Surgical management of univentricular heart with total anomalous pulmonary venous drainage and intrapulmonary vertical vein.

We report a patient with univentricular heart, heterotaxy syndrome with total anomalous pulmonary venous connection, and an intrapulmonary vertical vein draining into the left superior vena cava who underwent a successful bidirectional Glenn shunt without cardiopulmonary bypass. A polytetrafluoroethylene tube interposition graft was used between the superior vena cava and the pulmonary artery. This condition is unusual and physiologic palliation, rather than anatomic correction as described in this report, may be appropriate for managing this condition.