Heilmann Claudia, Trummer Georg, Beyersdorf Friedhelm, Brehm Kerstin, Berchtold-Herz Michael, Schelling Johannes, Geisen Ulrich, Zieger Barbara
Department of Cardiovascular Surgery, University Heart Center Freiburg-Bad Krozingen, Freiburg, Germany.
Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Eur J Cardiothorac Surg. 2017 Mar 1;51(3):587-590. doi: 10.1093/ejcts/ezw348.
Impaired binding of Von Willebrand factor (VWF) to platelets and to collagen due to acquired Von Willebrand syndrome (AVWS) is associated with support from a ventricular assist device (VAD) and can contribute to bleeding tendencies in patients with VADs. The onset of AVWS has been shown to occur immediately after VAD implantation. Our aim was to determine long-term data on AVWS in VAD patients.
We analysed 278 data sets of 74 patients on HeartMate II (HMII) support for 3-80 months after implantation (11.2 ± 12.1, median 6.3 months.). Ristocetin cofactor activity (VWF:RCo), collagen binding capacity (VWF:CB), VWF antigen (VWF:Ag) and the ratios of VWF:RCo/VWF:Ag and VWF:CB/VWF:Ag were determined. Further, the presence of high molecular weight (HMW) multimers of VWF was investigated.
Abnormally low values of VWF:RCo/VWF:Ag and VWF:CB/VWF:Ag were found in 69% and 97% of blood samples, respectively. Only ten of 181 multimer analyses showed a normal pattern, and HMW multimers were present in both specimens in only one of the 74 patients. No significant changes in these parameters were observed over time. The VWF:CB/VWF:Ag ratio correlated with the multimer patterns, whereas the VWF:RCo/VWF:Ag ratio seemed to be less sensitive for AVWS.
Our data indicate that AVWS is a typical phenomenon in patients with VAD support and that there are no time-dependent changes in these parameters apparent in most patients on long-term support with HMII.
获得性血管性血友病综合征（AVWS）导致血管性血友病因子（VWF）与血小板及胶原蛋白的结合受损，这与心室辅助装置（VAD）的支持有关，并可导致VAD患者出现出血倾向。已证明AVWS在VAD植入后立即发病。我们的目的是确定VAD患者AVWS的长期数据。
我们分析了74例接受HeartMate II（HMII）支持的患者在植入后3至80个月（11.2±12.1，中位数6.3个月）的278个数据集。测定了瑞斯托霉素辅因子活性（VWF:RCo）、胶原蛋白结合能力（VWF:CB）、VWF抗原（VWF:Ag）以及VWF:RCo/VWF:Ag和VWF:CB/VWF:Ag的比值。此外，还研究了VWF高分子量（HMW）多聚体的存在情况。
分别在69%和97%的血样中发现VWF:RCo/VWF:Ag和VWF:CB/VWF:Ag值异常低。在181次多聚体分析中，只有10次显示正常模式，74例患者中只有1例的两个样本中均存在HMW多聚体。随着时间的推移，这些参数未观察到显著变化。VWF:CB/VWF:Ag比值与多聚体模式相关，而VWF:RCo/VWF:Ag比值对AVWS似乎不太敏感。
我们的数据表明，AVWS是接受VAD支持患者的典型现象，在大多数接受HMII长期支持的患者中，这些参数没有明显的时间依赖性变化。
