Repair of total anomalous pulmonary venous connection in early infancy

From May 1995 through October 2001, 19 infants less than 90 days old underwent surgical correction of total anomalous pulmonary venous connection. In 15 babies with isolated total anomalous pulmonary venous connection, there was one operative death. In 4 with complex anomalies, there were 2 operative deaths. The vertical vein was not ligated in 6 cases for various reasons. Two patients died during reoperation for early pulmonary venous obstruction. In the late follow-up, 2 babies required reoperation for late anastomotic stricture; one needed additional balloon dilatation. Of the 14 surviving patients, one had a small residual gradient and infrequent supraventricular tachycardia, the others were asymptomatic and without gradients. Surgical correction of total anomalous pulmonary venous connection can be carried out in early infancy with low mortality and morbidity. However, associated complex cardiac anomalies and small caliber pulmonary arteries and veins carry higher risks. Recurrent pulmonary venous obstruction and diffuse pulmonary vein stenosis are causes of early reoperation and poor surgical outcome.