Collateral or fistula? Coronary artery as the primary source of pulmonary blood flow in a patient with pulmonary atresia and ventricular septal defect

Abstract

In patients with pulmonary atresia and ventricular septal defect (PA/VSD), a coronary artery being the primary source of pulmonary blood flow is a rare entity. We describe two cases of PA/VSD with coronary-to-pulmonary artery fistula with emphasis on the role of Computed Tomographic Angiography (CTA) in depicting all the sources of pulmonary blood supply, to predict surgical management and need for unifocalization of Major Aortopulmonary Collateral Arteries (MAPCA's).

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

“…2 In the study by Yadav et al with two patients, the pulmonary arteries of both patients originated from the left main coronary artery. 1 In our study, coronary artery-pulmonary artery fistula originated from the left main coronary artery in both of our patients and these fistulas were the main source of pulmonary blood flow. Small coronary pulmonary fistulas between coronaries and pulmonary arteries are common in patients with tetralogy of Fallot and patients with pulmonary atresia with ventricular septal defect.…”

“…These are called coronary artery-pulmonary artery fistulas. 1 We present coronary artery-pulmonary artery fistula in two patients with pulmonary atresia with ventricular septal defect. In the first patient who was operated 10 years ago, the main pulmonary artery was supplied by a fistula from the left main coronary artery.…”

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

“…Figure 2 A shows the age distribution of 51 patients at the time of CPF diagnosis. Twenty patients (39%) were diagnosed with CPF at 0 years of age, whereas five (10%) were diagnosed in their adulthood with symptoms of shortness of breath on exertion, chest pain, edema, and palpitation [ 17 , 27 , 30 , 34 , 40 ]. In addition, most CPF diagnoses under one year of age were made at 0 months of age ( Figure 2 B).…”

“…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 ].…”

Coronary artery to pulmonary artery collateral as the primary source of pulmonary blood supply in tetralogy of Fallot

“…However, it is an invasive procedure, operator‐dependent. With the development signs of progress of CTA scanning technologies, systemic–pulmonary collaterals can be delineated in a noninvasive imaging method with the added advantage of detection of extracardiac anomalies 6 . We report a case of tetralogy of Fallot with one large CAPA as the primary source of pulmonary blood supply, the case highlights the important value of CTA in the accurate preoperative diagnosis and the appropriate surgical strategy.…”