Key Facts

Rheumatic heart disease (RHD) is the chronic cardiac manifestation of acute rheumatic fever (ARF), an autoimmune inflammatory condition triggered by Group A streptococcal (GAS) pharyngitis. It results in permanent valvular damage, most commonly mitral stenosis.

Epidemiology

• ~33 million people affected worldwide; >300,000 deaths annually
• Predominantly affects children and young adults (5-15 years for ARF)
• Rare in UK (<1 per 100,000); common in developing countries, Aboriginal populations, Pacific Islanders
• UK cases predominantly in immigrant populations from endemic areas

Aetiology

• Acute rheumatic fever: autoimmune response 2-4 weeks after untreated GAS pharyngitis
• Molecular mimicry: antibodies against streptococcal M protein cross-react with cardiac myosin, valvular glycoproteins, and other tissues
• Recurrent episodes of ARF cause cumulative valve damage
• Genetic susceptibility: HLA associations (HLA-DR7, HLA-DR4)

Pathophysiology

• Acute: pancarditis (endocarditis, myocarditis, pericarditis) with Aschoff nodules (pathognomonic granulomatous lesions)
• Chronic: valvular scarring, commissural fusion, leaflet thickening, chordal shortening
• Mitral valve: stenosis (most common), regurgitation, or mixed
• Aortic valve: second most commonly affected
• Tricuspid/pulmonary: rare in isolation
• Recurrent ARF episodes → progressive valve damage → heart failure

Acute Rheumatic Fever

• Presents 2-4 weeks after GAS pharyngitis
• Migratory polyarthritis (most common manifestation, ~75%; large joints)
• Carditis (~50-60%): pericarditis, myocarditis, valvulitis (new murmur — typically MR)
• Sydenham chorea (~15%): involuntary purposeless movements, emotional lability
• Erythema marginatum (~5%): annular erythematous rash, trunk
• Subcutaneous nodules (~5%): firm, painless nodules over extensor surfaces

Chronic Rheumatic Heart Disease

• Symptoms of mitral stenosis: dyspnoea, palpitations (AF), haemoptysis
• Heart failure: orthopnoea, PND, peripheral oedema
• Embolic events: stroke, peripheral embolism (especially with AF)
• Murmur characteristics depend on valve lesion (see Mitral Stenosis topic)

Red Flags

• New murmur with fever in a child/young adult
• Chorea in a school-age child
• Heart failure in a young person from an endemic area
• Recurrent sore throats without appropriate treatment

Bedside

• ECG: prolonged PR interval (minor criterion), AF in chronic RHD
• Temperature: fever >38.5°C (minor criterion)

Bloods

• ASOT (anti-streptolysin O titre): elevated (peaks 4-5 weeks post-infection)
• Anti-DNase B: elevated; more reliable than ASOT
• ESR/CRP: elevated (minor criteria)
• FBC: leucocytosis, normocytic anaemia
• Throat swab: may be negative by time of ARF (but confirms GAS if positive)
• Blood cultures: to exclude endocarditis

Imaging

• Echocardiography: valvular regurgitation/stenosis, pericardial effusion, myocardial dysfunction
  • Subclinical carditis may be detected by echo alone
• CXR: cardiomegaly, pulmonary congestion

Jones Criteria for ARF Diagnosis

Requires: evidence of preceding streptococcal infection (ASOT, anti-DNase B, positive culture) PLUS:

• 2 major criteria, OR
• 1 major + 2 minor criteria

Major: carditis, migratory polyarthritis, chorea, erythema marginatum, subcutaneous nodules Minor: fever, arthralgia, prolonged PR, elevated ESR/CRP

Non-pharmacological

• Bed rest during acute carditis
• Education about secondary prophylaxis adherence

Pharmacological

Acute rheumatic fever:

• Benzylpenicillin (or phenoxymethylpenicillin) to eradicate streptococcal infection
• Aspirin (high-dose): joint inflammation — 80-100mg/kg/day in children (monitor salicylate levels)
• Corticosteroids (prednisolone 1-2mg/kg/day): for severe carditis with heart failure
• Carbamazepine or valproate: for Sydenham chorea if needed
• Heart failure management: diuretics, ACEi as appropriate

Secondary prophylaxis (prevention of recurrent ARF):

• Phenoxymethylpenicillin 250mg BD orally
• OR IM benzathine penicillin G 1.2 MU every 3-4 weeks (more reliable)
• If penicillin-allergic: erythromycin 250mg BD
• Duration: at least 10 years from last episode, or until age 40 (whichever is longer); lifelong if severe RHD

Surgical/Interventional

• Valve surgery as per specific lesion (see Mitral Stenosis, Mitral Regurgitation topics)
• PMBV: for suitable mitral stenosis
• Valve replacement: if severe valve destruction

Referral Criteria

• Suspected ARF: urgent paediatric/medical admission
• Chronic RHD with valve disease: cardiology follow-up
• Heart failure or embolic events: specialist management

• ARF with carditis: ~60-70% develop chronic RHD
• ARF without carditis: excellent prognosis, no chronic valve disease
• Recurrent ARF episodes: cumulative valve damage with each episode
• Secondary prophylaxis reduces recurrence by >80%
• Chronic RHD: prognosis depends on severity of valve lesion (see specific valve topics)
• In developing countries: RHD remains a major cause of cardiovascular death in young people
• With modern surgical/interventional treatment: outcomes have improved significantly

Jones Criteria Summary

Secondary Prophylaxis Duration