Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine.
Department of Molecular and Cellular Biology, Institute of Development, Aging, and Cancer, Tohoku University.
J Atheroscler Thromb. 2020 Mar 1;27(3):271-277. doi: 10.5551/jat.49239. Epub 2019 Aug 3.
An 83-year-old woman with severe aortic stenosis was admitted to our hospital due to heart failure with refractory anemia requiring blood transfusions. She had repetitive bleeding episodes from endoscopically proven angiodysplasia in the stomach. Moreover, she repeatedly underwent endoscopic argon plasma coagulation for hemostasis. Importantly, she had a deficiency of the high-molecular-weight (HMW) multimers of von Willebrand factor (VWF), and she was diagnosed with Heyde's syndrome.After she underwent transcatheter aortic valve implantation (TAVI), aortic valve area and mean left ventricular aorta pressure gradient improved. Notably, endoscopy showed cessation of bleeding at 10 days after TAVI and the disappearance of angiodysplasia at 4 months after TAVI. Even at 2 years after TAVI, follow-up endoscopy showed remaining free of angiodysplasia in the stomach. She experienced no episodes of anemia since TAVI procedure. Additionally, analysis of HMW multimers demonstrated immediate and lasting recovery after TAVI.Recovery of HMW multimers of VWF with cessation of gastrointestinal bleeding following aortic valve replacement has been previously reported in a patient diagnosed with Heyde's syndrome. To the best our knowledge, this is the first case to demonstrate that angiodysplasia disappears after TAVI for a long term with endoscopic images in a patient with Heyde's syndrome. Here, we summarized case reports of patients with Heyde's syndrome that required aortic valve intervention. Cessation of gastrointestinal bleeding and anemia after aortic valve intervention for severe aortic stenosis may be attributed not only to recovery of HMW multimers of VWF but also to the disappearance of angiodysplasia.
一位 83 岁的老年女性因严重的主动脉瓣狭窄导致心力衰竭合并难治性贫血而需要输血入院。她因内镜证实的胃血管扩张症反复发生出血事件。此外,她曾多次因止血而行内镜氩等离子凝固术。重要的是,她存在高分子量(HMW)von Willebrand 因子(VWF)多聚体缺乏,并被诊断为 Heyde 综合征。在接受经导管主动脉瓣置换术(TAVI)后,主动脉瓣面积和平均左心室主动脉压力梯度改善。值得注意的是,内镜检查显示 TAVI 后 10 天出血停止,TAVI 后 4 个月血管扩张症消失。即使在 TAVI 后 2 年,随访内镜检查仍显示胃内无血管扩张症。自 TAVI 手术后,她未再发生贫血。此外,HMW 多聚体分析表明 TAVI 后立即和持续恢复。
先前有报道称,在诊断为 Heyde 综合征的患者中,主动脉瓣置换术后 VWF 的 HMW 多聚体恢复和胃肠道出血停止。据我们所知,这是首例在 Heyde 综合征患者中通过内镜图像显示 TAVI 后长期血管扩张症消失的病例。在这里,我们总结了需要主动脉瓣干预的 Heyde 综合征患者的病例报告。严重主动脉瓣狭窄的主动脉瓣介入治疗后胃肠道出血和贫血的停止可能不仅归因于 VWF 的 HMW 多聚体的恢复,还归因于血管扩张症的消失。