Repair of a simple total anomalous pulmonary venous connection coexisting with a persistent left superior vena cava

Abstract

A simple total anomalous pulmonary venous connection (TAPVC) coexisting with a persistent left superior vena cava (PLSVC) is extremely rare. Connection of the PLSVC with the coronary sinus behind the left atrium induces coronary sinus dilatation. This reduces the free posterior wall space to which the common pulmonary vein is anastomosed for repairing the anomalous connection. Postoperative recurrent pulmonary venous obstruction (PVO) is the most important complication. To prevent PVO, sufficient tension-free anastomosis is necessary. When dilated, the coronary sinus becomes an obstacle for obtaining sufficient incision length in the left atrial cavity. We encountered two cases of a simple TAPVC with a PLSVC in infants weighing 1.8 and 2.9 kg, respectively. To obtain sufficient incision length, we extended the incision line to the right atrium for an atypical supracardiac TAPVC and incised from the left atrium to the coronary sinus via the right atrium for an infracardiac TAPVC. Moreover, we recreated the atrial septum with a rightward shift using a tanned pericardium in both cases. The postoperative courses were uneventful, without recurrent PVO.