From 1973 through December 1980, 41 children with total anomalous pulmonary venous connection (TAPVC) were seen in our hospital, and 35 underwent surgery. The supracardiac type of TAPVC was found in 19 cases (46%), the intracardiac type in 6 infants (15%), the infracardiac type in 15 cases (37%), and the mixed type in one child (2%). Clinical and cardiac catheterization data demonstrated that children with the infracardiac type of TAPVC were referred very early in life and had the highest pulmonary artery pressures. Operation was performed in 35 cases, 15 of supracardiac, 6 of cardiac, 13 of infracardiac, and 1 of mixed type of TAPVC. Total in-hospital mortality was 39%, and was 28% in the operated children. The initial operative mortality of 71% in the years 1973 to 1975 has decreased to 18% in the last 5 years. Twelve of the surviving infants underwent re-catheterization showing excellent results. Only in one case, with additional aplasia of the left lung, did half-systemic pulmonary artery pressure persist. Corrective surgery offers the only chance of survival in most children with TAPVC. The operative risk can be reduced by increased surgical experience supported by optimal medical preparation of the patient. After adequate surgical correction long-term results appear to be excellent.
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