Total anomalous pulmonary venous connection in infancy. Ten years' experience including studies of postoperative ventricular function

The surgical experience with total anomalous pulmonary venous connection (TAPVC) at the Venderbilt Hospital between the years 1969 and 1979 was reviewed. Twenty-five patients with TAPVC were studied. All but one patient presented at less than 1 year of age, and 11 patients were operated upon in the first 2 weeks of life. Operative mortality rate was 20% (5/25). Four of the five deaths were in critically ill neonates requiring preoperative ventilatory support. All operative deaths were in patients with pulmonary venous obstruction. All operative survivors have been followed for a mean 4.5 years (6 months to 10 years). There have been no late deaths or cases of symptomatic pulmonary venous obstruction. All children have had normal growth patterns. Ten patients have been recatheterized. Pulmonary artery systolic pressure, which was markedly elevated prior to operation, fell to normal levels after operation. Ventricular function has been evaluated by quantitative angiocardiography in nine patients before and after operation. Left ventricular size and function were markedly depressed preoperatively and rose to normal levels in postoperative survivors. Left atrial maximal volume was found to be 94% of normal values. These data support the contention that preoperative pulmonary venous obstruction is the single risk factor predicting higher risk of operative death. The presence of severe depression of left ventricular size and function preoperatively does not predict a higher operative risk, and postoperative survivors can expect normal ventricular function.