Abnormalities of the coronary arterial circulation represent a broad spectrum of anomalies. Some of these coronary arterial anomalies are of little clinical significance and are generally discovered either as incidental findings at autopsy or at the time of diagnostic coronary angiography in patients with atherosclerotic ischaemic cardiac disease. Some of these coronary arterial anomalies, however, are associated with a markedly abnormal natural history that may include significant cardiac morbidity very early in infancy, or increased risk of cardiac sudden death in childhood or adolescence. It is therefore important that we have a system for the naming and classification of these anomalies, to stratify them according to the associated risk and to develop rational approaches to evaluation and management. To apply any system of nomenclature reliably and responsibly, it is essential that each term be associated with a definition that is known and accepted by users of terminology. Such definitions are included in the specifications of both the Society of Thoracic Surgeons Congenital Heart Surgery Database and the European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Database. These databases use the Diagnostic Short List of the version of the International Paediatric and Congenital Cardiac Code derived from the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons. This article will provide the nomenclature and definitions used by these databases for anomalies of the coronary arteries.

Congenital anomalies of the coronary arteries are an uncommon, but important, cause of pain in the chest, myocardial ischaemia and even sudden cardiac death, especially in young individuals. This paper focuses on the surgical treatment of congenital anomalies of the coronary arteries in adults; indications for surgery and the different surgical options will be reviewed.

Congenital coronary arterial abnormalities as isolated lesions are exceedingly rare. The electrocardiogram, while a reasonable adjunct in the diagnosis of coronary arterial abnormalities, should not supplant a good history and physical examination. Careful attention must be devoted to any signs or symptoms of ischaemic pain in the chest or syncope, which must not be overlooked. Exertional pain in the chest and exertional syncope should prompt an extensive evaluation by both the echocardiographer and the electrophysiologist. Clearance for participation in sports should be curtailed until a complete evaluation has ruled out the presence of any of the following disorders: a channelopathic mutation, a cardiomyopathy, or a congenital coronary arterial anomaly. Major abnormalities in the coronary arteries may present in the first few months of life or remain dormant until the exertional demands of adolescence unmask symptoms of myocardial ischaemia. Congenital coronary arterial anomalies may be analysed in the following major diagnostic groups: anomalous origin of the left coronary artery from the pulmonary artery, anomalous aortic origin of a coronary artery from the wrong aortic sinus of Valsalva, atresia of the left main coronary artery, myocardial bridges, and coronary arterial fistulas. The advent of state-of-the-art modalities of imaging seems, at times, to have supplanted the electrocardiogram in making the diagnosis of potentially serious coronary artery abnormalities, especially in asymptomatic patients. However, as is also the case for a detailed history and physical examination, the electrocardiogram provides a potentially insightful look at the coronary arteries. Furthermore, the past decade has witnessed an increase in the use of the electrocardiogram as a screening tool in the assessment of the risk of sudden cardiac death in athletes in high school.