Clinical and Prognostic Significance of Anomalous Origin of a Coronary Artery in Adults

Abstract

Background: The clinical significance and outcome predictors of anomalous aortic origin of a coronary artery (AAOCA) in adults remains unclear. Therefore, the aim of this study was to analyze the clinical and prognostic implications of AAOCA in a large cohort of patients undergoing coronary computed tomography angiography (CCTA) in an Italian tertiary referral center.

Methods: Consecutive adults with AAOCA identified through CCTA from September 2004 to September 2024 were included. Data on clinical indications of CCTA, AAOCA subtypes, evidence of inducible myocardial ischemia, and concomitant coronary atherosclerotic disease were collected. Patients were followed up for the end points of all-cause mortality and major adverse cardiac events (nonfatal acute coronary syndromes, revascularization procedures, and heart failure hospitalization). Outcomes were compared with matched controls with normal coronary artery anatomy.

Results: Among 17 454 CCTAs performed over a span of 20 years, AAOCA was detected in 173 patients (62±15 years of age, n=58 women [34%]). Chest pain (34%) was the most common indication of CCTA. Obstructive coronary atherosclerotic disease was present in 36 patients (21%), and myocardial ischemia was detected in 60% of those (n=62) who underwent functional imaging testing. AAOCA repair was performed in 9 patients (5%), whereas the majority of patients were treated conservatively. After a median 37-month follow-up (17 to 69 months), mortality (P=0.321) and major adverse cardiac events (P=0.392) were similar between patients with AAOCA and controls. Only obstructive coronary atherosclerotic disease was associated with a higher event rate during follow-up, whereas AAOCA subtype and ischemia were not.

Conclusions: In adults with AAOCA, concomitant obstructive coronary atherosclerotic disease is the primary determinant of adverse events. Given the low prevalence of AAOCA and event rates, multicenter registries are needed to refine risk stratification and management of these patients.

Keywords: congenital heart disease; coronary anomalies; coronary atherosclerotic disease; myocardial ischemia; sudden cardiac death.