Total Anomalous Pulmonary Venous Return

Abstract

Total anomalous pulmonary venous return is reported to constitute about 2% of all congenital cardiac malformations. At The Children's Hospital, Columbus, the incidence from 1952 to date is 4.5% of all autopsied congenital heart cases. The basic anomaly consists of the direct or indirect return of all the pulmonary venous blood to the right atrium instead of the left. Survival is possible because a patent foramen ovale or ductus arteriosus is always associated with the anomaly. Cases of total anomalous pulmonary venous return are presently classified on the basis of the specific point of insertion of the pulmonary veins into the systemic venous circuit, thus resulting in a multiplicity of separate entities. From the clinical standpoint, however, all of these entities may be consolidated into two well-defined and strikingly different types—supradiaphragmatic and infradiaphragmatic, depending upon the general level of pulmonary vein insertion. The clinical manifestations, x-ray appearance, and length of