Kasai Mio, Osako Motohiko, Inaba Yu, Yamabe Kentaro, Aoki Michiko
Department of Cardiovascular Surgery, National Hospital Organization Tokyo Medical Center, Meguro, Tokyo, Japan.
J Card Surg. 2020 Sep;35(9):2396-2398. doi: 10.1111/jocs.14796. Epub 2020 Jul 11.
The association of aortic valve stenosis with gastrointestinal bleeding was first described by Edward Heyde in 1958. Since then, there have been numerous case reports of Heyde syndrome in the medical literature worldwide.
Recently, the definition of Heyde syndrome has been updated to include the combination of aortic valve stenosis, intestinal angiodysplasia, and acquired von Willebrand factor syndrome (AVWS). However, an association between aortic or mitral regurgitation and AVWS is not well established.
MATERIALS & METHODS: The present case of a patient with endocarditis-associated severe aortic regurgitation and mitral regurgitation exhibited a clinically significant bleeding diathesis secondary to AVWS.
After surgical valve repair, the von Willebrand factor (VWF) activity spontaneously normalized.
AVWS secondary to cardiovascular diseases occurs from a selective loss of the largest multimers of VWF due to high shear forces in the blood circulation. Although it is established that stenotic valvular lesions are associated with AVWS, there have only been rare reports of regurgitant lesions leading to AVWS. We successfully treated this patient with perioperative supplementation of VWF and factor VIII.
主动脉瓣狭窄与胃肠道出血的关联最早由爱德华·海德于1958年描述。从那时起，全球医学文献中有大量关于海德综合征的病例报告。
最近，海德综合征的定义已更新，包括主动脉瓣狭窄、肠道血管发育异常和获得性血管性血友病因子综合征（AVWS）的组合。然而，主动脉瓣或二尖瓣反流与AVWS之间的关联尚未明确确立。
本病例为一名患有心内膜炎相关严重主动脉瓣反流和二尖瓣反流的患者，表现出继发于AVWS的具有临床意义的出血素质。
手术瓣膜修复后，血管性血友病因子（VWF）活性自发恢复正常。
心血管疾病继发的AVWS是由于血液循环中的高剪切力导致VWF最大多聚体的选择性丢失。虽然已确定狭窄性瓣膜病变与AVWS有关，但仅有罕见的关于反流性病变导致AVWS的报告。我们通过围手术期补充VWF和凝血因子VIII成功治疗了该患者。
