Cardiovascular outcomes of adults diagnosed with anomalous aortic origin of the coronary arteries

Background/Introduction

Anomalous Aortic origins of coronary arteries (AAOCAs) occur infrequently in the adult population, with prevalence estimated at 0.3-2.3%(1). They have been specifically implicated in the occurrence of sudden cardiac death in cardiac arrest survivors and post-mortem studies(2,3). The clinical significance and burden of risk imposed on individuals by the presence of AAOCAs remain uncertain. Thus, we analysed the cardiovascular outcomes of adult patients diagnosed with AAOCAs at our institution.

Purpose

The 2020 ESC guidelines for the Management of Adult Congenital Heart Disease recommend surgical intervention for high-risk variants with risk defined by a combination of clinical, anatomical and functional frameworks. There is yet to be equipoise on where the burden of risk lies in all patients with AAOCAs, especially in patients diagnosed at an older age.

Methods

We conducted a retrospective review of all adult patients who had Cardiovascular Computed Tomography scans with contrast at our institution between January 2013 and January 2025. A total of 20292 scans were digitally reviewed using key search terms such as “coronary anomalies”, “anomalous coronaries”, and aberrant coronary”. A total of 188 scans that met the search term criteria were subsequently screened. The primary endpoint was a composite MACE of cardiovascular death, non-fatal stroke, transient ischemic attack (TIA), acute coronary syndromes (ACS) and non-ACS troponin positive events.

A simple logistic regression model assessed the impact of coronary anatomy risk profile on the occurrence of MACE. Multivariable logistic regression models assessed the impact of coronary anatomy, CAD RADS class, age and ejection fraction on the occurrence of MACE. Kaplan Meier event-free survival curves were constructed stratified by CADRADS ≥ 3.

Results

We identified AAOCAs in 96 scans from 93 unique patients, resulting in a prevalence of 0.45% with a median age of 60.8years (IQR:52.8-70.3Y). The circumflex coronary artery origin was most frequently found to be anomalous (n=41, 44%). Of the 21 patients who had stress testing, just 1 of these was positive(4.8%), which occurred in a patient with CADRADS 4 CAD. MACE occurred in 9 patients, of which 3 were non-atherosclerotic events.

On univariable analysis, CADRADS was strongly associated with MACE (OR 2.04, 95% CI 1.27–3.28, p=0.003), whereas high-risk inter-arterial anatomy was not (OR 1.75, p=0.43). In multivariable analysis, CADRADS remained independently predictive (adjusted OR 1.91, 95% CI 1.15–3.16). Kaplan–Meier analysis showed no significant survival difference between high- and low-risk anatomy (log-rank p=0.45). The overall discriminative performance of high-risk anatomy alone was poor (AUC 0.57).

Conclusion

In adults diagnosed with AAOCAs at our institution, obstructive coronary atherosclerosis is the strongest independent predictor of the occurrence of MACE.