Year: 2020
Type: Article
Title: Diagnosis and Management of Acquired von Willebrand Disease in Heart Disease: A Review of the Literature
Author: Mate Petricevic
Author: Jadranka Knezevic
Author: Gordan Samoukovic
Author: Bozena Bradaric
Author: Ivica Safradin
Author: Marija Mestrovic
Author: Vasil Papestiev
Author: Alen Hodalin
Author: Tomislav Madzar
Author: Mario Mihalj
Author: Ante Rotim
Author: Bojan Biocina
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 occult gastrointestinal (GI) or mucocutaneous hemorrhage frequently accompanied by signs of anemia and/or increased bleeding during surgical procedures. The primary change is loss of high-molecular weight von Willebrand factor multimers (HMWM). Whereas the loss of HMWM in patients with structural heart disease is caused by increased HMWM cleavage by von Willebrand factor (vWF)-cleaving protease, ADAMTS13, AvWS in MCS patients is predominantly a result of a high shear stress coupled with mechanical destruction of vWF itself.This manuscript provides a comprehensive review of the evidence regarding both diagnosis and contemporary management of AVWS in patients with heart disease.
Publisher: Thieme
Relation: The Thoracic and Cardiovascular Surgeon
Identifier: oai:repository.ukim.mk:20.500.12188/11134
Identifier: http://hdl.handle.net/20.500.12188/11134Identifier: 10.1055/s-0038-1673670
