2018
DOI: 10.1055/s-0038-1673670
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Abstract: 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 occul… Show more

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Cited by 4 publications
(5 citation statements)
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References 72 publications
(138 reference statements)
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“…This syndrome is characterized by a qualitative and quantitative defect of large VWF multimers followed by a dysfunctional interaction with platelets (19,20). It may be induced by increased shear stress caused by the ectatic aortic root in MFS patients (9) which gives rise to a conformational change of the VWF antigens and results in an increased susceptibility to proteases (ADAMTS13) (10,11,(21)(22)(23)(24).…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…This syndrome is characterized by a qualitative and quantitative defect of large VWF multimers followed by a dysfunctional interaction with platelets (19,20). It may be induced by increased shear stress caused by the ectatic aortic root in MFS patients (9) which gives rise to a conformational change of the VWF antigens and results in an increased susceptibility to proteases (ADAMTS13) (10,11,(21)(22)(23)(24).…”
Section: Discussionmentioning
confidence: 99%
“…In order to confirm our theory, further investigations will be necessary. A standardized, universally accepted diagnostic criteria for acquired von Willebrand disease is still lacking (24) and there are different opinions about the sensitivity of the laboratory tests (33)(34)(35)(36). Another point which may add greatly to this field will be the development of further laboratory tests to differentiate between different subtypes of the von Willebrand syndromes.…”
Section: Discussionmentioning
confidence: 99%
“…A decreased VWF:RCo/VWF: Ag is indicative of either absence or lack of highmolecular-weight multimer levels. Therefore, a confirmatory test of VWF multimer distribution should be performed using gel electrophoresis [42,43].…”
Section: Diagnosismentioning
confidence: 99%
“…Other studies show that an increased shear stress affects the von Willebrand factor, which becomes more susceptible to proteases, resulting in a dysfunctional interaction with platelets [365][366][367]. Such effects have been described in patients with acquired von Willebrand syndrome type 2a [366,368] and could also be found in patients with aortic valve stenosis [369][370][371] or dysfunctional heart valve prostheses [372].…”
Section: Acquired Von Willebrand Disease Type 2amentioning
confidence: 99%