Background: We evaluated the surgical options of repair in patients with total anomalous pulmonary venous connection (TAPVC).

Methods: The study included 61 children (27 girls, 34 boys; mean age 10.7±17.9 months; range 6 days to 9 years) who underwent repair of TAPVC at our institution. The localization of the TAPVC was supracardiac in 27 patients (44.2%), cardiac in 24 patients (39.3%), infracardiac in four patients (6.5%), and mixed in six patients (9.8%). The mean followup period was 38.8±28.1 months.

Results: The overall hospital mortality was 22% (n=14). Mortality rate for the last 19 patients was 10.5% (n=2). The highest incidence of mortality was seen in patients having the mixed type (33.3%) of TAPVC, followed by infracardiac (25%), cardiac (25%), and supracardiac (18.5%) types. Pulmonary venous obstruction was observed in 28 patients preoperatively. Pulmonary hypertensive crisis was the main reason for mortality. The surviving patients who were followed-up by regular echocardiographic examinations had a mean functional capacity of New York Heart Association (NYHA) class I.

Conclusion: In parallel with enhanced experience with surgical and intensive care management and improved diagnostic methods, the success rate of corrective surgical procedures performed for TAPVC has considerably increased resulting in less mortality.

Keywords : Cardiovascular surgical procedures/methods; child; heart defects, congenital/surgery/mortality; pulmonary veins/abnormalities/ surgery; pulmonary veno-occlusive disease; survival rate