[Unusual total anomalous pulmonary venous connection; report of a case]

We report a case of a 7-hour-old infant with total anomalous pulmonary venous connection having abberant origin of the vertical vein. His clinical condition presented hemodynamically severe pulmonary vein obstruction as common pulmonary vein atresia. Without making the definitive diagnosis, he underwent the operation at 11 hours after birth under cardiopulmonary bypass. During the operation, we could not identify the drainage vein. Although we ligated the vessel sized less than 2 mm in diameter draining into superior vena cava which was suspected to be the vertical vein. After the operation pulmonary edema was severe, but his clinical condition improved by using nitric oxide without extracorporeal membrane oxygenation (ECMO) support. Postoperative cardiac catheterization showed normal cardiac function without pulmonary venous obstruction, and moreover identified the drainage vein originated from right upper pulmonary vein. To the best of our knowledge, this is the first case where the vertical vein originated from right upper pulmonary vein. Immediate surgical treatment before the circuratory exacerbation improve the outcome for this congenital anomaly.