Coronary artery disease in adults with anomalous aortic origin of a coronary artery

Abstract

Objectives: This study sought to characterize coronary artery disease (CAD) among adults diagnosed with an anomalous aortic origin of a coronary artery (AAOCA). We hypothesized that coronaries with anomalous origins have more severe CAD stenosis than coronaries with normal origins.

Methods: This single-center study of 763 adults with AAOCA consisted of 620 patients from our cardiac catheterization database (1958-2009) and 273 patients from electronic medical records query (2010-2021). Within left main, anterior descending, circumflex, and right coronary arteries, the CAD stenosis severity, assessed by invasive or computer tomography angiography, was modeled with coronary-level variables (presence of an anomalous origin) and patient-level variables (age, sex, comorbidities, and which of the four coronaries was anomalous).

Results: Of the 763 patients, 472 (60%) had obstructive CAD, of whom, 142/472 (30%) had obstructive CAD only in the anomalous coronary. Multivariable modeling showed similar CAD stenosis severity between coronaries with anomalous versus normal origins (P = .8). Compared with AAOCA of other coronaries, the anomalous circumflex was diagnosed at older ages (59.7 ± 11.1 vs 54.3 ± 15.8 years, P < .0001) and was associated with increased stenosis in all coronaries (odds ratio, 2.7; 95% confidence interval, 2.2-3.4, P < .0001).

Conclusions: Among adults diagnosed with AAOCA, the anomalous origin did not appear to increase the severity of CAD within the anomalous coronary. In contrast to the circumflex, AAOCA of the other vessels may contribute a greater ischemic burden when they present symptomatically at younger ages with less CAD. Future research should investigate the interaction between AAOCA, CAD, and ischemic risk to guide interventions.

Keywords: AAOCA, anomalous aortic origin of a coronary artery; CABG, coronary artery bypass grafting; CAD, coronary artery disease; LAD, left anterior descending coronary artery; LCx, left circumflex; LMCA, left main coronary artery; RCA, right coronary artery; adult congenital heart disease; congenital heart disease; coronary artery disease; coronary artery imaging; coronary surgery.

Graphical abstract

Atherosclerotic stenosis severity in coronaries among patients with AAOCA.

Figure 1

Coronary stenosis severity in patients with anomalous aortic origin of a coronary artery (AAOCA). Among 793 adults with an AAOCA, the maximum severity of coronary artery disease (CAD) stenosis was characterized for the left main, anterior descending, circumflex, and right coronary arteries. The heatmap depicts the proportions of anomalous and normal-origins coronaries with each stenosis severity. The stenosis in coronary arteries with normal origins was similar to those with anomalous origins after adjusting for age, sex, and comorbidities.

Figure E1

Flow diagram of overall cohort and modern subcohort. The cardiac catheterization database identified 620 patients who were previously coded for an anomalous aortic origin of a coronary artery (AAOCA) and atherosclerotic coronary artery disease (CAD) severity. An additional 273 patients were identified by querying the electronic medical records database and reviewing all available coronary imaging reports to confirm the presence of AAOCA and CAD assessment by invasive or computed tomography angiography. The combined cohorts of patients with AAOCA formed the overall cohort (n = 793) used in the primary analysis. The modern subcohort (n = 273) included more detailed coronary anatomy and was used for the secondary analysis. LMCA, Left main coronary artery; LAD, left anterior descending; LCx, left circumflex.

Figure E2

Dot plot of coronary artery disease stenosis stratified by the coronary and anomalous origin, Percent diameter stenosis due to coronary artery disease stratified first by the coronary and then by whether that coronary was anomalous. AAOCA, Anomalous aortic origin of a coronary artery; LMCA, left main coronary artery; LAD, left anterior descending; LCx, left circumflex; RCA, right coronary artery.