Repair of subdiaphragmatic total anomalous pulmonary venous connection without cardiopulmonary bypass

Abstract

Two neonates with asplenia syndrome, subdiaphragmatic total anomalous pulmonary venous connection, and pulmonary stenosis underwent a palliative operation without cardiopulmonary bypass. With the use of a side-biting clamp, and anastomosis was created between the pulmonary venous confluence and the right atrium. (Since children with asplenia syndrome have common mixing lesions, pulmonary venous drainage to the right atrium is not physiologically detrimental.) The descending vertical vein was ligated. A systemic-pulmonary shunt was performed. Both infants were discharged from the hospital less than 1 week after the operation. Both infants subsequently died, at 4 months and at 16 months of age. At autopsy, pulmonary venous drainage was unobstructed, with a surgical orifice 86% to 90% of the aortic anulus. We conclude that, in infants with asplenia syndrome and obstructed total anomalous pulmonary venous drainage, relief of pulmonary venous obstruction can be accomplished without cardiopulmonary bypass.