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

Autori
Petričević, Mate ; Knežević, Jadranka ; Samouković, Gordan ; Bradarić, Božena ; Šafradin, Ivica ; Meštrović, Marija ; Papestiev, Vasil ; Hodalin, Alen ; Madžar, Tomislav ; Mihalj, Mario ; Rotim, Ante ; Biočina, Bojan

Izvornik
The thoracic and cardiovascular surgeon (0171-6425) 68 (2020), 03; 200-211

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, pregledni rad, znanstveni

Ključne riječi
cardiac ; cardiopulmonary bypass ; CPB ; hematology ; surgery ; complications ; vascular science

Sažetak
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.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA