Cardiac imaging findings in anomalous origin of the coronary arteries from the pulmonary artery; narrative review of the literature

Abstract

Introduction Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare coronary artery malformation with an incidence of 0.002% in patients undergoing coronary angiography. It can lead to an increased risk of myocardial infarction (MI) and sudden cardiac death, even in asymptomatic patients. Methods We conducted a review of published cases of ARCAPA using PubMed and Scopus databases and included patients over 18 years old with adequate echocardiographic data. Results We evaluated 28 patients with ARCAPA with a mean age of 42.8 from 1979 to 2021. Patients were diagnosed mostly by angiography and echocardiography, the most performed treatment was reimplantation (15, 53.6%) and the main echocardiographic findings were dilated coronary arteries (9, 32.1%), coronary collaterals (8, 28.6%), and retrograde flow from right coronary arteries to main pulmonary trunk (7, 25%). Conclusion Although ARCAPA is rare and not as deadly as the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) still there is a chance of serious outcomes, therefore surgical treatment should be performed upon diagnosis. Angiography is the gold standard for diagnosis, but echocardiography can be a convenient, non-invasive, and most reliable method as the primary step whenever ARCAPA is suspected.

A Multimodality Imaging and Multidisciplinary Approach to Manage Anomalous Right Coronary Artery from the Pulmonary Artery in Pregnancy

“… 2 Even though it frequently presents as an isolated lesion, 40% of patients with ARCAPA have other congenital cardiac defects, including aortopulmonary window (most common), atrial septal defects, ventricular septal defects, bicuspid aortic valve, and tetralogy of Fallot. 3 While anomalous origin of the left coronary artery from the PA (ALCAPA) tends to exhibit a more severe clinical presentation characterized by malignant arrhythmias, systolic dysfunction, myocardial infarction, and/or sudden cardiac death, ARCAPA is frequently diagnosed incidentally likely because of the lower right ventricular oxygen demands and the relatively lower contribution of the RCA to left ventricular blood supply as compared to the left coronary system. 4 However, patients with a dominant RCA system do not tolerate the ARCAPA circulation when compared to those with a left dominant circulation.…”

Anomalous arising of left coronary artery from the pulmonary artery (ALCAPA)

“…The arising of the left common coronary artery (LCCA) from the PA (ALCAPA), known as Bland‐White‐Garland syndrome, was first described in 1933 1 . This represent 0.24%–0.46% of all congenital heart malformations, which represents an incidence of one in 300 000 2,3 . ALCAPA is a life‐threatening congenital heart defect.…”

“…In younger patients, collateral arterial flow in the ventricular septum can lead to misdiagnosis of multiple muscular ventricular septal defects (VSD). For differential diagnosis, ALCAPA has continuous flow in collateral arteries, whereas in VSD cases the major finding is systolic color flow 2 . Coronary CT angiography has excellent special and temporal resolution, which can show the origin and course of the coronary artery 1,3 .…”

“…1 This represent 0.24%-0.46% of all congenital heart malformations, which represents an incidence of one in 300 000. 2,3 ALCAPA is a life-threatening congenital heart defect.…”

“…For differential diagnosis, ALCAPA has continuous flow in collateral arteries, whereas in VSD cases the major finding is systolic color flow. 2 Coronary CT angiography has excellent special and temporal resolution, which can show the origin and course of the coronary artery. 1,3 Preoperative CT is crucial for diagnostics of extremely rare congenital coincidental heart anomalies.…”