 major criteria (JONES: Joint/polyarthritis, O-carditis, Nodules, Erythema marginatum, Sydenham chorea)
  • 2Carditis is the ONLY manifestation that causes permanent damage (RHD). Arthritis resolves without sequelae
  • 3Erythema marginatum: serpiginous pink rash on trunk, spares face, evanescent. Subcutaneous nodules = severe carditis
  • 4Rheumatic fever follows pharyngitis ONLY — never skin infections (impetigo → post-strep GN, not ARF)
  • 5Migratory polyarthritis of large joints + recent pharyngitis + elevated ASO = rheumatic fever. Responds dramatically to aspirin
  • 6Prolonged PR interval on ECG is a minor criterion, NOT a sign of severe carditis
  • 7Secondary prophylaxis with penicillin is critical to prevent recurrence — duration depends on severity (up to lifelong if RHD)
  • 8Sydenham chorea can present in isolation months after pharyngitis and is sufficient for ARF diagnosis even without other Jones criteria if GAS evidence is present

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Verified Sources & References

AHA 2015 Revised Jones Criteria
WHO Technical Report on Rheumatic Fever and RHD
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