Coronary artery fistula as source of pulmonary circulation in pulmonary atresia with ventricular septal defect.

Four patients are described with pulmonary atresia and ventricular septal defect, in whom the pulmonary circulation was dependent on a fistula from the left coronary artery to the pulmonary artery. The issue in this complex anomaly is complete preoperative diagnosis, including anatomic information on the coronary artery fistula and the pulmonary vasculature. This was achieved in the last 2 patients. In the last patient echocardiography turned out to be an important diagnostic tool in this rare anomaly and facilitated selective angiocardiography. All 4 patients were successfully operated by closing the fistula, closing other aortopulmonary connections and inserting a valved conduit between right ventricle and pulmonary artery. The ventricular septal defect was closed in 3 patients with a patch. In the setting of an already existing pulmonary hypertension and a possibly inadequate pulmonary arterial system at surgery, a perforated patch was inserted in the ventricular septal defect of the remaining patient.