Total anomalous pulmonary venous connection. Long-term results following repair under 3 months of age.

The surgical experience with total anomalous pulmonary venous connection (TAPVC) at the University of Louvain (Brussels) between the years 1975 and 1986 is reviewed. Nineteen patients aged two days to three months with TAPVC were studied. The types of TAPVC were supracardiac in 9 patients, cardiac in 4, infracardiac in 4 and mixed in 2. Profound hypothermia induced by surface cooling, limited cardiopulmonary by-pass and total circulatory arrest were used in all cases. The 4 early deaths concerned the first four neonates who were critically ill. All operative survivors are followed for a mean of 3.5 years (12 months to 8 years). There are two late deaths due to reoperation for pulmonary venous obstruction. All 13 survivors are well at last review. Eleven of them have been recatheterized 4 to 33 months after repair (19 months in average). The pulmonary artery and capillary pressures fell to a normal level after a few months. Ventricular function which was markedly depressed preoperatively, was evaluated by quantitative angiocardiography and echocardiography. It returned to normal late postoperatively. The hospital mortality for the repair of TAPVC in the neonates remains appreciable. Total correction at one operation is advisable. The incidence of postoperative pulmonary venous obstruction is of particular concern. The late postoperative functional and hemodynamic results are excellent. The repair of TAPVC can be considered curative.