PMID- 27143258
OWN - NLM
STAT- MEDLINE
DCOM- 20170802
LR  - 20220330
IS  - 1528-0020 (Electronic)
IS  - 0006-4971 (Print)
IS  - 0006-4971 (Linking)
VI  - 127
IP  - 25
DP  - 2016 Jun 23
TI  - Acquired von Willebrand syndrome associated with left ventricular assist device.
PG  - 3133-41
LID - 10.1182/blood-2015-10-636480 [doi]
AB  - Left ventricular assist devices (LVAD) provide cardiac support for patients with 
      end-stage heart disease as either bridge or destination therapy, and have 
      significantly improved the survival of these patients. Whereas earlier models 
      were designed to mimic the human heart by producing a pulsatile flow in parallel 
      with the patient's heart, newer devices, which are smaller and more durable, 
      provide continuous blood flow along an axial path using an internal rotor in the 
      blood. However, device-related hemostatic complications remain common and have 
      negatively affected patients' recovery and quality of life. In most patients, the 
      von Willebrand factor (VWF) rapidly loses large multimers and binds poorly to 
      platelets and subendothelial collagen upon LVAD implantation, leading to the term 
      acquired von Willebrand syndrome (AVWS). These changes in VWF structure and 
      adhesive activity recover quickly upon LVAD explantation and are not observed in 
      patients with heart transplant. The VWF defects are believed to be caused by 
      excessive cleavage of large VWF multimers by the metalloprotease ADAMTS-13 in an 
      LVAD-driven circulation. However, evidence that this mechanism could be the 
      primary cause for the loss of large VWF multimers and LVAD-associated bleeding 
      remains circumstantial. This review discusses changes in VWF reactivity found in 
      patients on LVAD support. It specifically focuses on impacts of LVAD-related 
      mechanical stress on VWF structural stability and adhesive reactivity in 
      exploring multiple causes of AVWS and LVAD-associated hemostatic complications.
CI  - (c) 2016 by The American Society of Hematology.
FAU - Nascimbene, Angelo
AU  - Nascimbene A
AD  - Center for Advanced Heart Failure, Health Science Center at Houston, University 
      of Texas, Houston, TX;
FAU - Neelamegham, Sriram
AU  - Neelamegham S
AD  - Department of Chemical and Biological Engineering, University at Buffalo, the 
      State University of New York, Buffalo, NY;
FAU - Frazier, O H
AU  - Frazier OH
AD  - Texas Heart Institute, Baylor College of Medicine, Houston, TX;
FAU - Moake, Joel L
AU  - Moake JL
AD  - Texas Heart Institute, Baylor College of Medicine, Houston, TX; J.W. Cox 
      Laboratory for Biomedical Engineering, Rice University, Houston, TX;
FAU - Dong, Jing-Fei
AU  - Dong JF
AD  - BloodWorks Northwest Research Institute, Seattle, WA; and Division of Hematology, 
      Department of Medicine, School of Medicine, University of Washington, Seattle, 
      WA.
LA  - eng
GR  - R01 HL071895/HL/NHLBI NIH HHS/United States
GR  - R01 HL077258/HL/NHLBI NIH HHS/United States
GR  - R01 HL125957/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Review
DEP - 20160503
PL  - United States
TA  - Blood
JT  - Blood
JID - 7603509
RN  - 0 (von Willebrand Factor)
SB  - IM
MH  - Heart Failure/surgery
MH  - Heart Transplantation/adverse effects/instrumentation
MH  - Heart-Assist Devices/*adverse effects
MH  - Hemostasis/physiology
MH  - Humans
MH  - Thrombosis/etiology
MH  - Ventricular Dysfunction, Left/physiopathology/*surgery
MH  - von Willebrand Diseases/*etiology
MH  - von Willebrand Factor/physiology
PMC - PMC4920020
EDAT- 2016/05/05 06:00
MHDA- 2017/08/03 06:00
PMCR- 2017/06/23
CRDT- 2016/05/05 06:00
PHST- 2015/10/14 00:00 [received]
PHST- 2016/04/24 00:00 [accepted]
PHST- 2016/05/05 06:00 [entrez]
PHST- 2016/05/05 06:00 [pubmed]
PHST- 2017/08/03 06:00 [medline]
PHST- 2017/06/23 00:00 [pmc-release]
AID - S0006-4971(20)34432-3 [pii]
AID - 2015/636480 [pii]
AID - 10.1182/blood-2015-10-636480 [doi]
PST - ppublish
SO  - Blood. 2016 Jun 23;127(25):3133-41. doi: 10.1182/blood-2015-10-636480. Epub 2016 
      May 3.