Acquired von Willebrand syndrome associated with left ventricular assist device

Nascimbene, Angelo; Neelamegham, Sriram; Frazier, O.H.; Moake, Joel L.; Dong, Jing

doi:10.1182/blood-2015-10-636480

Cited by 187 publications

(158 citation statements)

References 124 publications

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“…Circulatory assistance devices such as LVADs can also cause loss of HMWM and result in bleeding and angiodysplasia. LVAD bleeding is similar to that observed in Heyde’s syndrome 100,101. In many cases, both VWF:RCo and VWF:Ag levels remain normal 100,102,103.…”

Section: Acquired Vws and Left Ventricular Assist Devices (Lvads)supporting

confidence: 72%

Von Willebrand Disease in the elderly: clinical perspectives

Chapin

2018

CIA

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Von Willebrand disease (VWD) is an inherited bleeding disorder that affects up to 1% of the population. In most cases, VWD results from a mutation in the von Willebrand Factor (VWF) gene, which alters the amount and function of VWF, a key glycoprotein in both primary and secondary hemostasis. A comprehensive analysis of patients with VWD should include VWF activity, antigen levels, platelet function, and a careful bleeding history. Treatment options include antifibrinolytics, desmopressin, and VWF replacement therapy. VWF levels fluctuate due to age, stress, environmental exposures, and pharmacologic treatment. Treatment guidelines exist to treat and prevent bleeding for patients undergoing surgery and medical procedures, but often these must be reevaluated in the setting of age-related comorbidities including cardiovascular events, venous thrombosis, and malignancy. In addition, many age-related complications are associated with a secondary acquired von Willebrand syndrome (AVWS), including malignancies, hypothyroidism, cardiovascular diseases, and cardiac replacement devices. The current literature is limited by a lack of older patients in clinical trials. Larger studies are needed to determine if age-related comorbidities affect VWD patients at different frequencies than the general elderly population. There is also a significant need for registry-based studies to evaluate many age-related comorbidities in VWD patients.

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Section: Acquired Vws and Left Ventricular Assist Devices (Lvads)supporting

confidence: 72%

Von Willebrand Disease in the elderly: clinical perspectives

Chapin

2018

CIA

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“…This may have resulted in greater shear stress and exacerbated von Willebrand factor deficiency. 24 Further investigation into this association may shed more light on the mechanism of increased bleeding risk in obese patients. The present study had several limitations.…”

Section: Discussionmentioning

confidence: 99%

Higher Body Mass Index Increases Risk of HeartMate II Pump Thrombosis But Does Not Adversely Affect Long-Term Survival

Han

Sooppan

Johnson

et al. 2017

Circ J

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Background: Obesity has been correlated with various adverse events in patients who receive left ventricular assist devices (LVAD). In this study, we sought to further characterize the role of obesity in this patient population. Methods and Results:We performed a retrospective analysis of 164 patients implanted with a HeartMate II from August 2008 to December 2014. Patients were categorized into 2 BMI groups based on WHO guidelines: BMI 18.5-30 kg/m 2 (n=99) and BMI >30 kg/m 2 (n=65). Patient demographics, adverse outcome and long-term survival were compared between the 2 groups. For any outcome associated with BMI groups, we performed a Cox regression to identify confounding comorbidities. Preoperative demographics and comorbidities were similar. Patients with BMI >30 were younger (P=0.01) and had a higher incidence of type 2 diabetes (P=0.01). While rate of pump thrombosis was higher among patients with BMI >30 (P=0.02), overall survival at 2 years did not differ. The most common cause of death was hemorrhagic stroke in the obese group. On multivariable cox regression analysis, BMI was an independent risk factor of pump thrombosis. Conclusions:Higher BMI does not reduce survival after VAD implantation but it does appear to increase the risk of pump thrombosis. Further studies to characterize the role of BMI in survival and thrombosis rates are warranted.

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“…27 In addition, more recent data have demonstrated that AVWS is also common in patients with cardiovascular disorders including aortic valve stenosis and those treated with left ventricular assist devices. 28 Management of bleeding complications in AVWS frequently poses a major clinical challenge. Although DDAVP and VWF-containing concentrates can be useful in transiently increasing plasma VWF levels, markedly enhanced clearance of VWF and FVIII in AVWS limits the efficacy of these agents.…”

Section: Clinical Management Of Bleeding Episodes and Adjunctive Thermentioning

confidence: 99%

New treatment approaches to von Willebrand disease

Lavin

O’Donnell

2016

Hematology

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von Willebrand disease (VWD) is the commonest inherited bleeding disorder and results from either a quantitative or qualitative deficiency in the plasma glycoprotein von Willebrand factor (VWF). Recent large cohort studies have significantly enhanced our understanding of the molecular mechanisms involved in the pathogenesis of VWD. In contrast, however, there have been relatively few advances in the therapeutic options available for the treatment of bleeding in patients with VWD. Established treatment options include tranexamic acid, 1-deamino-8-D-arginine vasopressin (DDAVP), and plasma-derived VWF concentrates. In addition, a recombinant VWF has also recently been developed. In this review, we focus on how recent insights into the clinical and molecular aspects underpinning VWD are already beginning to influence treatment in the clinic. For example, a number of different bleeding assessment tools (BATs) have been developed to objectively assess bleeding symptoms in patients with VWD. Interestingly, however, these BAT scores may also have an important role to play in predicting bleeding risk in VWD. Furthermore, recent studies have demonstrated that enhanced VWF clearance plays a critical role in the etiology of both type 1 and type 2 VWD. These findings have direct translational relevance with respect to the use of DDAVP in patients with VWD. As understanding of the mechanisms involved in VWD pathogenesis continues to advance, novel treatment options are likely to emerge. Critically, however, large adequately powered and stratified clinical trials will be required to address the outstanding questions that remain regarding VWD treatment optimization. Learning Objectives• The reader will recognize the various therapeutic options available for the treatment of von Willebrand disease (VWD), including the recently developed recombinant VWF concentrate • The reader will understand how emerging data regarding the molecular pathogenesis underlying VWD are influencing the design of personalized treatment regimens for individual patients with VWD

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Acquired von Willebrand syndrome associated with left ventricular assist device

Cited by 187 publications

References 124 publications

Von Willebrand Disease in the elderly: clinical perspectives

Von Willebrand Disease in the elderly: clinical perspectives

Higher Body Mass Index Increases Risk of HeartMate II Pump Thrombosis But Does Not Adversely Affect Long-Term Survival

New treatment approaches to von Willebrand disease

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