Hemodynamic activation of von Willebrand factor in children with congenital heart diseases
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 moti...
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| Main Authors: | , , |
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| Format: | Article |
| Language: | Russian |
| Published: |
IRBIS LLC
2024-11-01
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| Series: | Акушерство, гинекология и репродукция |
| Subjects: | |
| Online Access: | https://www.gynecology.su/jour/article/view/2231 |
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| Summary: | 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. |
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| ISSN: | 2313-7347 2500-3194 |