Infradiaphragmatic total anomalous pulmonary venous drainage: surgical treatment and long-term results

Since 1971, 20 infants (1 day to 3 months old) with infradiaphragmatic total anomalous pulmonary venous drainage (TAPVD) have undergone repair at The Hospital for Sick Children, Great Ormond Street, London. All patients were seen with severe cyanosis (mean partial pressure of arterial oxygen, 35.8 mm Hg) and pulmonary edema. All had a persistent ductus arteriosus. Right-sided pressures were equal to or greater than systemic in 17 patients. During surgical repair, the confluence of the pulmonary veins was anastomosed to the left atrium. The descending vein was ligated in 11, divided in 4, and left open in 5 patients. Atrial septal defects were closed with a patch in 6 and sutured in 14. Eight patients died (40%). Acidosis on admission was significantly greater among nonsurvivors (mean pH, 7.29 +/- 0.02) than survivors (mean ph, 7.37 +/- 0.02) (p less than 0.05). Twelve survivors are well four months to 8 years after operation. All are asymptomatic and none requires cardiac medication. Six have undergone repeat catheterization, demonstrating normal pressures and no shunts. We conclude that the mortality of patients with infradiaphragmatic TAPVD depends mainly on the condition of the child on admission.