Coronary artery–pulmonary artery fistula in pulmonary atresia with ventricular septal defect: report of two surgical cases

Abstract

Pulmonary atresia with ventricular septal defect is a complex congenital cardiac anomaly. The blood is supplied to the lungs through a patent ductus arteriosus, a major aortopulmonary collateral artery, or in very rare cases from a coronary artery–pulmonary artery fistula. We present two cases with coronary artery–pulmonary artery fistula which underwent surgical intervention. In our first patient, the main pulmonary artery was supplied from the left main coronary artery. In the second patient, the right pulmonary artery originated from the left main coronary artery and continued to the right lung posteriorly to the aorta, while the left pulmonary artery originated from the patent ductus arteriosus. The difference in our cases is that the coronary artery pulmonary artery fistulas behave like major aortopulmonary collateral arteries originating from the coronary arteries. These fistulas were the main source of pulmonary blood flow.

Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect

“…Of these patients, eight were excluded due to inadequate data [ 4 , 5 ]. Finally, the remaining 69 patients, who were reported in 42 articles, were included in the present study [ 2 , 3 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 ]. Of these articles, 11 involving 21 patients (30%) were published before 1990 [ 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 ].…”