A CASE OF AORTIC REGURGITATION ASSOCIATED WITH OSTEOGENESIS IMPERFECTA SUCCESSFULLY TREATED BY AORTIC VALVE REPLACEMENT

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  • 骨形成不全症に併存した大動脈弁閉鎖不全症の１例

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A 48-year-old woman with osteogenesis imperfecta was found to have aortic regurgitation on screening done when she was 40-years-old. The patient was 143cm tall. Since childhood the patient had suffered from several bone fractures. On physical examination, bone deformities blue sclera, and her stature were the clinically manifestations of osteogenesis imperfecta. An aortic valve replacement with a 25-mm. On-X prosthetic valve was successfully performed for aortic valve insufficiency and slight annulo-aortic ectasia. She had a history of open-heart surgery for an atrial septal defect in childfood ; her soft tissues and sternum were fragile. Thus, the surgery required the utmost care. On pathology, the aortic valve and media layer of the aorta had mucoid deposition. The patient recovered from surgery and was discharged. Osteogenesis imperfecta is one of the collagen diseases caused by a gene abnormality, which results in fragile bones that are easily fractured. Cardiovascular disease is rarely associated with osteogenesis imperfecta, and the surgical mortality rate is high.

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