Repair of Combined Total Anomalous Pulmonary Venous Connection and Anomalous Systemic Connection in Early Infancy

Abstract

A simple intraatrial reconstruction can be utilized in infants to simultaneously correct both the systemic and pulmonary venous returns. Two infants with polysplenia syndrome treated in the first months of life are presented. One had levocardia and combined total anomalous pulmonary venous connection (type II–Cardiac) and anomalous systemic venous connection. The other had dextrocardia l-loop, l-normal great arteries, and combined total anomalous pulmonary venous connection (type II—Cardiac) and anomalous systemic venous connection. The posterior position of the systemic atrioventricular valve and systemic ventricle in both patients allowed the use of this reconstructive technique. The extensive size and transverse position of this patch when used in the infant may require both continued postoperative controlled ventilation until it stiffens and prolonged attention to postoperative cardiac care until adaptation of the infant heart to the new atria can occur. Early restudy and reoperation should be performed if signs of stenosis develop.