Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery: A Systematic Review

Abstract

Background: Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital cardiac lesion that has been diagnosed in both children and adults with symptoms ranging from an asymptomatic murmur to sudden cardiac death. The aim of this review was to characterize published cases of ARCAPA to better understand this rare congenital coronary anomaly.

Methods: A systematic review was performed using PubMed, Embase, and Google Scholar for cases of ARCAPA. Keywords searched included: "anomalous origin of the right coronary artery from the pulmonary artery" and "ARCAPA."

Results: A total of 223 cases of ARCAPA were identified in 193 case reports. There was a slight male predominance (54.5%) and the median age at presentation was 14.0 years. Thirty-eight percent of patients were asymptomatic and most commonly identified during evaluation of a murmur. Angina and dyspnea were the most common presenting symptoms (22.4% and 17.0%, respectively). In symptomatic patients, a bimodal distribution of age at presentation was observed with a peak near birth and another between ages 40 and 60 years. The condition was most commonly diagnosed with coronary angiography (40.4%). Most cases were repaired surgically (72.6%) and reimplantation of the right coronary artery onto the aorta was the most common method of repair (62.3%).

Conclusions: ARCAPA represents a rare coronary anomaly with great variability in clinical presentation. An understanding of the pathophysiology associated with the lesion is critical when determining treatment strategies.