Anomalous pulmonary venous return in the staged palliation of functional univentricular heart defects

Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic, pulmonary, or systemic and pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure.

Between March 1990 and March 1997, 32 patients with functional single ventricle and anomalous pulmonary venous return underwent operation at our institution. Five of 25 patients who underwent neonatal palliation died in the early postoperative period, all of whom had obstructed anomalous pulmonary venous return. Twenty-one patients have undergone bidirectional cavopulmonary shunt, including 7 in whom this was the primary palliative procedure. There was one early and two late deaths after the bidirectional Glenn procedure, two in patients with asplenia syndrome and none in patients with previously obstructed pulmonary venous return. Seven patients have undergone Fontan completion, 5 with an extracardiac conduit. There was one early death and one take-down to a classic Glenn shunt, both in patients who did not undergo the extracardiac conduit Fontan operation.

Anomalous pulmonary venous return can significantly complicate the management of the single-ventricle patient, with the major impact on survival coming in the neonatal period. Palliation with the aim of performing an extracardiac conduit Fontan procedure allows greater latitude and more streamlined management in this group of patients.