Surgical treatment of total anomalous pulmonary venous connection

Fourteen infants ranging in age from 18 days to 10 months (median age = 4 months) underwent surgical repair of total anomalous pulmonary venous connection (TAPVC) between September, 1988 and December, 1989. The anomalous drainage was supracardiac in nine, cardiac in two, infracardiac in one and mixed in one. One patient had a complex type of TAPVC. There were two hospital deaths, one a critically ill three week old infant with obstructed infracardiac TAPVC and the other with complex TAPVC. There was no late death. All twelve survivors are in NYHA functional Class 1. Postoperative echocardiography revealed unobstructed pulmonary blood flow in all of them. Surgical correction of TAPVC in infancy has been performed with gratifying results. A high index of suspicion, early diagnosis, prompt referral and an aggressive surgical approach are essential for success in this otherwise lethal condition.