Abstract
An 11-year-old boy presented with myocardial ischemia and was found to have an aberrant left main coronary artery from the right sinus of Valsalva coursing between the aorta and pulmonary artery, as well as a small arteriovenous malformation from a right atrial branch of the right coronary artery to the right atrium. Distinctive echocardiographic findings were supported by angiographic and magnetic resonance imaging studies. Treadmill and scintigraphic stress testing were normal. Corrective surgery was accomplished by a modified technique to unroof the intramural proximal course of the left coronary artery, without postoperative complications. The anatomy and pathophysiology of this rare coronary lesion are reviewed.