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Clinical features

Authoring team

Symptoms rarely develop until 20 to 30 years of age, and include:

  • cyanosis
  • dyspnoea
  • fatigue
  • chest pain
  • syncope
  • haemoptysis

Examination reveals:

  • right ventricular heave
  • loud second heart sound with a narrow split
  • ejection systolic murmur audible along the left sternal border
  • Graham Steel murmur

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