Herein is reported a successful surgical case of aortic root replacement for aortic regurgitation in a patient with osteogenesis imperfecta. A 37-year-old male, who had been diagnosed as osteogenesis imperfecta of type IA, underwent mitral valve repair for mitral regurgitation. Severe aortic regurgitation developed four years after surgery. A stentless bioprosthesis was implanted using the full root technique for fear of progressive dilatation of the sinus of Valsalva or aortic dissection, which is well documented in osteogenesis imperfecta. Aortic root replacement with a stentless bioprosthesis for aortic regurgitation represents an alternative procedure of choice for a patient with osteogenesis imperfecta.
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