BACKGROUND: We report a retrospective analysis of the demographic. morphological and clinical profiles of patients along with results of operative repair for total anomalous pulmonary venous connection. METHODS AND RESULTS: In the last 15 years, 248 patients (168 boys, 80 girls) underwent repair for total anomalous pulmonary venous connection. Their ages ranged from I day to 24 years (median 8 months) and 145 of them were < or = 1 year of age. The patients' weight ranged from 2 to 52 kg (median 5 kg). About 70% of patients (n = 174) were less than the 50th percentile of predicted weight for age and sex. The anomalous connection was supracardiac in 134 (54%), cardiac in 80 (32.2%), infracardiac in 9 (3.6%) and mixed in 25 (10.1%) patients. Fifty (20.2%) patients had obstructed drainage and 76 patients (30.2%) had moderate or severe pulmonary arterial hypertension. Forty-five patients (18.1%) had to be operated upon on an emergency basis. All the patients were operated upon using moderately hypothermic cardiopulmonary bypass. In 114 patients, circulatory arrest was used. There were 45 (19.1%) in-hospital deaths. The major causes of early death were pulmonary arterial hypertensive crisis in 19 (7.7%) and low cardiac output syndrome in 17 (6.9%) patients. Age < or = 1 year (odds ratio 2.16; 95% confidence interval: 1.22-3.82, p=0.008), severe pulmonary arterial hypertension (odds ratio 5.86; 95% confidence interval: 2-17, p=0.001), and need for emergency surgery (odds ratio 3.65; 95% confidence interval: 1.59-8.38, p=0.002) were independent risk factors for early death. Follow-up ranged from 1 to 180 months (median 48 months). There were 4 lake deaths. Actuarial survival at 12 years was 92.6% +/- 2.8%. CONCLUSIONS: In Indian circumstances, mortality continues to be high in infants with total anomalous pulmonary venous connection. Severe pulmonary arterial hypertension appears to be the most important predictor of operative mortality. Severe malnutrition, delayed diagnosis and late referrals possibly contribute to the high mortality.
BACKGROUND: We report a retrospective analysis of the demographic. morphological and clinical profiles of patients along with results of operative repair for total anomalous pulmonary venous connection. METHODS AND RESULTS: In the last 15 years, 248 patients (168 boys, 80 girls) underwent repair for total anomalous pulmonary venous connection. Their ages ranged from I day to 24 years (median 8 months) and 145 of them were < or = 1 year of age. The patients' weight ranged from 2 to 52 kg (median 5 kg). About 70% of patients (n = 174) were less than the 50th percentile of predicted weight for age and sex. The anomalous connection was supracardiac in 134 (54%), cardiac in 80 (32.2%), infracardiac in 9 (3.6%) and mixed in 25 (10.1%) patients. Fifty (20.2%) patients had obstructed drainage and 76 patients (30.2%) had moderate or severe pulmonary arterial hypertension. Forty-five patients (18.1%) had to be operated upon on an emergency basis. All the patients were operated upon using moderately hypothermic cardiopulmonary bypass. In 114 patients, circulatory arrest was used. There were 45 (19.1%) in-hospital deaths. The major causes of early death were pulmonary arterial hypertensive crisis in 19 (7.7%) and low cardiac output syndrome in 17 (6.9%) patients. Age < or = 1 year (odds ratio 2.16; 95% confidence interval: 1.22-3.82, p=0.008), severe pulmonary arterial hypertension (odds ratio 5.86; 95% confidence interval: 2-17, p=0.001), and need for emergency surgery (odds ratio 3.65; 95% confidence interval: 1.59-8.38, p=0.002) were independent risk factors for early death. Follow-up ranged from 1 to 180 months (median 48 months). There were 4 lake deaths. Actuarial survival at 12 years was 92.6% +/- 2.8%. CONCLUSIONS: In Indian circumstances, mortality continues to be high in infants with total anomalous pulmonary venous connection. Severe pulmonary arterial hypertension appears to be the most important predictor of operative mortality. Severe malnutrition, delayed diagnosis and late referrals possibly contribute to the high mortality.