Coronary Artery Anomalies Revisited: Description of the Types, Pathophysiology and Treatment Options Based on Latest Guidelines

Coronary artery anomalies (CAAs) represent a rare but clinically significant group of congenital abnormalities, often implicated in sudden cardiac death among young individuals. Despite increasing recognition, standardized diagnostic and management pathways remain underdeveloped, particularly regarding surgical intervention. This narrative review aims to synthesize current evidence on the classification, pathophysiology, and contemporary treatment options for CAAs, with a specific focus on the surgical indications and guidelines. A systematic literature search was conducted through February 2025 across PubMed, Embase, Google Scholar, and UpToDate, using relevant MeSH terms and keywords related to coronary anomalies, sudden cardiac death, imaging modalities, and treatment strategies. Priority was given to high-level evidence including guidelines, systematic reviews, and large observational studies, in English and French. CAAs encompass a wide anatomical and clinical spectrum. Among these, anomalies of origin and course, such as anomalous aortic origin of a coronary artery (AAOCA), are now better defined and integrated into international guidelines, with surgical repair (e.g., unroofing, reimplantation, bypass) increasingly recommended in symptomatic patients or those with high-risk anatomical features. In contrast, anomalies like myocardial bridges, coronary artery fistulas, or ectasia remain controversial in both diagnosis and management, with inconsistent thresholds for medical versus surgical treatment. The variability in care stems from the lack of a unified classification system, limited prospective data, and underutilization or misinterpretation of imaging modalities such as coronary CT angiography and intravascular imaging. The management of coronary artery anomalies is evolving, particularly in the domain of surgical indications. Clear consensus exists only for select anomalies, leaving others subject to individualized, often non-standardized decisions. There is an urgent need for a harmonized diagnostic framework and outcome-based criteria to guide surgical and non-surgical interventions. A multidisciplinary, evidence-informed approach is essential to optimize outcomes and reduce the risk of sudden cardiac events.