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

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.