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Repair of total anomalous pulmonary venous connection in infancy: experience from a developing country
S K Choudhary1, A Bhan, R Sharma
1Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Center, All India Institute of Medical Sciences, New Delhi.
Insights
Delayed recognition of total anomalous pulmonary venous connection (TAPVC) in infants leads to severe complications and poor outcomes. Early diagnosis and intervention are crucial for improving survival rates in developing countries.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Disease
- Critical Care Medicine
Background:
- Total anomalous pulmonary venous connection (TAPVC) surgery in infants has high morbidity and mortality in developing nations.
- Factors contributing to adverse outcomes require thorough evaluation.
Purpose of the Study:
- To evaluate factors influencing high morbidity and mortality rates in corrective surgery for TAPVC in infancy.
- To identify risk factors for operative mortality and long-term survival.
Main Methods:
- A retrospective analysis of 73 infants undergoing TAPVC repair from 1987-1997.
- Data collected included patient demographics, type of TAPVC, preoperative conditions, surgical approach, and outcomes.
- Follow-up ranged from 1 to 108 months.
Main Results:
- Operative mortality was 23.3%, with pulmonary hypertensive crisis being the primary cause of death.
- Emergency operations and low weight (<25th percentile) were significant risk factors for mortality.
- Actuarial survival at 9 years was 72.87%.
Conclusions:
- Delayed diagnosis and referral lead to cardiac cachexia, respiratory infections, and pulmonary hypertension, negatively impacting outcomes.
- Early recognition and timely intervention are critical for improving surgical success in TAPVC.
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.
