Shibamoto Akihiko, Kawaratani Hideto, Kubo Takuya, Nishimura Norihisa, Sato Shinya, Seki Kenichiro, Sawada Yasuhiko, Takaya Hiroaki, Okura Yasushi, Takeda Kousuke, Uejima Masakazu, Namisaki Tadashi, Moriya Kei, Mitoro Akira, Yamao Junichi, Yoshiji Hitoshi
Third Department of Internal Medicine, Nara Medical University.
Department of Endoscopy, Nara Medical University.
J Nippon Med Sch. 2017;84(4):193-197. doi: 10.1272/jnms.84.193.
Heyde syndrome describes the triad of aortic stenosis, acquired coagulopathy, and anemia due to bleeding from intestinal angiodysplasia. An 87-year-old man with iron deficiency anemia due to melena was admitted to our hospital. On examination, a systolic murmur was heard and echocardiography confirmed the presence of aortic stenosis. Esophagogastroduodenoscopy and colonoscopy were unremarkable. Capsule endoscopy and double balloon endoscopy revealed angiodysplasia throughout the small intestine. Laboratory investigations were significant for reduced plasma levels of high molecular weight von Willebrand factor multimers. On the basis of these findings, the patient was diagnosed with Heyde syndrome. The patient required frequent blood transfusions because of the intestinal bleeding, and underwent bioprosthetic aortic valve replacement. Twenty months after the operation, the gastrointestinal bleeding resolved and the patient no longer required blood transfusions. This is the first case report to describe an improvement in bleeding from angiodysplasia, one year after aortic valve replacement. It demonstrates the effective treatment of Heyde syndrome with aortic valve replacement, and highlights the importance of considering this differential diagnosis when evaluating patients presenting with repeated episodes of gastrointestinal bleeding and a concurrent systolic murmur.
海德综合征描述了主动脉瓣狭窄、获得性凝血病以及因肠道血管发育异常出血导致的贫血这一三联征。一名因黑便导致缺铁性贫血的87岁男性入住我院。检查时,听到收缩期杂音,超声心动图证实存在主动脉瓣狭窄。食管胃十二指肠镜检查和结肠镜检查均无异常。胶囊内镜和双气囊内镜检查发现整个小肠存在血管发育异常。实验室检查显示高分子量血管性血友病因子多聚体的血浆水平降低。基于这些发现,该患者被诊断为海德综合征。由于肠道出血,患者需要频繁输血,并接受了生物人工主动脉瓣置换术。术后20个月,胃肠道出血得到缓解,患者不再需要输血。这是第一例描述主动脉瓣置换术后一年血管发育异常出血情况改善的病例报告。它证明了主动脉瓣置换术对海德综合征的有效治疗,并强调了在评估反复出现胃肠道出血并伴有收缩期杂音的患者时考虑这一鉴别诊断的重要性。