Surgical treatment of Loeys-Dietz syndrome in a 3-year-old: case report and review of literature

Aortic complications of Loeys-Dietz syndrome (LDS) rarely present in children. Here we describe a case of LDS type 2 in a 3-year-old child with severe aortic root dilation and severe aortic regurgitation. A Bentall procedure combined with a Cabrol-type coronary modification was used to treat this child. In order to minimize the need for reintervention as the child grows. We chose a composite valve-graft by a St Jude Regent 21# mechanical valve seated within a 24 mm Gore-Tex graft to finish the Bentall procedure. Echocardiographic studies demonstrated good valve and ventricular function at 1-year follow-up. This child is one of the youngest LDS patients to receive a Bentall procedure and the way using a composite valve-graft to finish the operation can provide a reference for the surgical strategies of such patients in the future. Children with LDS and severe aortic annulus dilatation combined with severe aortic valve regurgitation require early surgical intervention, and implantation of a mechanical valved-conduit can be utilized successfully. Life-long follow-up of the valved-conduit and arterial vessels of these patients is necessary. The experience gained from this case contributes to the management of the rare LDS patient who presents at an early age with aortic root and valve pathology. Close monitoring and early intervention are important.