Triple Outlet Right Ventricle, With Duplication of the Aortic Root and Intrapericardial Ascending Aorta

Abstract

We present a case of a highly unusual congenital cardiac malformation, namely, triple outlet right ventricle with duplication of the aortic root and the intrapericardial component of the ascending aorta. A girl, aged five, presented with complaints of cyanosis and effort intolerance and was diagnosed with double-outlet right ventricle and subpulmonary infundibular stenosis. Intraoperatively, we noted that the aortic root was guarded by two separate aortic valves, oriented anteroposteriorly relative to each other, and separated within the right ventricle by a muscle bar. Postoperative interrogation by both echocardiography and computed tomography confirmed the surgical findings. To the best of our knowledge, our case is the first example of duplication of the aortic root to produce triple outlet right ventricle.

Keywords: aorta; cardiac anatomy; computed tomography; embryology.