PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM - MANAGEMENT OF THE CORONARY ARTERIAL ANOMALIES

Over a period of 12 years, we encountered 30 cases of pulmonary atresi a with intact ventricular septum. The overall mortality was 53% (16 pa tients). Coronary arterial abnormalities were detected in 16 patients and, in 10 of these, the coronary arterial circulation was partially o r totally dependent on the right ventricle. The mortality in this grou p was 80%. The strategies for management are reviewed in 6 cases to tr y to define the best approach to diagnosis, and to assess the benefits of the various modes of treatment used for specific coronary arterial anomalies. Two cases (2 and 3) underwent occlusion of a fistula from the right ventricle to a coronary artery using embolization coils. Thi s modality is considered to be appropriate and useful when the coronar y circulation supplied by the fistula is not dependent on flow from th e right ventricle. The dependency of the coronary arterial circulation on the right ventricle was extensive in two cases (Case 4 and 6), and complete in one because of atresia of the orifices of the coronary ar teries (Case 5). These three patients died. Cardiac transplantation is suggested as the most appropriate treatment in this group. In two cas es (1 and 6), there was a supernumerary vessel connecting the right ve ntricle to the pulmonary trunk. These connections do not appear to be of therapeutic concern, as they do not seem to contribute to myocardia l blood supply. The selection of the most appropriate protocol for man agement of patients with coronary arterial anomalies depends upon a th orough and aggressive approach to investigation.