Mohammad Asim Khan, Tariq Waqar.
Total-Anomalous-Pulmonary-Venous-Connection Management and Outcome, Experience from Children’s Hospital, & Ittefaq Hospital, Lahore, Pakistan.
Ann Pak Inst Med Sci Jan ;8(3):196-9.

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.

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