Dilation of the aortic root and the ascending aorta is a well-known cardiovascular manifestation in pediatric patients with underlying connective tissue disorders,E1Go often leading to aortic valve incompetence and to aortic dissection risk. The Bentall procedure, a preventive operation, is a low-risk operation with considerably durable outcomes.1Go Alternatively, other more conservative techniques have been proposed: aortic root remodeling and reimplantation.2,3Go

Arterial tortuosity syndrome (ATS) is a rare condition with an autosomal recessive inheritance characterized by tortuosity, elongation, stenosis, and aneurysm formation in the major arteries.4Go Approximately 35 patients from 21 families have been reported thus far worldwide. This is the first case ever reported in the literature of a patient with ATS undergoing aortic root surgery with sparing of the aortic valve. For the full Article Click here