Valve-sparing aortic root replacement and aortic valve repair for a 2-year-old girl with Loeys-Dietz syndrome

We report the case of a 2-year-old girl with Loeys-Dietz syndrome complicated by aortic root dilatation and aortic regurgitation. We performed valve-sparing aortic root replacement with reimplantation technique and aortic valve repair using central plication and free-margin reinforcement simultaneously. The postoperative course was uneventful and the latest echocardiography, 5 years after procedure, revealed trivial aortic insufficiency. Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder characterized by arterial tortuosity and aneurysm, hypertelorism and bifid uvula or cleft palate.