Total anomalous pulmonary venous connection. Long-term results following repair in infancy

Twenty-three infants, aged 5 days to 10 months, underwent repair of total anomalous pulmonary venous connection (TAPVC). Coronary sinus drainage was unusually common, accounting for 39 percent of the total. There were three early and four late deaths. Although the hospital deaths were related to perioperative errors, two of the infants appeared to have small left ventricles. Two late deaths in infants with coronary sinus drainage were due to obstruction to pulmonary venous return some distance proximal to the surgical anastomosis. All 16 survivors were well at last review, one after revision of his repair. Fifteen have undergone late reassessment, and one of these, also with coronary sinus drainage, showed evidence of pulmonary venous obstruction. Although the early survival rate is satisfactory, the late mortality rate has been significant and the high incidence of pulmonary venous obstruction in infants with coronary sinus drainage is of particular concern.