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

TitleAbnormalities in the Von Willebrand-Angiopoietin Axis Contribute to Dysregulated Angiogenesis and Angiodysplasia in Children With a Glenn Circulation.
Publication TypeJournal Article
Year of Publication2021
AuthorsBartoli CR, Hennessy-Strahs S, Dowling RD, J Gaynor W, Glatz AC
JournalJACC Basic Transl Sci
Volume6
Issue3
Pagination222-235
Date Published2021 Mar
ISSN2452-302X
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.

DOI10.1016/j.jacbts.2020.12.014
Alternate JournalJACC Basic Transl Sci
PubMed ID33778210
PubMed Central IDPMC7987544