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.

• Authors: Carlo R Bartoli, Samson Hennessy-Strahs, Robert D Dowling, J William Gaynor, Andrew C Glatz
• Journal: JACC. Basic to translational science (JACC Basic Transl Sci) Vol. 6 Issue 3 Pg. 222-235 (Mar 2021) ISSN: 2452-302X [Electronic] United States
• PMID: 33778210 (Publication Type: Journal Article)
• Copyright: © 2021 The Authors.