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.