Soma Katsura, Abe Hajime, Takeda Norihiko, Shintani Yukako, Takazawa Yutaka, Kojima Toshiya, Fujiu Katsuhito, Semba Hiroaki, Yamashita Hiroshi, Hirata Yasunobu, Fukayama Masashi, Nagai Ryozo
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, Japan.
Int Heart J. 2012;53(1):75-7. doi: 10.1536/ihj.53.75.
Mitral and aortic valve regurgitation is commonly found in osteogenesis imperfecta (OI) patients, however, little is known about the myocardial involvement in this disorder. An 82-year-old man with OI developed heart failure and was admitted to our hospital. Echocardiogram revealed severe mitral regurgitation without left ventricular (LV) dilatation, but with LV wall thickening. Histological analysis exhibited interstitial fibrosis of the myocardium in addition to myxoid changes of the mitral leaflet. These findings suggest that OI patients may develop LV remodeling together with diastolic dysfunction.
二尖瓣和主动脉瓣反流在成骨不全（OI）患者中很常见，然而，对于这种疾病中心肌受累的情况却知之甚少。一名82岁的OI男性患者出现心力衰竭并入住我院。超声心动图显示严重二尖瓣反流，无左心室（LV）扩张，但有LV壁增厚。组织学分析显示心肌间质纤维化，二尖瓣叶有黏液样改变。这些发现表明，OI患者可能会出现LV重塑并伴有舒张功能障碍。
