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合并瓣膜病伴疑似获得性血管性血友病综合征

[Combined Valvular Disease with Suspected Acquired von Willebrand Syndrome].

作者信息

Mori Kazuki, Anai Hirofumi, Okamoto Keitaro, Shuto Takashi, Wada Tomoyuki, Miyamoto Shinji

机构信息

Department of Cardiovascular Surgery, Oita University, Yufu, Japan.

出版信息

Kyobu Geka. 2020 Mar;73(3):192-195.

PMID:32393701
Abstract

A 73-year-old female was referred to our department for surgical treatment of combined rheumatic valvular disease. She had undergone percutaneous transluminal mitral commissurotomy 29 years before. She underwent double valve replacement with bioprosthetic valves and tricuspid annuloplasty combined with left atrial appendectomy for chronic atrial fibrillation. Because of remarkable bleeding following surgery, rapid blood transfusion and re-thoracotomy were immediately performed. However, the bleeding persisted even after re-thoracotomy, and acquired von Willebrand syndrome due to valvular disease was suspected. Von Willebrand factor/factor Ⅷ preparation was administered, and bleeding promptly became under control. Recently, acquired von Willebrand syndrome due to valvular disease has attracted attention. We need to keep it in mind during perioperative management for patients with valvular disease.

摘要

一名73岁女性因风湿性心脏瓣膜病合并症被转诊至我科接受手术治疗。她29年前曾接受经皮二尖瓣交界切开术。此次她接受了生物瓣膜双瓣置换术以及三尖瓣环成形术,并因慢性心房颤动行左心耳切除术。术后出现大量出血,遂立即进行快速输血及再次开胸手术。然而,再次开胸后出血仍持续,怀疑是瓣膜病导致的获得性血管性血友病综合征。给予血管性血友病因子/Ⅷ因子制剂后,出血迅速得到控制。近年来,瓣膜病导致的获得性血管性血友病综合征受到关注。在瓣膜病患者围手术期管理过程中我们需要牢记这一点。

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