Von Willebrand factor revisited

Abstract

Degenerative aortic valve stenosis is the most common valvular heart disease seen in developed countries. Since the implementation of transcatheter aortic valve replacement, there has been a surge of efforts to improve procedural technique as well as reduce mortality. Until recently, the mainstay method of assessment of valve implantation as well as paravalvular regurgitation has relied heavily on imaging techniques including transesophageal echocardiography and aortic root angiography. There has been a lot of scientific inquiry to identify biomarkers in the management of patients with aortic stenosis. von Willebrand factor activity and high molecular weight multimer level assessment has emerged as an excellent biomarker with intraoperative, point of care potential. These implications can both supplement imaging modalities as well as potentially serve as a more accurate assessment TAVR complications.

The Vascular Endothelium and Coagulation: Homeostasis, Disease, and Treatment, with a Focus on the Von Willebrand Factor and Factors VIII and V

“…vWF ( Figure 3 ) is a multimeric protein that is part of the subendothelial matrix of endothelial cells, where it is secreted by the Weibel–Palade bodies [ 136 ]. During primary hemostasis, it binds to the platelets and circulates in the bloodstream, where it forms a complex with (and stabilizes) FVIII [ 137 , 138 , 139 ].…”

“…The gene that codes for vWF is located in region p13.2 of chromosome 12 (12p13.2) and consists of a total of 52 exons, measuring around 175 kb in total [ 137 ]. This factor is synthetized mainly in the endoplasmic reticulum of endothelial cells and megakaryocytes as a pre-protein formed by a signaling peptide, a propeptide, and a mature protein monomer comprising 2813 amino acids [ 140 , 141 ].…”

Structurally Robust and Functionally Highly Versatile—C-Type Lectin (-Related) Proteins in Snake Venoms

“…Platelets are easily accessible and tangible targets for snake venoms [19,60,61,70,98,99]. They express receptors on their surface, which bind to extracellular matrix molecules, such as von Willebrand factor (vWF), collagen, and fibrin, which becomes accessible or available during vessel damage and coagulation, respectively [71,99,100,101,102]. vWF and collagen especially provide signals to the platelets.…”

Shear Stress-Induced Activation of von Willebrand Factor and Cardiovascular Pathology

“…The quantitative deficiency of HMWM of vWF leads to the acquired von Willebrand syndrome type 2A. Its clinical manifestation in patients with heart valve disease is called Heyde’s syndrome [ 53 ]. It is characterized by cryptogenic gastrointestinal bleedings from submucous arterial malformations, developing together with the progression of aortic stenosis and its cardiovascular symptoms (such as fatigue and angina).…”

“…It is characterized by cryptogenic gastrointestinal bleedings from submucous arterial malformations, developing together with the progression of aortic stenosis and its cardiovascular symptoms (such as fatigue and angina). Bleeding from arterial malformations is thought to be provoked by increased shear rates, due to the complex structure of vessels, which cause further consumption of vWF [ 53 ]. Patients with severe aortic stenosis also report skin and mucosal bleeding.…”