Congenital anomalies of the coronary artery are rare. However, they can cause sudden death because of arrhythmia. We present a case of a 62-year-old man with severe aortic valve regurgitation associated with an anomalous origin of a narrowed right coronary artery (IB2 according to the Shirani Classification) detected on preoperative coronary three-dimensional computed tomography (CT) . The patient underwent both aortic valve replacement for aortic regurgitation, and coronary artery bypass. The postoperative course was uneventful.
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