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主动脉瓣与出血:海德氏综合征。

Of aortic valve and bleeding: Heyde's syndrome.

作者信息

Famularo Giuseppe, Marrollo Marzia

机构信息

Internal Medicine and Gastroenterology, San Camillo Hospital, Rome, Italy.

Internal Medicine and Gastroenterology, San Camillo Hospital, Rome, Italy.

出版信息

Am J Emerg Med. 2020 Nov;38(11):2493.e1-2493.e2. doi: 10.1016/j.ajem.2020.06.024. Epub 2020 Jun 23.

Abstract

An 80-year-old woman with severe aortic stenosis presented with relapsing enterorrhagia and severe anemia. A video capsule pan-endoscopy showed multiple sites of complex mucosal angiodysplasia in the jejunum. Direct hemostatic treatment of accessible angiodysplasia was done with argon plasma coagulation, and the patient was urgently referred for trans-catheter aortic valve replacement (TAVR). At follow-up 1 month and 3 months later, she was doing well with no further episodes of bleeding. Heyde's syndrome is referred to as the association of aortic stenosis, gastrointestinal angiodysplasia, bleeding, and anemia. It is an acquired type2A von Willebrand syndrome caused by the proteolysis and loss of the largest polymers of vWF due to the high shear forces generated through the stenotic aortic valve. The qualitative and quantitative vWF defects play a central role in the angiogenesis and development of gastrointestinal angiodysplasia The vWF abnormalities are closely associated with the hemodynamic severity of the aortic valve stenosis. Valve replacement is the pivotal strategy to achieve the long-term resolution of bleeding recurrences. TAVR is a valuable option particularly in high-risk patients for whom surgical valve replacement is not feasible.

摘要

一名80岁重度主动脉瓣狭窄女性患者出现复发性肠出血和严重贫血。视频胶囊全内镜检查显示空肠有多个复杂黏膜血管发育异常部位。对可触及的血管发育异常进行了氩等离子体凝固直接止血治疗,患者被紧急转诊接受经导管主动脉瓣置换术(TAVR)。在1个月和3个月后的随访中,她情况良好,未再出现出血发作。海德综合征是指主动脉瓣狭窄、胃肠道血管发育异常、出血和贫血之间的关联。它是一种获得性2A型血管性血友病综合征,由狭窄主动脉瓣产生的高剪切力导致血管性血友病因子(vWF)最大聚合物的蛋白水解和丢失所致。vWF的定性和定量缺陷在胃肠道血管发育异常的血管生成和发展中起核心作用。vWF异常与主动脉瓣狭窄的血流动力学严重程度密切相关。瓣膜置换是实现出血复发长期缓解的关键策略。TAVR是一种有价值的选择,尤其对于手术瓣膜置换不可行的高危患者。

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