title: Acquired von Willebrand syndrome associated with left ventricular assist device.
abstract: 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.
authors.affiliation: Center for Advanced Heart Failure, Health Science Center at Houston, University of Texas, Houston, TX;
authors.forename: Angelo
authors.initials: A
authors.lastname: Nascimbene
authors.affiliation: Department of Chemical and Biological Engineering, University at Buffalo, the State University of New York, Buffalo, NY;
authors.forename: Sriram
authors.initials: S
authors.lastname: Neelamegham
authors.affiliation: Texas Heart Institute, Baylor College of Medicine, Houston, TX;
authors.forename: O H
authors.initials: OH
authors.lastname: Frazier
authors.affiliation: Texas Heart Institute, Baylor College of Medicine, Houston, TX; J.W. Cox Laboratory for Biomedical Engineering, Rice University, Houston, TX;
authors.forename: Joel L
authors.initials: JL
authors.lastname: Moake
authors.affiliation: BloodWorks Northwest Research Institute, Seattle, WA; and Division of Hematology, Department of Medicine, School of Medicine, University of Washington, Seattle, WA.
authors.forename: Jing-Fei
authors.initials: JF
authors.lastname: Dong
mesh_terms: D006333:Heart Failure / Q000601:surgery; D016027:Heart Transplantation / Q000009:adverse effects / Q000295:instrumentation; D006353:Heart-Assist Devices / Q000009:adverse effects*; D006487:Hemostasis / Q000502:physiology; D006801:Humans; D013927:Thrombosis / Q000209:etiology; D018487:Ventricular Dysfunction, Left / Q000503:physiopathology / Q000601:surgery*; D014842:von Willebrand Diseases / Q000209:etiology*; D014841:von Willebrand Factor / Q000502:physiology
chemical_list: D014841:von Willebrand Factor