The value of early repair for total anomalous pulmonary venous drainage

A total of 20 children with total anomalous pulmonary venous drainage (TAPVD) underwent complete repair within the past six years. The drainage was supracardiac in 11, infracardiac in seven, and into the coronary sinus in two. At repair mean age was nine weeks, and weight was 3.7 km. Operative technique in extracardiac types included complete mobilization of the common vein with division of anomalous channel when possible. The incision in the confluent vein was extended into the lobar veins when necessary to permit extensive incorporation of this structure into the posterior wall of the left atrium and resulted in a nonobstructing stellate-type anastomosis. Operative mortality was 10% (2 of 20). Deaths occurred only in the group of infants in whom severe obstruction to pulmonary venous return required emergency operation, and one of these patients has persistent neurologic deficit. Late cardiac catheterization has been performed in 11 of 18 survivors. Nine had no or only minor abnormalities and two required reoperation. There have been no late deaths with follow-up of 2-8 years (mean = 4 years). Currently, all of the survivors are without cardiac symptoms and only one requires cardiac medication. Our experience identifies pulmonary venous obstruction with critical symptoms as the major operative risk factor in patients with TAPVD. With early operation prior to onset of critical symptoms, mortality is low and functional results are excellent.