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Rheumatic heart disease

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WHO rheumatic heart disease fact sheet prevention benzathine penicillin

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https://www.who.int/news-room/fact-sheets/detail/rheumatic-h…

Rheumatic heart disease (RHD)

RHD is permanent damage to one or more heart valves after acute rheumatic fever, an autoimmune complication that can follow untreated group A streptococcal throat infection, usually in childhood. The immune response mistakenly attacks heart tissue, especially the mitral valve and sometimes the aortic valve.

What happens

  • Initial inflammation commonly causes valve leakage, especially mitral regurgitation.
  • Over years, recurrent rheumatic fever can scar, thicken, and fuse valve leaflets.
  • This can lead to mitral stenosis, regurgitation, or mixed valve disease.
  • Consequences include atrial fibrillation, heart failure, pulmonary hypertension, stroke from emboli, infective endocarditis, and complications in pregnancy.

Symptoms

Early RHD may have no symptoms. Later symptoms can include:
  • Breathlessness, reduced exercise tolerance, fatigue
  • Palpitations or irregular heartbeat
  • Leg swelling, orthopnea, coughing blood in severe mitral stenosis
  • Chest discomfort, dizziness, or fainting
Seek urgent care for severe breathlessness at rest, fainting, new neurologic symptoms, chest pain, or rapid persistent palpitations.

Diagnosis

The key test is echocardiography with Doppler, which defines which valve is affected and how severe the stenosis or regurgitation is. ECG and chest radiography help assess rhythm disturbance and cardiac enlargement. A history of rheumatic fever or childhood recurrent sore throat supports the diagnosis.

Management

Treatment depends on valve severity and complications:
  1. Secondary antibiotic prophylaxis to prevent recurrent rheumatic fever, commonly long-acting intramuscular benzathine penicillin G every 3-4 weeks for years. The exact regimen and duration must be individualized by a clinician, particularly for penicillin allergy and established valve disease.
  2. Medicines for complications, such as diuretics for congestion or heart failure, rate control for atrial fibrillation, and anticoagulation when indicated.
  3. Valve intervention for severe disease: balloon mitral valvotomy in suitable mitral stenosis, or surgical valve repair/replacement when necessary.
  4. Regular cardiology follow-up and echocardiographic surveillance.
Valve damage is generally permanent, but recurrent injury and progression can often be prevented. The WHO RHD guidance emphasizes prompt treatment of streptococcal sore throat and long-term prevention of recurrent infection in people with prior rheumatic fever or RHD.

Prevention

  • Prompt assessment and appropriate antibiotic treatment of suspected streptococcal sore throat.
  • Reliable secondary prophylaxis after rheumatic fever/RHD.
  • Reducing overcrowding and improving access to primary care also lowers risk.
The underlying mechanism is molecular mimicry: antibodies and T cells induced by streptococcal antigens cross-react with cardiac myosin and valve proteins, causing valvular inflammation and scarring. Firestein & Kelley's Textbook of Rheumatology, Rheumatic Heart Disease section.
Recent systematic reviews continue to show that RHD remains concentrated in resource-limited settings and has substantial global burden, including sex-related differences in prevalence (PMID 41282342).
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