Anomalous origin of the left main coronary artery from the right sinus of Valsalva with an intramural course identified by transesophageal echocardiography in a 14 year old with acute myocardial infarction.

Coronary artery anomalies have an incidence of 0.6% to 1.3% in angiographic studies and 0.3% in an autopsy series. Anomalous origin of the left main coronary artery (LMCA) from the right sinus of Valsalva (RSOV) represents a small fraction (1.3%) of these anomalies, with an overall prevalence of 0.017% to 0.03% in angiographic studies. The high incidence of sudden cardiac death associated with this specific anomaly during or immediately after vigorous physical exercise makes identification and appropriate surgical intervention critical. We present a case report of a 14-year-old patient with an LMCA arising from the RSOV with an initial intramural course, presenting with acute myocardial infarction (AMI) as the first indication of the anomaly. Transthoracic echocardiogram suggested this anomaly, which was confirmed by cardiac catheterization and transesophageal echocardiogram.