Repair of total anomalous pulmonary venous connection in patients younger than 6 months old. Late postoperative hemodynamic and electrophysiologic status.

During a 31/2 year period, 11 consecutive infants with total anomalous pulmonary venous connection younger than 6 months of age underwent repair at our institution. Early mortality was 19% (one of 11) and long-term mortality 18% (two of 11), yielding an overall survival of 73%. One late death was due to bradyarrhythmia and the other was due to recurrent pulmonary venous obstruction. Postoperative catheterization in seven of eight survivors and in three additional patients disclosed normal or nearly normal pulmonary artery pressure and pulmonary arteriolar resistance. Left ventricular diastolic volume was normal before operation (n = 5) and on follow-up study (n = 9). Mild left ventricular outflow obstruction or coarctation or both developed during follow-up in two patients. Sinus node function was normal in all seven late survivors studied, and atrioventricular conduction was normal in three of four. This experience indicates that total anomalous pulmonary venous connection can be repaired early in infancy, with preservation of normal hemodynamics and electrophysiologic state.