Clinical features

The clinical picture is variable depending upon the nature of any associated defects and the haemodynamics in the particular patient concerned.

Single ventricle with pulmonary stenosis:

• cyanosis present in infancy and increases through childhood
• clubbing
• polycythaemia
• dyspnoea
• systolic ejection murmur, systolic thrill and left parasternal lift

Single ventricle without pulmonary outflow obstruction:

• tachypnoea
• dyspnoea
• recurrent pulmonary infections
• congestive heart failure
• cardiomegaly
• mild or moderate cyanosis
• systolic ejection murmur and left parasternal lift

NB the development of pulmonary vascular disease in children who have not had surgical correction may lead to restriction of pulmonary blood flow, increasing cyanosis and improvement in the signs of cardiac failure.