Maekawa Y, Hayashi T, Fujito T, Hoshi K, Kamishirado H, Iwasaki Y, Hisauchi I, Inoue T, Morooka S
Department of Cardiology, Koshigaya Hospital, Dokkyo University School of Medicine, Saitama.
J Cardiol. 1997;29 Suppl 2:89-94.
A 54-year-old man presented with osteogenesis imperfecta complicated with both aortic regurgitation due to annuloaortic ectasia and mitral regurgitation secondary to tendon rupture. He had spinal and carpal deformities in his childhood, and heart murmurs were identified at the age of 25. He was admitted complaining of dyspnea on effort. His height was 142 cm and his weight was 46 kg. He had kyphosis, scoliosis and carpal deformity. Blue sclera was not observed. Chest radiography showed cardiomegaly and lung congestion. Echocardiography showed annuloaortic ectasia, mild aortic regurgitation, and serious mitral regurgitation due to postero-apical tendon rupture. Bone deformity and his statues were indicative of osteogenesis imperfecta. He received modified Bentall and mitral valve replacements.
一名54岁男性，患有成骨不全症，并发主动脉瓣环扩张所致的主动脉瓣反流以及腱索断裂继发的二尖瓣反流。他童年时即出现脊柱和腕骨畸形，25岁时发现心脏杂音。因劳力性呼吸困难入院。他身高142厘米，体重46千克。有脊柱后凸、脊柱侧凸和腕骨畸形。未观察到蓝色巩膜。胸部X线片显示心脏扩大和肺淤血。超声心动图显示主动脉瓣环扩张、轻度主动脉瓣反流以及后心尖腱索断裂导致的严重二尖瓣反流。骨骼畸形及体征提示成骨不全症。他接受了改良Bentall手术及二尖瓣置换术。
