Coronary artery-pulmonary artery fistula in pulmonary atresia with ventricular septal defect

Summary This paper reports two patients presenting a combination of coronary artery-pulmonary artery fistula and pulmonary atresia with ventricular septal defect. A confluent central pulmonary artery arose from the proximal portion of the left coronary artery in both patients. The fistulous segment between the coronary artery and the central pulmonary artery was large, and no coronary arterial stenosis was found in either patient. Besides this fistulous origin of the pulmonary blood supply, large collateral vessels originated from the descending aorta in both patients. Neither patient has shown findings of myocardial ischemia, at least in consecutive rest and exercise electrocardiograms. Both patients were successfully operated, at the ages of 3 and 8 years, respectively. The clinical and embryological implications of this rare malformation are briefly reviewed.