Diagnosis and Management of Acquired von Willebrand Disease in Heart Disease: A Review of the Literature

Petričević, Mate; Knezević, J; Samoukovic, Gordan; Bradarić, Božena; Šafradin, Ivica; Meštrović, Marija; Papestiev, Vasil; Hodalin, Alen; Madžar, Tomislav; Mihalj, Mario; Rotim, Ante; Biočina, Bojan

doi:10.1055/s-0038-1673670

Cited by 6 publications

(5 citation statements)

References 73 publications

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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%

“…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%

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Hemostatic abnormalities in adult patients with Marfan syndrome

Kornhuber¹,

Seidel²,

Pujol³

et al. 2019

Cardiovasc. Diagn. Ther.

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Background: Aortic root ectasia might induce hemostatic disorders in patients with Marfan syndrome (MFS) via altered blood flow and rheology. The aim of this study was to explore the hemostasis in patients with MFS compared with healthy controls.Methods: In this cross-sectional case-control study we included patients with verified MFS (n=51) and sexand age-matched healthy controls (n=50). Main criteria were the aortic root in echocardiography and cardiac magnetic resonance imaging (MRI), and the coagulation status.Results: When compared with healthy controls, patients with MFS showed significantly increased diameters of the aortic roots (43.0±7.72 vs. 28.8±3.74 mm, P<0.001) and aortic Z-scores (4.36±2.77 vs. 0.948±1.09, P<0.001), considerably higher values of Multiplate ® tests (e.g., MP-ADP: 878.4±201.7 vs.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Hemostatic abnormalities in adult patients with Marfan syndrome

Kornhuber¹,

Seidel²,

Pujol³

et al. 2019

Cardiovasc. Diagn. Ther.

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“…20 In lesions that result in high shear stress, such as severe aortic stenosis, there is increased cleavage of HMW multimers by VWF cleaving protease ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) and possible mechanical destruction of VWF itself. 21 There is limited evidence regarding how to manage AVWS in the perioperative setting. The efficacy of desmopressin (1-desamino-8-d-arginine vasopressin [DDAVP]) is questionable in this situation.…”

Section: Bleeding Disordersmentioning

confidence: 99%

Hemostatic Management of Extracorporeal Circuits Including Cardiopulmonary Bypass and Extracorporeal Membrane Oxygenation

Fang

Navaei

Hensch

et al. 2019

Semin Thromb Hemost

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Cardiopulmonary bypass and extracorporeal membrane oxygenation (ECMO) cause hemostatic derangements that can predispose patients to both bleeding and thrombotic complications. Often, patients present for urgent surgery while taking medications including antiplatelet agents, vitamin K antagonists, and direct oral anticoagulants, which must be recognized, monitored, and managed. During extracorporeal circulation, appropriate anticoagulation, most commonly with heparin, is required to maintain blood flow and avoid thrombotic complications. However, anticoagulation and other effects of extracorporeal circuits can also have an undesired consequence of bleeding. Extracorporeal circulation leads to coagulopathy that may require therapy with blood products such as platelets, cryoprecipitate, and plasma in case a patient bleeds. Platelet dysfunction related to exposure to a foreign circuit is a primary concern, as is the development of acquired von Willebrand syndrome, which frequently remains undetected on routine testing. Hemorrhagic complications in ECMO, such as intracranial hemorrhage, pulmonary hemorrhage, and hemithorax, can occur. Hemostatic agents including antifibrinolytics, desmopressin, fibrinogen concentrates, and other factor concentrates may be needed to achieve hemostasis in these often-challenging patients. Managing bleeding on extracorporeal support requires careful monitoring and a thoughtful approach.

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“…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%

Reappraising the spectrum of bleeding gastrointestinal angioectasia in a degenerative calcific aortic valve stenosis: Heyde’s syndrome

2021

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Background The occurrence of bleeding gastrointestinal angioectasia in elderly patients with degenerative calcific aortic stenosis is one of the most challenging clinical scenarios. A number of studies have shown that this clinical phenomenon is known as Heyde’s syndrome. Main body of the abstract The pathogenesis of Heyde’s syndrome is mainly due to the loss of high-molecular-weight von Willebrand factor (HMW vWF) multimers, as a consequent fragmentation of HMW vWF multimers as they pass through the stenosed aortic valve leading to acquired von Willebrand syndrome type IIA. Aortic valve replacement has proven to be a more effective management approach in the cessation of recurrent episodes of gastrointestinal bleeding. Short conclusion Physicians should have a high index of suspicion when dealing with elderly patients with established aortic stenosis presenting with iron deficiency anemia or unclear gastrointestinal bleeding. Parallel consultations between different specialties are essential for appropriate management.

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Diagnosis and Management of Acquired von Willebrand Disease in Heart Disease: A Review of the Literature

Cited by 6 publications

References 73 publications

Hemostatic abnormalities in adult patients with Marfan syndrome

Hemostatic abnormalities in adult patients with Marfan syndrome

Hemostatic Management of Extracorporeal Circuits Including Cardiopulmonary Bypass and Extracorporeal Membrane Oxygenation

Reappraising the spectrum of bleeding gastrointestinal angioectasia in a degenerative calcific aortic valve stenosis: Heyde’s syndrome

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