Acquired Von Willebrand Syndrome In Aortic Stenosis: Case Report And Review.

Said Alsidawi, Marian Couto, Angel López-Candales
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Abstract

We present a case of a 61 year old man who presented with a 3 week history of easy bruising and ecchymoses in both thighs and right arm without significant trauma. Physical exam was remarkable for oozing gums, diminished second heart sound, a systolic ejection murmur at the aortic position with radiation to carotids, and delayed pulses. Laboratories were remarkable for iron deficiency anemia. Echocardiogram was consistent with severe aortic stenosis. Colonoscopy revealed several arteriovenous malformations throughout the colon. There is an association between severe aortic stenosis and gastrointestinal bleeding. The pathogenesis of Heyde's Syndrome involves iron deficiency anemia due to acquired von Willebrand factor (vWF) deficiency and ultimately gastrointestinal angiodysplasia. Correct diagnosis and management warrants a multidisciplinary approach.

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主动脉狭窄的获得性血管性血友病:病例报告和回顾。
我们报告一个61岁的男性病例,他表现为3周的大腿和右臂容易瘀伤和瘀斑,没有明显的创伤。体格检查显示牙龈渗出,第二心音减弱,主动脉部位收缩期射血杂音伴颈动脉放射,脉搏延迟。实验室在缺铁性贫血方面表现出色。超声心动图显示主动脉严重狭窄。结肠镜检查显示整个结肠有几个动静脉畸形。严重的主动脉狭窄与胃肠道出血之间存在关联。Heyde综合征的发病机制涉及获得性血管性血友病因子(vWF)缺乏引起的缺铁性贫血,最终导致胃肠道血管发育不全。正确的诊断和管理需要多学科的方法。
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