Abnormalities in the Von Willebrand-Angiopoietin Axis Contribute to Dysregulated Angiogenesis and Angiodysplasia in Children With a Glenn Circulation

Carlo R. Bartoli; Samson Hennessy-Strahs; Robert D. Dowling; J. William Gaynor; Andrew C. Glatz

doi:10.1016/j.jacbts.2020.12.014

Abnormalities in the Von Willebrand-Angiopoietin Axis Contribute to Dysregulated Angiogenesis and Angiodysplasia in Children With a Glenn Circulation

Carlo R. Bartoli, Samson Hennessy-Strahs, Robert D. Dowling, J. William Gaynor, Andrew C. Glatz

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10 Scopus citations

Abstract

Children with a bidirectional superior cavopulmonary (Glenn) circulation develop angiodysplasia and pulmonary arteriovenous malformations (AVMs). The von Willebrand factor (vWF)–angiopoietin axis plays a major role in AVM formation in multiple diseases. We observed derangements in global angiogenic signaling, vWF metabolism, angiopoietins, and in vitro angiogenesis in children with a Glenn circulation versus controls and within Glenn pulmonary versus systemic circulations. These findings support the novel hypothesis that abnormalities in the vWF-angiopoietin axis may dysregulate angiogenesis and contribute to Glenn pulmonary AVMs. The vWF-angiopoietin axis may be a target to correct angiogenic imbalance in Glenn patients, for whom no targeted therapy exists.

Original language	English
Pages (from-to)	222-235
Number of pages	14
Journal	JACC: Basic to Translational Science
Volume	6
Issue number	3
DOIs	https://doi.org/10.1016/j.jacbts.2020.12.014
State	Published - Mar 2021

Keywords

Glenn
angiogenesis
angiopoietin
arteriovenous malformation
von Willebrand factor

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title = "Abnormalities in the Von Willebrand-Angiopoietin Axis Contribute to Dysregulated Angiogenesis and Angiodysplasia in Children With a Glenn Circulation",

abstract = "Children with a bidirectional superior cavopulmonary (Glenn) circulation develop angiodysplasia and pulmonary arteriovenous malformations (AVMs). The von Willebrand factor (vWF)–angiopoietin axis plays a major role in AVM formation in multiple diseases. We observed derangements in global angiogenic signaling, vWF metabolism, angiopoietins, and in vitro angiogenesis in children with a Glenn circulation versus controls and within Glenn pulmonary versus systemic circulations. These findings support the novel hypothesis that abnormalities in the vWF-angiopoietin axis may dysregulate angiogenesis and contribute to Glenn pulmonary AVMs. The vWF-angiopoietin axis may be a target to correct angiogenic imbalance in Glenn patients, for whom no targeted therapy exists.",

keywords = "Glenn, angiogenesis, angiopoietin, arteriovenous malformation, von Willebrand factor",

author = "Bartoli, {Carlo R.} and Samson Hennessy-Strahs and Dowling, {Robert D.} and Gaynor, {J. William} and Glatz, {Andrew C.}",

note = "Funding Information: This project was performed with support from the Big Hearts to Little Hearts Foundation and the Congenital Heart Disease Coalition. The authors have reported that they have no relationships relevant to the contents of this paper to disclose. Publisher Copyright: {\textcopyright} 2021 The Authors",

year = "2021",

month = mar,

doi = "10.1016/j.jacbts.2020.12.014",

language = "English",

volume = "6",

pages = "222--235",

journal = "JACC: Basic to Translational Science",

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AU - Bartoli, Carlo R.

AU - Hennessy-Strahs, Samson

AU - Dowling, Robert D.

AU - Gaynor, J. William

AU - Glatz, Andrew C.

N1 - Funding Information: This project was performed with support from the Big Hearts to Little Hearts Foundation and the Congenital Heart Disease Coalition. The authors have reported that they have no relationships relevant to the contents of this paper to disclose. Publisher Copyright: © 2021 The Authors

PY - 2021/3

Y1 - 2021/3

N2 - Children with a bidirectional superior cavopulmonary (Glenn) circulation develop angiodysplasia and pulmonary arteriovenous malformations (AVMs). The von Willebrand factor (vWF)–angiopoietin axis plays a major role in AVM formation in multiple diseases. We observed derangements in global angiogenic signaling, vWF metabolism, angiopoietins, and in vitro angiogenesis in children with a Glenn circulation versus controls and within Glenn pulmonary versus systemic circulations. These findings support the novel hypothesis that abnormalities in the vWF-angiopoietin axis may dysregulate angiogenesis and contribute to Glenn pulmonary AVMs. The vWF-angiopoietin axis may be a target to correct angiogenic imbalance in Glenn patients, for whom no targeted therapy exists.

AB - Children with a bidirectional superior cavopulmonary (Glenn) circulation develop angiodysplasia and pulmonary arteriovenous malformations (AVMs). The von Willebrand factor (vWF)–angiopoietin axis plays a major role in AVM formation in multiple diseases. We observed derangements in global angiogenic signaling, vWF metabolism, angiopoietins, and in vitro angiogenesis in children with a Glenn circulation versus controls and within Glenn pulmonary versus systemic circulations. These findings support the novel hypothesis that abnormalities in the vWF-angiopoietin axis may dysregulate angiogenesis and contribute to Glenn pulmonary AVMs. The vWF-angiopoietin axis may be a target to correct angiogenic imbalance in Glenn patients, for whom no targeted therapy exists.

KW - Glenn

KW - angiogenesis

KW - angiopoietin

KW - arteriovenous malformation

KW - von Willebrand factor

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JO - JACC: Basic to Translational Science

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ER -

Abnormalities in the Von Willebrand-Angiopoietin Axis Contribute to Dysregulated Angiogenesis and Angiodysplasia in Children With a Glenn Circulation

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