Coronary artery anomalies that take place during fetal development are determined in approximately 1.3% of coronary angiograms. The right coronary artery originating from the left coronary system is an extremely rare variation of the single coronary artery anomaly in which the prognosis is usually benign provided that the anomalous vessel dose not pass between the aorta and the pulmonary artery. Anomalous right coronary artery anomaly has been rarely associated with other congenital cardiovascular anomalies such as transposition of the great vessels and tetralogy of Fallot.
To date, a few attempts at classification have been made for coronary artery anomalies, but none of them seems comprehensive or practical for clinicians. The clinical significance of coronary anomalies is usually determined by underlying anatomic features of the wrong coronary origin and/or coronary atherosclerosis. Although coronary angiography is an important diagnostic method, new non-invasive methods such as coronary computed tomography angiography and cardiac magnetic resonance imaging have important roles to play in characterizing this coronary anomaly. It should be noted that the management strategy of these patients may vary based on clinical presentation, anatomical details and additional findings. (Cardiol J 2012; 19, 2: 122–129)