Carlo R. Bartoli, Samson Hennessy-Strahs, Robert D. Dowling, J. William Gaynor, Andrew C. Glatz
Hospital of the University of Pennsylvania, Children’s Hospital of Philadelphia and University of Pennsylvania. Penn State University College of Medicine.
United States
Journal of the American College of Cardiology Basic Translational Science
JACC Basic Transl Sci 2021; 6: 222-235
DOI: 10.1016/j.jacbts.2020.12.014
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.
Category
Pulmonary Arteriovenous Malformations
Vascular Cell Biology and Mechanisms of Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes