Coronary artery anomalies (CAA) are vanishingly rare, affecting less than 1% of the general population. While the majority of anomalies do not cause significant symptoms; those that do, have devastating outcomes on the patient. Seventeen percent of deaths from exercise is attributed to CAA, and over half of these present as sudden death making CAA the second most common cause of sudden cardiac death in individuals. Computed tomography is generally regarded as the first-line investigation due to its superior ability to delineate the course of the coronary vessels and the surrounding structures, while intravascular coronary angiography can be helpful in assessing the vessels if there is evidence of stenosis. A multidisciplinary approach is adopted with patient expectations at the core of the management. Once the decision to operate has been made, there are multiple techniques available to the surgeon for the management of anomalous vessels. Surgical repair forms the key management step in such patients. Currently, surgery in elective cases is associated with extremely low morbidity and mortality and it is considered a safe option with a fantastic long-term prognosis. The ideal approach for assessment and risk stratification remains uncertain, and the inherent variability of coronary anomalies and patient factors demands a multidisciplinary team with an individualized approach.
Keywords: ALCAPA; cardiac surgery; congenital heart disease; coronary anomalies.
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