Infant total anomalous pulmonary venous connection: factors influencing timing of presentation and operative outcome.

The surgical experience in 75 patients with total anomalous pulmonary venous connection (TAPVC) between 1975 and 1986 was reviewed. Most of these patients underwent operation at less than 1 month of age (39 of 75, 52%). Operative approaches used were the standardized left-sided approach for supracardiac and infracardiac TAPVC, reserving transatrial repair for only the intracardiac lesions. Cardiopulmonary bypass was managed by deep hypothermia and circulatory arrest in 16 of 75 (29%) and continuous hypothermic bypass with low flow was used for the remaining 59 of 75 (71%). Operative policy included maintaining patency of existing patent ductus (1 patient) and atrial (4 patients) or ventricular (3 patients) defects to allow additional shunting in cases of severe obstruction. Operative mortality was 9.3% (7 of 75) and late mortality was 6.7% (5 of 75). The determinants of operative deaths were (1) the type of TAPVC (highest rates in types III and IV with 3 of 17 or 19%, and 2 of 8 or 25%, respectively) and (2) associated severe preoperative pulmonary-to-systemic venous obstruction. Determinants of late death were (1) residual pulmonary-to-systemic obstruction (two patients type III and 1 patient type I), and (2) associated intracardiac lesions (two patients type IV with atrioventricular canal). In summary, the timing of corrective surgery and outcome are influenced by the clinical condition of the infant as a direct reflection of the anatomic type and severity of associated lesions.