Anomalous origin of the right coronary artery (RCA) from the left sinus of Valsalva is a rare but clinically relevant congenital anomaly, since the RCA may be subjected to cyclical compression due to its interarterial course. At least in the past, most patients experienced bad outcomes before being diagnosed with a malignant variant. Chronic (often subclinical) myocardial ischemia and possible arrhythmias are common complications. Once symptoms or electrocardiographic signs of myocardial ischemia become apparent, a quick diagnosis is mandatory. We report the case of a late symptomatic woman in whom RCA originated from the opposite sinus of Valsalva. The malignant variant was confirmed at transesophageal echocardiography directly in the catheterization laboratory, soon after angiographic diagnosis of aberrant origin.

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