Correction of total anomalous pulmonary venous connection in infancy.

From January 1985 through January 1993, 41 patients less than 1 year of age underwent operative correction of isolated total anomalous pulmonary venous connection. There were 24 boys and 17 girls. The median age at operation was 13 days (range 1 to 282 days) and weight was 3.6 kg (2.5 to 5.2 kg). Locations of the connections were supracardiac in 19, cardiac in 9, infracardiac in 11, and mixed supracardiac and cardiac in 2. Obstruction of the pulmonary veins was severe in 24, mild in 3, and absent in 14. Preoperative stabilization included mechanical ventilation for 15 patients for a mean duration of 2 1/2 days and extracorporeal membrane oxygenation for 1 patient for 1 day. All operations were performed with deep hypothermia and circulatory arrest (mean arrest time 34 minutes). Supracardiac connections were repaired by performing a side-to-side anastomosis between the pulmonary venous confluence and the dome of the left atrium through a superior approach between the superior vena cava and the aorta. Coronary sinus connections were repaired by enlarging the atrial septal defect and the coronary sinus communication with the left atrium and closing the atrial defect with a large patch. Infracardiac repairs included elevation and rotation of the heart to the right and an elongated side-to-side anastomosis between the common venous confluence and the left atrium. One patient died 1 week postoperatively of persistent pulmonary hypertension. Another patient, who was supported by extracorporeal membrane oxygenation before the operation, died 3 months after the operation as a consequence of pulmonary lymphangiectasia. All other patients are alive and well with a mean follow-up of 26 months (range 3 to 77 months). One patient required two subsequent reoperations for persistent pulmonary venous obstruction, and another patient had superior vena cava obstruction necessitating reoperation. Operative treatment of total anomalous pulmonary venous connection in infants can be performed with low mortality and an infrequent need for reoperations.