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We describe a successful surgical treatment in a 2.5-year old boy with Loeys-Dietz syndrome, in whom we performed aortic arch and ascending aorta replacement with a valve-sparing operation (VSO) of the aortic root because of significant aortic insufficiency and dilation of the aortic root. We believe that VSO is ideal for treating young patients with aortic root aneurysm with normal or minimally diseased aortic cusps to avoid the disadvantages of prosthetic valve replacements.

Valve-sparing replacement of the ascending aorta and aortic arch in a child with Loeys–Dietz syndrome