Hudzik Bartosz, Kaczmarski Jacek, Pacholewicz Jerzy, Zakliczynski Michal, Gasior Mariusz, Zembala Marian
3 Department of Cardiology, SMDZ in Zabrze, Medical University of Silesia in Katowice, Silesian Centre for Heart Disease, Zabrze, Poland.
Haemostasis Laboratory, Silesian Centre for Heart Disease, Zabrze, Poland.
Kardiochir Torakochirurgia Pol. 2015 Sep;12(3):233-7. doi: 10.5114/kitp.2015.54459. Epub 2015 Sep 28.
Mechanical circulatory support (MCS) is an umbrella term describing the various technologies used in both short- and long-term management of patients with either end-stage chronic heart failure (HF) or acute HF. Most often, MCS has emerged as a bridge to transplantation, but more recently it is also used as a destination therapy. Mechanical circulatory support includes left ventricular assist device (LVAD) or bi-ventricular assist device (Bi-VAD). Currently, 2- to 3-year survival in carefully selected patients is much better than with medical therapy. However, MCS therapy is hampered by sometimes life-threatening complications including bleeding and device thrombosis. Von Willebrand factor (vWF) has two major functions in haemostasis. First, it plays a crucial role in platelet-subendothelium adhesion and platelet-platelet interactions (aggregation). Second, it is the carrier of factor VIII (FVIII) in plasma. Von Willebrand factor prolongs FVIII half-time by protecting it from proteolytic degradation. It delivers FVIII to the site of vascular injury thus enhancing haemostatic process. On one hand, high plasma levels of vWF have been associated with an increased risk of thrombosis. On the other, defects or deficiencies of vWF underlie the inherited von Willebrand disease or acquired von Willebrand syndrome. Here we review the pathophysiology of thrombosis and bleeding associated with vWF.
机械循环支持(MCS)是一个概括性术语,描述了用于终末期慢性心力衰竭(HF)或急性HF患者短期和长期管理的各种技术。大多数情况下,MCS已成为移植的桥梁,但最近它也被用作终末期治疗。机械循环支持包括左心室辅助装置(LVAD)或双心室辅助装置(Bi-VAD)。目前,精心挑选的患者2至3年生存率比药物治疗要好得多。然而,MCS治疗有时会受到包括出血和装置血栓形成等危及生命的并发症的阻碍。血管性血友病因子(vWF)在止血过程中有两个主要功能。首先,它在血小板与内皮下的黏附以及血小板与血小板之间的相互作用(聚集)中起关键作用。其次,它是血浆中凝血因子VIII(FVIII)的载体。血管性血友病因子通过保护FVIII免受蛋白水解降解来延长其半衰期。它将FVIII输送到血管损伤部位,从而增强止血过程。一方面,血浆中vWF水平升高与血栓形成风险增加有关。另一方面,vWF的缺陷或缺乏是遗传性血管性血友病或获得性血管性血友病综合征的基础。在此,我们综述与vWF相关的血栓形成和出血的病理生理学。