Coronary artery anomalies (CAAs) are a rare but clinically significant group of congenital abnormalities that are associated with myocardial ischaemia, malignant arrhythmias and sudden cardiac death, particularly in young people and athletes. Despite increasing recognition of these conditions and advances in cardiovascular imaging, there are still significant challenges regarding their classification, risk stratification and management, particularly with respect to surgical indications. This review provides a comprehensive overview of the current evidence on the classification, pathophysiology, diagnosis and management of CAAs, with a particular focus on surgical decision-making and contemporary guideline recommendations. A systematic literature search was conducted up to February 2025 using PubMed and Google Scholar. Priority was given to international guidelines, consensus statements, systematic reviews, meta-analyses and large observational studies. CAAs encompass a broad spectrum of anatomical variants and clinical presentations. Among these, anomalies of coronary origin are the most extensively studied. Surgical management is well established for coronary arteries arising from the pulmonary artery, particularly for anomalous left coronary artery from the pulmonary artery (ALCAPA). Substantial advances have also been made in the diagnosis, risk stratification, and treatment of anomalous aortic origin of a coronary artery (AAOCA), which has become a major focus of contemporary guideline recommendations. For patients with AAOCA, surgical correction, including unroofing, coronary reimplantation or coronary artery bypass grafting, is recommended for individuals with symptoms and/or high-risk anatomical features. In contrast, the diagnosis and management of myocardial bridging, coronary artery fistulas, and coronary artery ectasia remain controversial, with considerable variability in the indications for medical, percutaneous, and surgical treatment. The management of CAAs is an evolving field. While there is consensus for a limited number of anomalies, most cases still require individualised decision-making. It is essential to develop standardised diagnostic frameworks, improved risk stratification tools and outcome-based management criteria. A multidisciplinary, evidence-based approach involving cardiologists, cardiac imagers, interventional cardiologists and cardiac surgeons is crucial in order to optimise patient outcomes and reduce the risk of adverse cardiovascular events, including sudden cardiac death.

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.