Chapter 4.9 Rheumatic heart disease

Chronic rheumatic heart disease is a complication of acute rheumatic fever, which leaves permanent damage to the heart valves. In some children, antibodies produced in response to group A ß-haemolytic streptococci lead to varying degrees of pancarditis, with associated valve insufficiency in the acute phase.

The risk for rheumatic heart disease is higher with repeated episodes of acute rheumatic fever. It leads to valve stenosis, with varying degrees of regurgitation, atrial dilatation, arrhythmia and ventricular dysfunction. Chronic rheumatic heart disease is a major cause of mitral valve stenosis in children.

Diagnosis

Rheumatic heart disease should be suspected in any child with a previous history of rheumatic fever who presents with heart failure or is found to have a heart murmur. Diagnosis is important because penicillin prophylaxis can prevent further episodes of rheumatic fever and avoid worse damage to the heart valves.

The presentation depends on the severity. Mild disease may cause few symptoms except for a heart murmur in an otherwise well child and is rarely diagnosed. Severe disease may present with symptoms that depend on the extent of heart damage or the presence of infective endocarditis.

History

  • chest pain
  • heart palpitations
  • symptoms of heart failure (including orthopnoea, paroxysmal nocturnal dyspnoea and oedema)
  • fever or stroke usually associated with infection of damaged heart valves
  • breathlessness on exertion or exercise
  • fainting (syncope)

Examination

  • signs of heart failure
  • cardiomegaly with a heart murmur
  • signs of infective endocarditis (e.g. conjunctival or retinal haemorrhages, hemiparesis, Osler nodes, Roth spots and splenomegaly)

Investigations

  • chest X-ray: cardiomegaly with congested lungs
  • an echocardiogram, if available, is useful for confirming rheumatic heart disease, the extent of valve damage and evidence of infective endocarditis.
  • full blood count
  • blood culture

Management

  • Admit the child if in heart failure or has suspected bacterial endocarditis.
  • Treatment depends on the type and extent of valvular damage.
  • Manage heart failure if present.

Give diuretics to relieve symptoms of pulmonary congestion and vasodilators when necessary.

Give penicillin or ampicillin or ceftriaxone plus gentamicin IV or IM for 4–6 weeks for infective endocarditis.

Refer for echocardiographic evaluation and decision on long-term management. May require surgical management in severe valvular stenosis or regurgitation.

Follow-up care

  • All children with rheumatic heart disease should receive routine antibiotic prophylaxis.

Give benzathine benzylpenicillin at 600 000 U IM every 3–4 weeks

  • Ensure antibiotic prophylaxis for endocarditis before dental and invasive surgical procedures.
  • Ensure that vaccinations are up to date.
  • Review every 3–6 months, depending on severity of valvular damage.

Complications

Infective endocarditis is more common. It presents with fever and heart murmur in a very unwell child. Treat with ampicillin and gentamicin for 6 weeks.

Atrial fibrillation or thromboembolism may occur, especially in the presence of mitral stenosis.