TOTAL ANOMALOUS PULMONARY VENOUS CONNECTIONS AND CONSIDERATION OF THEFONTAN OR ONE-VENTRICLE REPAIR

There is now a considerable literature that babies with right atrial i somerism have a poor outcome. The reasons for this are complex and mul tifactorial, but may be related at least in part to intrinsically smal l and abnormal pulmonary veins. We reviewed a series of consecutive pa tients seen at a single institution and found that babies with right a trial isomerism, severe pulmonary outflow tract obstruction or atresia , and total anomalous obstructed pulmonary veins had a grim outlook, e specially those requiring operation in the neonatal period. Others hav e reported a similarly concerning outcome. (C) 1998 by The Society of Thoracic Surgeons.