Abstract Body (Do not enter title and authors here): Background: Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital anomaly affecting around 0.1-0.3% of the population and is a known cause of myocardial ischemia and sudden cardiac death, particularly in young athletes. However, limited national data on AAOCA-related mortality are available.
Aims: We aim to characterize trends and demographic disparities in AAOCA-related mortality in the US from 1999 to 2023.
Methods: We queried the CDC WONDER multiple-cause-of-death database for AAOCA-related deaths (ICD-10 Q24.5) among all age groups. Age-adjusted mortality rates (AAMR) per 1,000,000 population with 95% confidence interval (CI), stratified by year, sex, race, regions, and urbanization status, were abstracted. Annual (APC) and average annual percent changes (AAPC) were computed by Joinpoint regression.
Results: From 1999 to 2023, there were 6,665 AAOCA-related deaths. The overall AAMR fell from 1.1 in 1999 to 0.9 by 2001 (APC: -10.5; 95% CI: -26.0, 8.3), rose to 1.1 in 2008 (APC: 2.2; 95% CI: -1.2, 5.8), declined significantly to 0.5 by 2020 (APC: -4.1; 95% CI: -5.7, -2.4), and then increased to 0.8 by 2023 (APC: 11.7; 95% CI: -3.3, 29.0). Male AAMRs exceeded females (1999: 1.3 vs 0.9; 2023: 1.2 vs 0.4), with women’s mortality significantly declining (AAPC: -3.4; p=0.02) versus stable males (AAPC: 0.09; p=0.9). Amongst races, both from 1999-2020 and 2021-2023, the non-Hispanic (NH) Blacks exhibited the highest AAMR (1.8 vs 1.5), trailed by NH Whites (0.8 vs 0.6) and Hispanics (0.5 vs 0.6); NH Whites saw the greatest decline (AAPC: -0.8; p=0.6). Regionally, AAMRs from 1999-2020 were 0.9 in the Northeast, Midwest, and South and 0.8 in the West. All regions showed a decline in mortality throughout the study period, with the steepest in the Midwest (AAPC: -2.0; p=0.0005). Both urban and rural AAMRs were 0.9, with the rural region showing a steeper but nonsignificant decline (AAPC: -3.8; p=0.1) than urban (AAPC: -1.9; p=0.00006). Among age groups, <44 years mortality declined, with the steepest drop in 15-44 years (AAPC: -3.2; p=0.007), while mortality for >45 years plateaued (AAPC: 0.2; p=0.8).
Conclusion: Although AAOCA mortality has declined overall, especially in children and young adults, it remains high among men, NH Blacks, and Midwest residents, and has recently rebounded, underscoring the need for targeted early detection, risk stratification, and surveillance to guide timely interventions in high-risk groups.