Isolated unilateral agenesis of the pulmonary artery: surgical repair with an artificial graft.

This report describes a 3-month-old Japanese boy with a diagnosis of isolated unilateral agenesis of the proximal right pulmonary artery with severe pulmonary hypertension. One-stage reconstruction of the right pulmonary artery was performed without cardiopulmonary bypass. The hilar right pulmonary artery and the distal main pulmonary artery were joined by anastomosis to an artificial ring graft. The boy's postoperative course was uneventful, and the pressure in the pulmonary artery was within the normal range. Although mild right pulmonary artery stenosis remained, the authors' therapeutic strategy may provide a clinically important option for isolated unilateral agenesis of the pulmonary artery.