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Osteogenesis imperfecta is a disease in which fragile bones readily cause fracture. Valvular disease concurrently develops. However, the surgery-related mortality rate is approximately 30%. In this study, we report 2 patients with osteogenesis imperfecta who underwent valvular heart surgery. Patient 1 was a 31-year-old male. He had previously been diagnosed as having osteogenesis imperfecta. Echocardiography suggested aortic valve insufficiency, and aortic valve replacement was performed. Patient 2 was a 59-year-old male. During admission, osteogenesis imperfecta was diagnosed. Echocardiography suggested mitral valve insufficiency, and mitral valve plasty was performed. In the 2 patients, intraoperative hemorrhage was marked. However, there were no fatal complications. We also reviewed the literature.
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