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胃窦血管扩张综合征合并主动脉瓣狭窄:黑德综合征的一种变体?

Gastric Antral Vascular Ectasia Syndrome With Aortic Stenosis: A Twist on Heyde Syndrome?

作者信息

Bathobakae Lefika, Khalid Noman, Ozgur Sacide S, Adalja Devina, Doshi Rajkumar, Melki Gabriel, Amer Kamal, Cavanagh Yana, Baddoura Walid

机构信息

Internal Medicine, St. Joseph's University Medical Center, Paterson, NJ, USA.

Cardiology, St. Joseph's University Medical Center, Paterson, NJ, USA.

出版信息

J Med Cases. 2025 Jan;16(1):17-22. doi: 10.14740/jmc4311. Epub 2024 Dec 21.

Abstract

Heyde syndrome is a triad of aortic stenosis (AS), gastrointestinal (GI) bleeding from angiodysplasia, and acquired von Willebrand disease (vWD). It is hypothesized that stenotic aortic valves cleave von Willebrand factor (vWF) multimers, predisposing patients to bleeding from GI angiodysplasias. This hypothesis is supported by the observation that aortic valve replacement often leads to the resolution of GI bleeding. Heyde syndrome is typically described in the context of AS and small bowel angiodysplasias (Dieulafoy's lesion, intestinal vascular malformation, and arteriovenous malformations). However, data on AS and gastric antral vascular ectasia (GAVE) association are scarce. GAVE is a vascular anomaly characterized by ectatic capillaries, arterioles, and venules, which can lead to upper GI bleeding. The paucity of data on GAVE-AS association may lead to underdiagnosis and/or under-reporting. Herein, we describe two cases of GAVE-AS that were diagnosed and treated at our institution. This case series focuses on patient presentations and clinical outcomes and aims to raise awareness about this rare association.

摘要

海德综合征是一种三联征,包括主动脉瓣狭窄(AS)、血管发育异常引起的胃肠道(GI)出血以及获得性血管性血友病(vWD)。据推测,狭窄的主动脉瓣会切割血管性血友病因子(vWF)多聚体,使患者易发生胃肠道血管发育异常出血。主动脉瓣置换术常常能使胃肠道出血得到缓解这一观察结果支持了这一假说。海德综合征通常是在主动脉瓣狭窄和小肠血管发育异常(迪厄拉富瓦病、肠道血管畸形和动静脉畸形)的背景下描述的。然而,关于主动脉瓣狭窄与胃窦血管扩张症(GAVE)关联的数据很少。胃窦血管扩张症是一种血管异常,其特征为毛细血管、小动脉和小静脉扩张,可导致上消化道出血。关于胃窦血管扩张症与主动脉瓣狭窄关联的数据匮乏可能导致诊断不足和/或报告不足。在此,我们描述了在我们机构诊断和治疗的两例胃窦血管扩张症合并主动脉瓣狭窄病例。本病例系列重点关注患者表现和临床结局,旨在提高对这种罕见关联的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e3c0/11699859/eb8ba0a07273/jmc-16-017-g001.jpg

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