Congenital absence of the pulmonary valve associated with ventricular septal defect

The authors describe 5 cases of an unusual variant of tetralogy of Fallot with a characteristic clinical course. Its most prominent clinical manifestations are recurrent and severe respiratory infections caused by compression of the inferior trachea and bronchi by a dilated pulmonary artery. Two of the patients died of irreversible respiratory insufficiency. Of the 3 surviving patients one is doing comparatively well.