Successful Bentall procedure in a patient with a Fontan circulation

A 33-year old male patient with absent left atrioventricular connection and double outlet left ventricle developed severe aortic root dilatation and aortic regurgitation. He had undergone initial banding of the pulmonary trunk and atrial septectomy, eventually followed by the bidirectional Glenn procedure. At the time of the total cavopulmonary connection (at 27 years of age), his ascending aorta was plicated and wrapped. Subsequently, the aortic root below the wrapped ascending aorta showed further dilatation. The Bentall procedure was successfully carried out.