Anomalous origin of the right coronary artery evaluated with multidetector computed tomography and its clinical relevance

Background Anomalous origin of the right coronary artery (AORCA) is a rare congenital anomaly that may cause myocardial ischemia and sudden death. Methods We reviewed the clinicopathological records of three cases of AORCA, and compared these with two cases of sudden cardiac death with AORCA revealed by autopsy. Results We report three juvenile cases with an AORCA originating above the commissural junction between the left and right aortic sinuses, with interarterial and intramural compression. They presented with exertional symptoms and were diagnosed with an AORCA by multidetector computed tomography (MDCT), which successfully delineated the spatial resolution of the anomalous origin and course of the right coronary artery (RCA), in the operating room. All three underwent successful surgical unroofing of the RCA. Two cases of sudden cardiac death with AORCA revealed by autopsy showed a slit-like orifice, acute-angled take-off, and long intramural course of the RCA, resembling the RCAs of three juvenile cases. Conclusions It is crucial to be alert to the presentation of exertional symptoms, as sudden death may be the first manifestation of an anomalous coronary artery, such as those observed in these three cases. MDCT provided an excellent definition and spatial resolution of the unusual origin and intramural course of the RCA, facilitating the correct surgical remedy and resulting in a good outcome for the patients.