Clinical features
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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