Abstract
Congenital coronary anomalies are classified into four categories: ① anomalies of the location of the orifice or abnormal course of the coronary artery; ② coronary ostial stenosis or atresia; ③ anomalies of the coronary vessels; and ④ anomalous termination of the coronary artery. The anomalous location of the orifice includes ectopic ostium within the aortic root and ectopic origin from other vessels, such as a pulmonary artery or brachiocephalic artery. These coronary anomalies present either as an isolated anomaly or in combination with other anomalies. Although congenital coronary anomalies are rare, we should be aware of them as the first 2 types of anomalies have a potential for life-threatening presentations, including myocardial infarction, arrhythmia, or sudden death. However, early detection by mass electrocardiographic screening of school-aged children in Japan is arduous. Congenital coronary anomalies are now recognized as one of the leading causes of sudden death in young athletes second only to cardiomyopathies, making early detection critical. Congenital coronary anomalies often occur alone, however, they may also be accompanied by congenital heart diseases, and some of the coronary anomalies may have a significant impact on the postoperative outcome. Increasing awareness of these pathologies may help earlier diagnosis and treatment of this potentially life-threatening disease.