Total-Anomalous-Pulmonary-Venous- Connection: Management and Outcome, Experience from Children’s Hospital, Ittefaq Hospital, Lahore, Pakistan

Objective; To determine factors contributing to mortality among patients of TAPVC undergoing surgical repair at Children Hospital, Lahore. Study Design: Descriptive case series. Place and Duration of the Study: September 2004 to October 2009 Materials and Methods: All patients of either gender who had TAPVC and unde went surgical repair during study period were included. Results: In the last 4.5 years, 44 patients (29 males 15 females) underwent repair for total anomalous pulmonary venous connection. Ages ranged from 2 day to 9 years (median 6 mean 17 months) and 26 of them were less than 1 year. Weight ranged from 2.4 to 20 kg (median 5 kg). The anomalous connection was supracardiac in 21 (48%), cardiac in 10 (23%), infracardiac in 5 (11%) and mixed in 8 (18%) patients. Fifteen (34%) patients had obstructed drainage and 21 (48%) had moderate or severe pulmonary arterial hypertension. 15 patients (34%) had to be operated upon on an emergency basis. Mortality was more in obstructed drainage patients (40%),compared with patients without obstruction (6%).The major causes of early death were weight 10%ile (OR 1.1; 95% CI: 0.1-6.5, p-0.009), obstruction (OR 9.8; 95% CI: 1.6-60, p-0.006) and sepsis (OR 23.3; 95% CI: 3 – 177, p-0.002). Follow-up ranged from 1 to 45 months (median 24 months). There was one late death due to late pulmonary vein stenosis and was re-operated upon and died. Conclusions: In a developing country like Pakistan, mortality continues to be high in infants with total anomalous pulmonary venous connection. Weight 10 percentile, obstruction, severe pulmonary arterial hypertension and post operative sepsis appears to be the most important predictor of operative mortality.