Thorac Cardiovasc Surg 2020; 68(03): 200-211
DOI: 10.1055/s-0038-1673670
Georg Thieme Verlag KG Stuttgart · New York

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

Mate Petricevic
1   Department of Cardiac Surgery, University of Zagreb School of Medicine, University Hospital Center Zagreb, Zagreb, Croatia
Jadranka Knezevic
2   Department of Transfusion Medicine, University Hospital Center Mostar, Mostar, Bosnia and Herzegovina
Gordan Samoukovic
3   Divisions of Cardiac Surgery and Critical Care, Department of Surgery, McGill University Health Centre, Montreal, Quebec, Canada
Bozena Bradaric
4   Merkur University Hospital, Vuk Vrhovac University Clinic for Diabetes, Endocrinology and Metabolic Disease, Zagreb, Croatia
Ivica Safradin
1   Department of Cardiac Surgery, University of Zagreb School of Medicine, University Hospital Center Zagreb, Zagreb, Croatia
Marija Mestrovic
1   Department of Cardiac Surgery, University of Zagreb School of Medicine, University Hospital Center Zagreb, Zagreb, Croatia
Vasil Papestiev
1   Department of Cardiac Surgery, University of Zagreb School of Medicine, University Hospital Center Zagreb, Zagreb, Croatia
Alen Hodalin
1   Department of Cardiac Surgery, University of Zagreb School of Medicine, University Hospital Center Zagreb, Zagreb, Croatia
Tomislav Madzar
5   University of Osijek School of Medicine, Osijek, Croatia
Mario Mihalj
6   Department of Neurology, University of Split School of Medicine, University Hospital Center Split, Split, Croatia
Ante Rotim
5   University of Osijek School of Medicine, Osijek, Croatia
Bojan Biocina
1   Department of Cardiac Surgery, University of Zagreb School of Medicine, University Hospital Center Zagreb, Zagreb, Croatia
› Author Affiliations
Further Information

Publication History

29 April 2018

05 September 2018

Publication Date:
20 November 2018 (online)


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.

Both authors contributed equally to this work.

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