Anomalous aortic origin of coronary arteries is a congenital heart defect in which one coronary artery arises from the opposite sinus (the left coronary artery from the right coronary sinus, or the right coronary artery from the left sinus). The initial segment of the abnormal artery courses between the great arteries and is usually located within the aortic wall. These anomalies are far from rare, affecting 0.1% to 0.3% of the population, and carry a high risk of sudden cardiac death. It is thought that the main mechanism of cardiac death is external compression of the abnormal coronary artery between the great arteries as they expand during strenuous exercise. The risk of sudden death is particularly high in patients with an anomalous left coronary artery or associated anatomic lesions (stenotic intramural segment, slit-like deformation of the ostium), and also in young athletes. A common presentation is sudden unexplained death. The diagnosis can be made by echocardiographic examination. Silent myocardial ischemia must be sought, with stress echocardiography and stress nuclear imaging. Various surgical techniques have been described. Our own technique involves the creation of a neo-ostium in the appropriate sinus. Surgical treatment is relatively safe and provides satisfactory results, most patients having no residual myocardial ischemia under stress. Surgery is recommended for all patients with an anomalous left coronary artery, regardless of symptom status, as well as for symptomatic patients with an anomalous right coronary artery (aborted sudden death, symptoms under stress, myocardial ischemia under stress). Because of the incidence and severity of these anomalies, careful echocardiographic evaluation should be performed routinely, at least in young athletes.
