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Review
. 2025 Aug 12;21(4):54-64.
doi: 10.14797/mdcvj.1613. eCollection 2025.

Coronary Artery Anomalies in Review: Anomalous Origin, Aneurysms, and Fistulae

Jack A Hoover  1   2 , Keshika Catakam  3 , Rachel E Wittenberg  3 , Jordan P Bloom  3 , Doreen DeFaria Yeh  3 , Q Joyce Han  3 , Ada C Stefanescu Schmidt  3
Affiliations
Review

Coronary Artery Anomalies in Review: Anomalous Origin, Aneurysms, and Fistulae

Jack A Hoover et al. Methodist Debakey Cardiovasc J. .

Abstract

Coronary artery anomalies are rare congenital abnormalities involving abnormal coronary artery origin and incomplete/abnormal vessel development. These anomalies encompass a broad anatomical spectrum with varied clinical presentations ranging from sudden cardiac arrest in young athletes to incidental findings in asymptomatic adults. While more data on anatomy and clinical presentation has emerged in the last decade, management remains highly individualized, guided by clinical presentation, anatomical characteristics, and patient preferences. This review provides an overview of each anomaly and recent advances in imaging, surgical planning, and risk stratification. We also highlight the challenges in optimizing management strategies, addressing gaps in evidence, and achieving consensus to refine risk stratification tools and support clinical decision-making.

Keywords: ALCAPA/ARCAPA; anomalous coronary arteries; coronary artery aneurysm; coronary artery fistula.

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Conflict of interest statement

Dr. Ada Stefanescu Schmidt is a consultant for reCross Cardio and B. Braun Medical Inc., and her research is partly supported by Edwards Lifesciences and Occlutech. The other authors have no competing interests to declare.

Figures

Two CCTA images of AAOCA-R with slit-like ostium and intramural course
Figure 1
A 33-year-old man with R-AAOCA diagnosed as an adult. (A) CCTA shows the anomalous origin of the dominant RCA from the left aortic sinus of Valsalva, (B) with slit-like ostium and intramural course. R-AAOCA: right anomalous origin of the left coronary artery; CCTA: coronary computed tomogram angiography; RCA: right coronary artery
Coronary angiogram, TTE, and CCTA showing ALCAPA with collateral vessels pre- and post-surgical repair
Figure 2
A 26-year-old woman with ALCAPA diagnosed as an adult. (A) Preoperative coronary angiogram shows a large RCA with septal and epicardial collaterals to the LAD and LCX that converge into LMCA draining into the PA. (B) Preoperative transthoracic echocardiography with color Doppler reveals collateral vessels in the interventricular septum. (C1,2) Pre- and postoperative CCTA demonstrate the abnormal origin of LM from the PA and postsurgical SVG graft placement, respectively. ALCAPA: anomalous left coronary artery from the pulmonary artery; RCA: right coronary artery; LAD: left anterior descending artery; LCX: left circumflex artery; LMCA: left main coronary artery; PA: pulmonary artery; cCTA: coronary computed tomogram angiography; SVG: saphenous vein graft
See this image and copyright information in PMC

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