CORONARY ARTERY-PULMONARY ARTERY FISTULA IN PULMONARY ATRESIA WITH VENTRICULAR SEPTAL-DEFECT

This paper reports two patients presenting a combination of coronary a rtery-pulmonary artery fistula and pulmonary atresia with ventricular septal defect. A confluent central pulmonary artery arose from the pro ximal portion of the left coronary artery in both patients. The fistul ous segment between the coronary artery and the central pulmonary arte ry was large, and no coronary arterial stenosis was found in either pa tient. Besides this fistulous origin of the pulmonary blood supply, la rge collateral vessels originated from the descending aorta in both pa tients. Neither patient has shown findings of myocardial ischemia, at least in consecutive rest and exercise electrocardiograms. Both patien ts were successfully operated, at the ages of 3 and 8 years, respectiv ely. The clinical and embryological implications of this rare malforma tion are briefly reviewed.