Central Michigan University, Saginaw, MI, USA.
Samaritan Medical Center, Watertown NY, USA.
J Investig Med High Impact Case Rep. 2021 Jan-Dec;9:2324709621997279. doi: 10.1177/2324709621997279.
Heyde syndrome is a multisystem disorder characterized by the classical triad of aortic stenosis, gastrointestinal (GI) angiodysplasias, and acquired von Willebrand syndrome. GI angiodysplasias, common in older patients, are tortuous, thin-walled blood vessels seen in the mucosa or submucosa of the GI tract and are highly prone to rupture resulting in GI bleeds. In this case report, we describe an elderly female with a past medical history of end-stage renal disease and chronic anemia who presented to the emergency department (ED) with complaints of dark-tarry stools and associated abdominal cramping. Patient reported a history of dark-tarry stools and multiple blood transfusions in the past, secondary to severe anemia. An inpatient echocardiogram was performed, revealing severe aortic stenosis. Additionally, gastroenterology was consulted for esophagogastroduodenoscopy and colonoscopy, which were negative for active bleeding. About a year ago, the patient underwent capsule endoscopy at an outlying facility, which was positive for angiodysplasia. Therefore, due to high clinical suspicion, presence of aortic stenosis, and GI angiodysplasia, a platelet function assay was ordered. It was found to be abnormal, pointing to the presence of acquired von Willebrand syndrome. Hence, a diagnosis of Heyde syndrome was established. The patient gradually improved and was discharged with a follow-up appointment with the cardiologist for a possible transcatheter aortic valve replacement procedure. The patient underwent the procedure without complications, after which she did not report episodes of GI bleeding. In this case report, we discuss the presentation, pathophysiology, diagnostic approach, and management of patients with Heyde syndrome.
海德综合征是一种多系统疾病,其特征为经典三联征,即主动脉瓣狭窄、胃肠道(GI)血管发育不良和获得性血管性血友病。GI 血管发育不良在老年患者中较为常见,是在胃肠道黏膜或黏膜下层可见到迂曲、薄壁的血管,易破裂导致 GI 出血。在本病例报告中,我们描述了一位老年女性患者,既往患有终末期肾病和慢性贫血病史,因黑便伴腹部绞痛就诊于急诊科。患者自述有黑便病史,过去曾因严重贫血多次输血。入院行超声心动图检查,提示严重主动脉瓣狭窄。此外,因怀疑存在出血,还请消化内科会诊行食管胃十二指肠镜和结肠镜检查,结果未见活动性出血。大约一年前,患者在外地医疗机构行胶囊内镜检查,提示存在血管发育不良。因此,鉴于高度临床怀疑、主动脉瓣狭窄和 GI 血管发育不良的存在,进行了血小板功能检测,结果异常,提示存在获得性血管性血友病。因此,诊断为海德综合征。患者逐渐好转,出院后预约心内科行可能的经导管主动脉瓣置换术。患者接受了该手术,无并发症发生,此后未再发生 GI 出血。在本病例报告中,我们讨论了海德综合征患者的临床表现、病理生理学、诊断方法和治疗管理。