Coronary Arterial Anomalies in Adults with Congenital Heart Disease: From Detection to Management Strategies

Congenital coronary artery anomalies, whether native, associated with structural heart defects, or acquired following surgical repair, are an increasingly recognized cause of morbidity and sudden cardiac death in the expanding adult congenital heart disease (ACHD) population. Their clinical significance is heightened as the first generation of arterial switch operation (ASO) recipients reaches adulthood. This review presents a practical, evidence-based framework for the classification, multimodality imaging evaluation, risk stratification, and surgical and interventional management of coronary anomalies across the ACHD spectrum. Recommendations from the 2025 ACC/AHA and 2020 ESC guidelines are synthesized, key divergences in the approach to asymptomatic patients are highlighted, and critical knowledge gaps, including emerging technologies and the uncertain long-term coronary outcomes of the ASO generation, are discussed.