Surgical palliation of cardiac malformations associated with right isomerism.

Between 1985 and 1993, palliative surgery was performed on 13 pediatric patients who had complex cardiovascular anomalies associated with right isomerism. The patients included two neonates, ten infants, and one child who were divided into two groups according to whether or not a total anomalous pulmonary venous connection (TAPVC) was present. Group 1 consisted of six patients with TAPVC and group 2 consisted of seven patients without TAPVC. In group 1, the surgical procedures involved TAPVC repair alone in two patients, combined TAPVC repair with a modified Blalock-Taussig shunt in two, combined TAPVC repair with pulmonary artery banding in one, and a modified Blalock-Taussig shunt alone in one. There were five hospital deaths and one late death in this group: pulmonary venous obstruction in two patients, perioperative myocardial failure in the two neonates, and congestive heart failure caused by increased pulmonary blood flow in two patients. In group 2, all the patients underwent systemic-pulmonary artery shunts, and there was one hospital death and three late deaths, the causes of which were unknown in two patients, and shunt failure and pneumonia in one patient each. These results suggest that surgical palliation for right isomerism produces poor results in young infants with obstructed TAPVC. Thus, we conclude that TAPVC repair should be performed without delay if pulmonary venous obstruction has been diagnosed clinically. Resolving pulmonary venous obstruction without cardiopulmonary bypass (CPB) may be preferable for infants, considering their difficult management. The systemic-pulmonary artery shunt should be of the low-calibrated type, especially if common atrioventricular valve regurgitation exists. If infants survive the surgery, they must be carefully followed up for a long period due to the risk of sudden death or infection.