Management of Total Anomalous Pulmonary Venous Drainage in Early Infancy

Abstract
Ten consecutive babies ranging in age from two weeks to five months, with total anomalous pulmonary venous drainage and pulmonary artery hypertension, have undergone surgical correction. Seven have survived, including five of six with supracardiac type and two of three with pulmonary venous drainage to the coronary sinus. Six of the last seven operations have been successful, and there have been no late deaths in the series. Factors considered to be important in successful management include: (1) early cardiac catheterization and surgical intervention; (2) high-flow buffered blood perfusion at normothermia; (3) surgical technique aimed at wider common pulmonary vein-left atrial anastomosis; (4) use of indwelling nasotracheal tube with respiratory support for the initial 24 to 48 hours postoperatively; (5) restricted fluid administration in the early postsurgical period; and (6) detailed attention to acid-base balance.