Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
Unit of Laboratory Medicine, Policlinico Tor Vergata, Rome, Italy.
Folia Biol (Praha). 2020;66(4):133-141. doi: 10.14712/fb2020066040133.
Von Willebrand disease is a commonly inherited bleeding disorder caused by defects of von Willebrand factor (vWF). In the most common valve diseases, aortic valve stenosis (AVS) and mitral valve regurgitation (MVR), a bleeding tendency has been described in a number of patients. This has been associated to a high turbulence of blood flow through the compromised valve, promoting degradation of vWF with loss of high-molecular-weight multimers of vWF (HMWM), leading to an acquired von Willebrand syndrome (AvWS). We analysed three groups of patients, one affected by AVS, treated with transcatheter aortic valve implantation (TAVI), the second group of patients affected by MVR, treated with Mitraclip® mitral valve repair. The third group was represented by patients also affected by AVS, but not eligible for TAVI and treated with standard surgery. A fourth group of patients that underwent percutaneous coronary intervention (PCI) with stenting was used as a control. Our results demonstrated that the level of vWF measured as antigen concentration (vWF:Ag) increases in all cohorts of patients after treatment, while in control PCI patients, no modification of vWF:Ag has been registered. Western blot analysis showed only a quantitative loss of vWF in the pre-treatment time, but without significant HMWM modification. The monitoring of the vWF:Ag concentration, but not the quality of HMWM, can indicate the status of blood flow in the treated patients, thus introducing the possibility of using the vWF antigen detection in monitoring the status of replaced or repaired valves.
血管性血友病是一种常见的遗传性出血性疾病，由血管性血友病因子（vWF）缺陷引起。在最常见的瓣膜病，即主动脉瓣狭窄（AVS）和二尖瓣反流（MVR）中，许多患者都存在出血倾向。这与通过受损瓣膜的血流高湍流有关，促进了 vWF 的降解，导致高分子量多聚体（HMWM）的 vWF 丢失，从而导致获得性血管性血友病综合征（AvWS）。我们分析了三组患者，一组患有 AVS，接受了经导管主动脉瓣植入术（TAVI）治疗，第二组患者患有 MVR，接受了 Mitraclip®二尖瓣修复术治疗。第三组患者也患有 AVS，但不符合 TAVI 治疗标准，接受了标准手术治疗。第四组患者接受了经皮冠状动脉介入治疗（PCI）和支架置入术作为对照。我们的结果表明，所有治疗后的患者的 vWF 水平（vWF：Ag）作为抗原浓度均增加，而在对照 PCI 患者中，vWF：Ag 没有发生变化。Western blot 分析显示，仅在治疗前时间存在 vWF 的定量丢失，但 HMWM 没有明显改变。vWF：Ag 浓度的监测，但不是 HMWM 的质量，可指示治疗患者的血流状态，从而引入了使用 vWF 抗原检测来监测替换或修复瓣膜的状态的可能性。
