Orthotopic heart transplantation for congenital heart defects: anomalies of the systemic venous return.

Anomalies of the systemic venous return are frequently associated with other congenital heart defects. Some anomalies do not complicate really orthotopic heart transplantation (such as azygos continuation of the inferior vena cava). Other anomalies raise more difficulties; the most frequent one is persistent left superior vena cava draining into either the coronary sinus or the left atrium. Sometimes, the left superior vena cava can be ligated without untoward effect. In most cases, the left superior vena cava must be anastomosed to the right atrial compartment, preferably using extracardiac procedures. Most problems can be solved by harvesting extra lengths of donor superior vena cava and innominate vein. In rare patients, associated anomalies of the pulmonary venous return may require additional partitioning of the atria. The early risk of heart transplantation is probably not increased by the presence of such anomalies.