Uemura S, Harada Y, Kasegawa H, Simura H, Murakami S, Morooka N
Division of Cardiovascular Surgery, Chiba Kaihin Municipal Hospital, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1991 Nov;39(11):2074-7.
A 34-year-old man with osteogenesis imperfecta who underwent successful mitral valve replacement due to mitral regurgitation was reported. Cardiac disease associated with osteogenesis imperfecta is very rare and only fifteen patients were operated under the extracorporeal circulation previously. While excessive hemorrhage due to tissue fragility was observed in 7 of 15 patients, perioperative course of the case reported here was completely uneventful. The difference of hemorrhagic tendency as well as etiology of osteogenesis imperfecta will be defined according to the advance of technology in collagen genetics and biochemistry in future.
报道了一名34岁成骨不全症男性患者，因二尖瓣反流接受二尖瓣置换术并获成功。与成骨不全症相关的心脏疾病非常罕见，此前仅有15例患者在体外循环下接受手术。15例患者中有7例因组织脆弱出现大量出血，而本文报道病例的围手术期过程完全平稳。未来，随着胶原遗传学和生物化学技术的进步，成骨不全症出血倾向的差异及病因将得以明确。
