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This abnormal coronary anatomy was demonstrated by simultaneous, selective coronary arteriography in a 40-year-old man with a secundum atrial septal defect (panel A: left lateral).
The ectopic right coronary artery (RCA) originated from the anterior aorta, 3.5 cm above the right sinus of Valsalva. The trunk descended steeply, gave rise to a small, non-dominant right coronary artery into the right coronary sulcus and a large dominant left circumflex (LCx) artery which took a retroaortic course to the posterior atrioventricular groove. Its position relative to these other structures was confirmed by a multislice computed tomographic scan (panel B). The left anterior descending coronary artery originated high in the left sinus of Valsalva near the sino-tubular junction and had a normal course.
Ectopic coronary arteries are seen in 0.05–0.1% of angiographic series. They may have a tangential origin or proximal intramural course, may lead to a slit-like, flow-compromising orifice (not present in this case), and have been associated with sudden death. Although this particular anomaly is benign, if necessary, it is important to bring it to the attention of the cardiac surgeon so as to avoid accidentally crossclamping or transecting the vessel during surgery. This association of an ectopic RCA with an anomalous LCx is a very rare abnormality.