Total anomalous pulmonary venous drainage

Forty-four patients with total anomalous pulmonary venous drainage underwent repair between 1979 and 1987. The anomalous drainage was supracardiac in 16, cardiac in 12, and infracardiac in 16. Median age at repair was 15 days and median weight, 3.3 kg. In 22 patients (50%) the venous drainage was obstructed. Emergency operation was necessary in 12 patients, and the condition of seven additional patients deteriorated while they were awaiting semiurgent operation. There was one hospital death (mortality rate 2.3%), occurring in a 2-week-old premature neonate with infracardiac drainage. In 17 of 44 patients, mean pulmonary artery pressure equaled or exceeded systemic arterial pressure immediately after repair. Four patients have required reoperation and two of these have died as a result of peripheral extension of pulmonary vein sclerosis. In the review period of from 1 month to 7 years, all survivors are well and receiving no medication.