Petricevic Mate, Knezevic Jadranka, Samoukovic Gordan, Bradaric Bozena, Safradin Ivica, Mestrovic Marija, Papestiev Vasil, Hodalin Alen, Madzar Tomislav, Mihalj Mario, Rotim Ante, Biocina Bojan
Department of Cardiac Surgery, University of Zagreb School of Medicine, University Hospital Center Zagreb, Zagreb, Croatia.
Department of Transfusion Medicine, University Hospital Center Mostar, Mostar, Bosnia and Herzegovina.
Thorac Cardiovasc Surg. 2020 Apr;68(3):200-211. doi: 10.1055/s-0038-1673670. Epub 2018 Nov 20.
The incidence of acquired von Willebrand syndrome (AvWS) in patients with heart disease is commonly perceived as rare. However, its occurrence is underestimated and underdiagnosed, potentially leading to inadequate treatment resulting in increased morbidity and mortality.In patients with cardiac disease, AvWS frequently occurs in patients with structural heart disease and in those undergoing mechanical circulatory support (MCS).The clinical manifestation of an AvWS is usually characterized by apparent or occult gastrointestinal (GI) or mucocutaneous hemorrhage frequently accompanied by signs of anemia and/or increased bleeding during surgical procedures. The primary change is loss of high-molecular weight von Willebrand factor multimers (HMWM). Whereas the loss of HMWM in patients with structural heart disease is caused by increased HMWM cleavage by von Willebrand factor (vWF)-cleaving protease, ADAMTS13, AvWS in MCS patients is predominantly a result of a high shear stress coupled with mechanical destruction of vWF itself.This manuscript provides a comprehensive review of the evidence regarding both diagnosis and contemporary management of AVWS in patients with heart disease.
心脏病患者获得性血管性血友病综合征（AvWS）的发病率通常被认为很低。然而，其发生率被低估且诊断不足，这可能导致治疗不当，从而增加发病率和死亡率。在心脏病患者中，AvWS常见于患有结构性心脏病的患者以及接受机械循环支持（MCS）的患者。AvWS的临床表现通常以明显或隐匿的胃肠道（GI）或皮肤黏膜出血为特征，常伴有贫血迹象和/或手术过程中出血增加。主要变化是高分子量血管性血友病因子多聚体（HMWM）的丢失。在结构性心脏病患者中，HMWM的丢失是由血管性血友病因子（vWF）裂解蛋白酶ADAMTS13对HMWM的裂解增加所致，而MCS患者的AvWS主要是高剪切应力与vWF本身的机械破坏共同作用的结果。本文对心脏病患者AvWS的诊断和现代治疗的相关证据进行了全面综述。
