akusherstvoObstetrics, Gynecology and ReproductionАкушерство, Гинекология и Репродукция2313-73472500-3194IRBIS LLC10.17749/2313-7347/ob.gyn.rep.2024.502akusherstvo-2231Research ArticleREVIEW ARTICLESНАУЧНЫЕ ОБЗОРЫHemodynamic activation of von Willebrand factor in children with congenital heart diseasesГемодинамическая активация фактора фон Виллебранда у детей с врожденными пороками сердцаhttps://orcid.org/0000-0001-5874-1803ТокмаковаК. А.TokmakovaK. A.

Токмакова Ксения Александровна, к.м.н.

121552 Москва, Рублевское шоссе, д. 135

Kseniya A. Tokmakova, MD, PhD

135 Rublevskoe Shosse, Moscow 121552

TokmakovaKA@yandex.ruhttps://orcid.org/0000-0001-5270-6820ШилкинД. Н.ShilkinD. N.

Шилкин Дмитрий Николаевич

121552 Москва, Рублевское шоссе, д. 135

Dmitriy N. Shilkin, MD

135 Rublevskoe Shosse, Moscow 121552

https://orcid.org/0000-0001-7673-4762КупряшовА. А.KupryashovA. A.

Купряшов Алексей Анатольевич, д.м.н. 

121552 Москва, Рублевское шоссе, д. 135

Aleksey A. Kupryashov, MD, Dr Med Sci

135 Rublevskoe Shosse, Moscow 121552

ФГБУ «Национальный медицинский исследовательский центр сердечно-сосудистой хирургии имени А.Н. Бакулева» Министерства здравоохранения Российской ФедерацииРоссияBakulev National Medical Research Center for Cardiovascular Surgery, Health Ministry of Russian FederationRussian Federation202405112024185693–705Copyright © Tokmakova K.A., Shilkin D.N., Kupryashov A.A., 20242024Токмакова К.А., Шилкин Д.Н., Купряшов А.А.Tokmakova K.A., Shilkin D.N., Kupryashov A.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.gynecology.su/jour/article/view/2231

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.

Клеточно-молекулярные механизмы изменений системы гемостаза у пациентов с врожденными пороками сердца (ВПС) достаточно сложны и нелинейны. Патогенетическое значение имеет нарушение соотношения активности фактора фон Виллебранда (англ. von Willebrand factor, vWF) и металлопротеиназы ADAMTS-13 (англ. a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13). Высокая скорость экзоцитоза vWF, появление его мультимерных растворимых форм в плазме, высокая скорость протеолиза данного гликопротеина до малых форм с потреблением металлопротеиназы ADAMTS-13 приводят к изменению оси vWF/ADAMTS-13, обеспечивая протромбогенный потенциал системы гемостаза. Активность vWF зависит от гидродинамических характеристик внутрисосудистого кровотока. Величина напряжения сдвига при ВПС способствует высокой скорости конформационных изменений мультимера vWF; таким образом, гемодинамические условия способны предопределять наличие изменений системы гемостаза.

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The authors declare that there are no conflicts of interest present.