Work

Hemodynamic activation of von Willebrand factor in children with congenital heart diseases

Year: 2024

Type: article

Abstract: Quite complex and non-linear cellular and molecular mechanisms underlie hemostasis changes in patients with congenital heart diseases (CHD). Altered activity ratio between von Willebrand factor (vWF) and metalloproteinase ADAMTS-13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13) is of pathogenetic significance. The high rate of vWF exocytosis, emergence of its multimeric soluble plasma forms and high rate of proteolysis to small counterparts along with ADAMTS-13 consumption lead to a affected the vWF/ADAMTS-13 axis resulting in hemostasis-related prothrombogenic potential. vWF activity depends on the hydrodynamic characteristics in intravascular blood flow. The magnitude of shear stress in CHD promotes a high rate of conformational changes in vWF multimer, thereby suggesting that hemodynamic conditions may determine developing hemostasis alterations.

Source: Obstetrics Gynecology and Reproduction

DOI: 10.17749/2313-7347/ob.gyn.rep.2024.502

Authors: K. A. Tokmakova, D. N. Shilkin, A. A. Kupryashov

Institutions: Bakulev Scientific Center for Cardiovascular Surgery Russian Academy of Medical Sciences, Ministry of Health of the Russian Federation

Language: English

Topic: Platelet Disorders and Treatments

Subfield: Hematology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $142