Abstract
This study reports the case of a 0-day-old girl who was transferred to our hospital for suspected total anomalous pulmonary venous return due to her postnatal cyanosis. After she was presented at our hospital, pulmonary vein stenosis was diagnosed and emergency surgery was planned. Preoperative computed tomography showed abnormal perfusion of the pulmonary veins into the left and right superior vena cava separately on the left and right sides. Given that the persistent left superior vena cava was refluxing into the coronary sinus, the coronary sinus was enlarged greatly, and the left atrial volume was small. In the neonatal period, the right pulmonary vein was anastomosed to the right atrium, and the left pulmonary vein was anastomosed to the unroofed coronary sinus. Thereafter, at 4 months of age, the patient underwent two-stage intracardiac repair with re-routing of the right pulmonary vein and extracardiac ligation of the left vena cava. The postoperative course was good. Intracardiac repair via a two-stage surgery was effective for total anomalous pulmonary venous return (Ib+Ib) with a persistent left superior vena cava and an enlarged coronary sinus.
