Modified Repair of Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

A modified technique for the repair of tetralogy of Fallot with absent pulmonary valve syndrome and stenosis at the origin of the lobar or segmental pulmonary artery branches is described. This consists of generous reduction plasty of the redundant anterior and posterior walls of the aneurysmally dilated branches of the pulmonary artery, resection of the main pulmonary artery, pericardial patch augmentation of the stenotic origins of the lobar or segmental pulmonary artery branches, insertion of a valved pulmonary allograft, and closure of the ventricular septal defect. The advantages of this technique, in addition to relief of the left-to-right shunt, consist of complete decompression of the tracheobronchial structures, and restoration of the capacitance of the central pulmonary arteries. This may result in a reduced tendency for postoperative respiratory insufficiency as well as long-term preservation of right ventricular compliance. The technique was successfully performed on 2 critically ill neonates and a 3-month-old infant.