Congenital Coronary Artery–to–Pulmonary Artery Fistula with Anomalous Origin of Right Coronary Artery from Pulmonary Artery: A Case of “Double Trouble”

Abstract

Congenital coronary artery fistula is a rare coronary anomaly. Its clinical significance focuses mainly on the mechanism of coronary steal phenomenon. A combination of left main (LM) coronary artery-to-main pulmonary artery (MPA) fistula with anomalous origin of right coronary artery from the pulmonary artery (ARCAPA) was encountered in a 3-month-old infant who presented with tachypnea. Evaluation with echocardiography and CT confirmed the diagnosis. The patient underwent surgical ligation of LM-to-MPA fistula with direct reimplantation of ARCAPA to aortic root.

Challenging Diagnosis of Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery

“…Williams et al conducted a review of the literature on 70 patients diagnosed with ARCAPA in published case reports and described a possible association with other structural cardiac lesions in more than one-third of the reports [ 3 ]. The most common associated lesions were: aortopulmonary window and tetralogy of Fallot, but additional lesions were encountered: ventricular septal defect, atrial septal defect, double outlet right ventricle, patent ductus arteriosus, coarctation of the aorta, aortic arch hypoplasia, pulmonary stenosis, aortic stenosis, and left main coronary artery-to-pulmonary artery fistula [ 3 , 11 , 12 ].…”

Giant Coronary-Pulmonary Artery Fistula Incidentally Detected in a Patient Presenting With Acute Inferior Myocardial Infarction

“…The co-occurrence of CPAFs and the abnormal origin of the right coronary artery (RCA) from the pulmonary artery (ARCAPA) is referred to as "double trouble." Surgical ligation is recommended in these patients due to coronary steal and myocardial ischemia [ 3 ]. In a study analyzing the imaging features of CPAFs, 3975 patients were examined, and a CPAF prevalence of 0.55% (n = 22) was detected.…”