Congenital absence of pulmonary valve leaflets with intact ventricular septum in a neonate

Symptomatic infants with congenital absence of pulmonary valve leaflets suffer primarily from respiratory insufficiency caused by bronchial compression by the dilated pulmonary arteries, and have a high mortality rate. We report the successful treatment of absent pulmonary valve syndrome with intact ventricular septum in a neonate. The treatment consisted of resection of the pulmonary artery aneurysm and enlargement of the pulmonary annulus.