Total anomalous pulmonary venous connection (surgical technique, early and late results)

Between March 1970 and October 1977, 36 patients underwent correction of total anomalous pulmonary venous connection. The ages ranged from 5 days to 16 years; 27 (75%) were under 1 year and 19 were under 3 months of age at the time of surgery. The overall mortality was 33%. Supracardiac connection was the commonest type and was associated with the lowest hospital mortality (30%). The highest mortality occurred in the mixed and infracardiac types and was related in part to the presence of associated intracardiac anomalies. The use of hypothermia and circulatory arrest in infancy has resulted in a considerably lower hospital mortality compared with cases operated on under conventional cardiopulmonary bypass. The mortality in 23 infants (under 1 year of age) was 26% using circulatory arrest and was lowest when correction was performed within the first three months of life (18%). All four infants operated on with standard cardiopulmonary bypass died, whereas this technique was found to be safe in older children. The surgical technique using a left anterolateral thoractomy with a trans-sternal extension is described. This technique gives an excellent exposure for fashioning a long anastomosis and has been associated with a low incidence of postoperative pulmonary complications. There have been no late deaths and all survivors, who are in excellent condition up to seven years after correction, have a normal exercise tolerance.