期刊
JOURNAL OF ATHEROSCLEROSIS AND THROMBOSIS
卷 26, 期 4, 页码 303-314出版社
JAPAN ATHEROSCLEROSIS SOC
DOI: 10.5551/jat.RV17031
关键词
Aortic stenosis; Heyde's syndrome; von Willebrand factor; Acquired von Willebrand's syndrome; ECMO; Left ventricular assist device
资金
- Ministry of Health, Labor and Welfare, Japan
- AMED [JP18ek0109370h0001, JP18ek0109256h0002, JP18ek0109246h0002]
- SENSHIN Medical Research Foundation
- Suzuken Memorial Foundation
- Cooperative Research Project Program of Joint Usage/Research Center at the Institute of Development, Aging and Cancer, Tohoku University
- Japanese Society of Thrombosis and Hemostasis
The blood glycoprotein von Willebrand factor (VWF) plays an important role in hemostasis and thrombosis. VWF is produced and secreted as large multimers by endothelial cells and megakaryocytes. It is then cleaved in a sheer-stress dependent manner by a specific protease, ADAMTS13, into multimers consisting of 2-80 sub-units. Among VWF multimers, high molecular weight (HMW) multimers play important roles in platelet aggregation. Therefore, their loss induces a hemostatic disorder known as von Willebrand disease (VWD) type 2A. Various cardiovascular diseases, such as aortic stenosis, hypertrophic obstructive cardiomyopathy (HOCM), and several congenital structural diseases, as well as mechanical circulatory support systems, generate excessive high shear stress in the bloodstream. These cause excessive cleavage of VWF multimers resulting in a loss of HMW multimers, known as acquired von Willebrand syndrome (AVWS), a hemostatic disorder similar to VWD type 2A. Bleeding often occurs in the gastrointestinal tract since a fragile angiodysplasia develops associated with these diseases. Radical treatment for AVWS is to remove the pathological high shear causing AVWS.
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