Occlusion of the left main coronary artery os by a tethered aortic valve cusp

Abstract

We report a 4-month-old girl who presented with poor ventricular function and was found to have occlusion of the left main coronary artery os by a tethered aortic cusp. The patient underwent surgical delamination of the aortic valve leaflet, revealing a normal left coronary os. After operative repair, her left ventricular function improved significantly. This diagnosis should be included in the differential in all children presenting with cardiac dysfunction.