[Operative outcome in children with total anomalous pulmonary venous connection]

The authors present their experience in surgical treatment of children with total anomalous pulmonary venous connection. A retrospective analysis was carried out in 42 patients operated on in the years 1979-1999. The group included 30 boys aged 7 days to 7 years (mean 170 +/- 480 days) and 12 girls aged 14 days to 7 months (mean 86 +/- 80 days). Diagnostic studies allowed to determine that 24 patients (57%) had supracardiac type (type I), nine (21%)--cardiac (type II), five (12%)--infracardiac (type III) and four (10%)--mixed (type IV). The diagnosis was based on clinical examinations and echocardiography, supplemented in 17 patients (40%) by hemodynamic studies and angiocardiography. Preoperative pulmonary hypertension was noted in 34 children (81%), including 15 patients (36%) diagnosed by hemodynamic studies and 19 children (45%) by echocardiography. All children were operated on in deep hypothermia with circulatory arrest. In the early postoperative period (30 days), seven children (17%) died, while four more (9.5%) died in the latter period. The former were characterized by significantly lower birth weight values (p = 0.023). The total mortality rate was higher in children with type II and III anomalies. No statistically significant association was found between preoperative pulmonary hypertension and early mortality. Thirty-one children (74%) remain in late follow-up. All of them are in NYHA (New York Heart Association) class I and develop normally. Total anomalous pulmonary venous connection is a rare congenital anomaly that can be fully repaired with improving early and late results.