Repair of total anomalous pulmonary venous connection in infancy: experience from a developing country

Background. Corrective surgery for total anomalous pulmonary venous connection in infancy still carries high morbidity and mortality rates in developing countries. The present study evaluates the factors responsible for it. Methods. Seventy-three infants were operated on for total anomalous pulmonary venous connection from January 1987 through October 1997. Age ranged from 5 days to 12 months (mean, 3.9 ± 0.24 months), with 10 (13.7%) patients younger than 1 month old. Patient weight varied from 2.0 to 5.2 kg (mean, 3.7 ± 0.27 kg). Most (90.5%) patients were small for their ages (< 50th percentile). Anomalous connection was supracardiac in 42 (57.5%), cardiac in 18 (24.7%), infracardiac in 4 (5.5%), and mixed in 9 (12.3%) patients. Thirty-five patients had obstructed drainage. Preoperatively, 30 patients received antibiotic therapy for respiratory tract infection, 3 patients had balloon atrial septostomy, and 4 patients required mechanical ventilation. Fifteen patients (20.5%) were operated on as an emergency procedure. For supracardiac and infracardiac connections, a posterior approach was used for anastomosis. In cardiac type, coronary sinus was unroofed and the resultant defect along with atrial septal defect was closed with a single patch. Results. The operative mortality rate was 23.3% (17 of 73). Pulmonary hypertensive crisis was the cause of death in 10 patients. Emergency operation and weight less than the 25th percentile were the important risk factors for operative mortality. Young age (< 1 month) and type of drainage did not affect the mortality. Follow-up ranged from 1 to 108 months (mean, 56.4 ± 26.0 months). There were two late deaths. The actuarial survival (Kaplan Meier) at 9 years was 72.87% ± 5.39%. Conclusion. Failure of early recognition, and thus delayed referral, accounted for onset of cardiac cachexia, respiratory tract infection, and severe pulmonary hypertension, which had a major effect on unfavorable outcome.