JACC Basic to Translational Science· 2026Q1
Decreased Endothelial Cell Retinoic Acid Signaling Accelerates Progression of Single Ventricle Pulmonary Vascular Malformations
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- Q1SCImago
- 2026year
Short summary
Decreased endothelial cell retinoic acid (ATRA) signaling accelerates pulmonary vascular malformations (PVMs) in a rat model of single ventricle heart disease, with excess dietary Vitamin A mitigating severity.
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Key points
- Single ventricle PVMs share decreased endothelial cell ATRA signaling with hereditary AVMs.
- scRNAseq identified significant EC gene expression changes post-Glenn surgery, including reduced ATRA signaling.
- Dietary Vitamin A deficiency worsened PVM shunting severity in rats.
- Excess dietary Vitamin A intake mitigated PVM shunting severity.
AI-generated from the title and abstract; the full text is not read.
Abstract
Pulmonary vascular malformations (PVMs) are vascular complications that universally develop in patients with single ventricle congenital heart disease after Glenn surgery, yet the pathogenesis of single ventricle PVMs remains unknown. Using a surgical rat model of Glenn circulation, single-cell RNA sequencing (scRNAseq) on whole lung samples identified dramatic endothelial cell (EC) gene expression changes post-surgery, including decreased all-trans retinoic acid (ATRA) signaling. Many gene expression differences (∼17% of all EC differentially expressed genes), including decreased ATRA signaling, were shared with 2 independent models of hereditary hemorrhagic telangiectasia arteriovenous malformations (AVMs). Subsequent dietary modification of vitamin A (Vit A) intake in rats, identified that dietary deficiency of Vit A increased PVM shunting severity, which was mitigated with excess dietary Vit A intake. Collectively, our study of post-Glenn PVMs identified shared patho-mechanisms with hereditary hemorrhagic telangiectasia AVMs, including decreased EC ATRA signaling, which is a novel potential target for treating AVMs.
The authors' abstract, as published at the source. JACC Basic to Translational Science, 2026 · DOI ↗
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Field: Physiology
PhysiologyMedicine