SURGICAL PALLIATION OF CARDIAC-MALFORMATIONS ASSOCIATED WITH RIGHT ISOMERISM

Between 1985 and 1993, palliative surgery was performed on 13 pediatri c patients who had complex cardiovascular anomalies associated with ri ght isomerism. The patients included two neonates, ten infants, and on e child who were divided into two groups according to whether or not a total anomalous pulmonary venous connection (TAPVC) was present. Grou p 1 consisted of six patients with TAPVC acid group 2 consisted of sev en patients without TAPVC. In group 1, the surgical procedures involve d TAPVC repair alone in two patients, combined TAPVC repair with a mod ified Blalock-Taussig shunt in two, combined TAPVC repair with pulmona ry artery banding in one, and a modified Blalock-Taussig shunt alone i n one. There were five hospital deaths and one late death in this grou p: pulmonary venous obstruction in two patients, perioperative myocard ial failure in the two neonates, and congestive heart failure caused b y 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 unknow n in two patients, and shunt failure and pneumonia in one patient each . These results suggest that surgical palliation for right isomerism-p roduces poor results in young infants with obstructed TAPVC. Thus, we conclude that TAPVC repair should be performed without delay if pulmon ary venous obstruction has been diagnosed clinically. Resolving pulmon ary venous obstruction without cardiopulmonary bypass (CPB) may be pre ferable for infants, considering their difficult management. Tile syst emic-pulmonary artery shunt should be of the low-calibrated type, espe cially if common atrioventricular valve regurgitation exists. If infan ts survive the surgery, they must be carefully followed up for a long period due to the risk of sudden death or infection.