Complete repair of total anomalous pulmonary venous connection in infancy

From 1983 to 1990, 20 infants underwent complete repair of isolated total anomalous pulmonary venous connection. Twelve were male; ages ranged from 1 day to 240 days (mean 32 days). The abnormal anatomic connection was supracardiac in nine, cardiac in four, mixed in five, and infradiaphragmatic in two. In seven patients the pulmonary venous drainage was obstructed. Corrective operations were performed 2.4 days after admission; after medical stabilization, seven patients underwent emergency operations, including extracorporeal membrane oxygenation in one. All operations were performed with the use of circulatory arrest (mean 33 minutes) and cardiopulmonary bypass (mean 54 minutes). There was one hospital death, which occurred in a premature infant (1.5 kg) who had supracardiac drainage. No preoperative risk factors correlated with a poor surgical outcome. Pulmonary hypertensive episodes were expectantly managed after the operation with 100% oxygen, sedation, and hyperventilation. Postoperative arrhythmias occurred predominantly in patients with intracardiac drainage. All survivors (mean follow-up of 42 months) are in sinus rhythm, receiving no medications, and are growing and developing normally. Surgical correction of total anomalous pulmonary venous connection in infancy can be performed at low risk with good results after aggressive preoperative stabilization and postoperative management.