BACKGROUND: Among all correctable congenital heart disease, total anomalous pulmonary venous connection (TAPVC) is known for its high rates of mortality and morbidity. METHODS: During the period of 1996 through 2008, twenty-four patients of TAPVC without heterotaxy syndrome received total correction surgery in our hospital. Their charts were reviewed. RESULTS: Twelve were male and 12 were female. Supracardiac, cardiac and infracardiac types of TAPVR appeared in 6, 12 and 6 patients. Their age of diagnosis ranged from one-day-old to 20-year-old (median: 7-days-old). Fourteen patients (58%) received urgent operation soon after birth, most were infracardiac or supracardiac type. Pulmonary hypertension occurred in 13 patients after operation and were managed with inhale NO. Early death (< 30 days) occurred in 2 patients (8%). Six patients (25%) developed postoperative progressive pulmonary vein stenosis (PPVS), including 3 infracardiac, 1 supracardiac, 2 cardiac type (directly to RA). Five of them were found post-operatively less than 3 months. Two of the 6 patients had anastomic stenosis, 1 ostial stenosis, and the other 3 had both anastomic and ostial stenosis. Reoperation trying to relieve PPVS by pericardial patch augmentation were performed on 2 patients and 1 died. Overall, 2 patients (33%) expired lately due to PPVS. One patient who had unilateral PPVS survived.
CONCLUSION: Urgent operation to rescue neonates with obstructed TAPVC is associated with high rates of mortality and complications, especially PPVS.