Coronary Artery-to-Pulmonary Artery Fistula in a Case of Pulmonary Atresia With Ventricular Septal Defect

Abstract

Pulmonary atresia with ventricular septal defect (PA-VSD) can be associated with varying pulmonary artery connections. The origin of pulmonary blood flow can vary greatly among patients, and some case reports have described the presence of left coronary artery-to-pulmonary artery fistula. Two cases of patients found to have coronary artery-to-pulmonary artery fistula in the settings of PA-VSD are reported.

Coronary‐pulmonary arterial fistula in a neonate with pulmonary atresia—ventricular septal defect and single coronary artery

“…reported 9 cases of 87 patients that had CPAF (incidence 10%), Collision et al . reported 4 cases of 50 patients (8%) although Hofbeck and colleagues reported 1 case of 32 patients (incidence 3%), and Sathanandam et al . reported one case of 76 patients (1.3%).…”

“…Aortopulmonary window and coronary artery‐pulmonary fistula as the primary source of pulmonary blood supply in these cases are extremely rare . The incidence of PA‐VSD with coronary fistula varies from 1.3% to 10% of all cases with PA‐VSD …”

Artery Fistula Causing Aortic Regurgitation in Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

“…Although CAPA fistulas steal coronary artery blood flow, there have been no reported cases of myocardial ischemia [1,2]. Coronary artery perfusion may be independent of the pulmonary bed and dependent only on the distal coronary bed as long as the proximal coronary artery is patent without stenosis and flow limitation [2,3]. However, in our patient, there were a few ischemic findings: the episode of ventricular fibrillation, a negative T wave on the electrocardiogram, and left ventricular noncompaction on echocardiography.…”

“…CAPA fistulas should be considered a source of pulmonary blood flow in patients with PA/VSD [2]. Commonly, CAPA fistulas arise from the left coronary artery [2,3]. In our patient, the fistula arose from the left anterior descending branch.…”

“…In the literature, CAPA fistulas are complicated with PA/VSD about 10% of the time [2][3][4]. CAPA fistulas should be considered a source of pulmonary blood flow in patients with PA/VSD [2].…”