John A. Burns School of Medicine, University of Hawai'i at Mānoa, Honolulu, Hawaii, USA
John A. Burns School of Medicine, University of Hawai'i at Mānoa, Honolulu, Hawaii, USA.
BMJ Open Gastroenterol. 2022 May;9(1). doi: 10.1136/bmjgast-2021-000866.
Heyde's syndrome (HS), a rare condition characterised by a unique relationship between severe aortic stenosis and angiodysplasia, is often diagnosed late increasing the risk for a prolonged hospital course and mortality in the elderly. The leading hypothesis explaining the aetiology of HS is acquired von Willebrand syndrome (AVWS) but not all studies support this claim. While individual cases of HS have been reported, here we present the first systematic review of case reports and focus on the prevalence of AVWS.
A systematic search was conducted through PubMed/MEDLINE, CINAHL-EBSCO, Web of Science and Google Scholar since inception. The resulting articles were screened by two independent reviewers based on inclusion criteria that the article must be a case report/series or a letter to the editor in English describing HS in an adult patient.
Seventy-four articles encompassing 77 cases met the inclusion criteria. The average age was 74.3±9.3 years old with a slight female predominance. The small intestine, especially the jejunum, was the most common location for bleeding origin. Capsule endoscopy and double balloon enteroscopy were superior at identifying bleeding sources than colonoscopy (p=0.0027 and p=0.0095, respectively) and oesophagogastroduodenoscopy (p=0.0006 and p=0.0036, respectively). The mean duration from symptom onset to diagnosis/treatment of HS was 23.8±39 months. Only 27/77 cases provided evidence for AVWS. Surgical and transcutaneous aortic valve replacement (AVR) were superior at preventing rebleeding than non-AVR modalities (p<0.0001).
Further research is warranted for a stronger understanding and increased awareness of HS, which may hasten diagnosis and optimal management.
Heyde 综合征（HS）是一种罕见的疾病，其特征是严重的主动脉瓣狭窄和血管发育不良之间存在独特的关系，这种疾病在老年人中往往诊断较晚，增加了延长住院时间和死亡的风险。解释 HS 病因的主要假说为获得性 von Willebrand 综合征（AVWS），但并非所有研究都支持这一说法。虽然已经报道了个别 HS 病例，但我们在此首次进行了病例报告的系统综述，并重点关注 AVWS 的患病率。
通过 PubMed/MEDLINE、CINAHL-EBSCO、Web of Science 和 Google Scholar 进行了系统搜索，从成立之初开始。根据纳入标准，由两名独立评审员筛选出的文章，这些标准是文章必须是描述成年患者 HS 的病例报告/系列或致编辑的信函，并且必须为英文。
74 篇文章共包含 77 例符合纳入标准的病例。平均年龄为 74.3±9.3 岁，女性略占优势。小肠，尤其是空肠，是出血起源的最常见部位。胶囊内镜和双气囊小肠镜在识别出血来源方面优于结肠镜（p=0.0027 和 p=0.0095）和食管胃十二指肠镜（p=0.0006 和 p=0.0036）。从症状出现到诊断/治疗 HS 的平均时间为 23.8±39 个月。77 例中有 27 例提供了 AVWS 的证据。手术和经皮主动脉瓣置换术（AVR）在预防再出血方面优于非 AVR 治疗方法（p<0.0001）。
需要进一步研究以更好地理解和提高对 HS 的认识，这可能会加速诊断和最佳管理。
