28 Eisenmenger syndrome
Salient features
History
• Symptoms may not appear until early late childhood or early adulthood
• Cyanosis (appears as right-to-left shunting develop)
• Dyspnoea on exercise and impaired exercise tolerance
• Palpitations (common and usually caused by atrial fibrillation or flutter)
• Haemoptysis (may occur as a result of pulmonary infarction, or rupture of dilated pulmonary arteries or aorticopulmonary vessels)
• Syncope (owing to inadequate cardiac output or, less commonly, an arrhythmia)
• Symptoms of hyperviscosity including visual disturbances, fatigue, headache, dizziness and paraesthesia
• Symptoms of heart failure are uncommon until the disease is in advanced stages.
Examination
• Clubbing of fingers and central cyanosis
• An ‘a’ waves in the JVP, ‘v’ wave if tricuspid regurgitation is also present
• Left parasternal heave and palpable P2
• Loud P2, pulmonary ejection click, early diastolic murmur of pulmonary regurgitation (Graham Steell murmur)
• Loud pansystolic murmur of tricuspid regurgitation
• Listen carefully to the second sound. The clinical findings from the underlying defect are as follows:
