Surgical treatment of absent pulmonary valve syndrome in infants: relief of bronchial obstruction.

Congenital absence of the pulmonary valve is usually associated with a malalignment type of ventricular septal defect as well as right ventricular outflow obstruction and aneurysmal dilatation of the pulmonary arteries. Symptomatic infants primarily have severe tracheobronchial obstruction caused by aneurysmal dilatation of the proximal pulmonary arteries; pulmonic and systemic blood flow are usually balanced. Surgical intervention to relieve the obstruction by plicating the pulmonary artery and its branches under deep hypothermia and circulatory arrest, together with patch closure of the ventricular septal defect, is advocated.