ArticleCell stem cell2020
Intrinsic Endocardial Defects Contribute to Hypoplastic Left Heart Syndrome.
Article in Cell stem cell, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 114 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
114 citing papers in PubMed, 166 citations in OpenAlex.
- AP2B1, a protein involved in endocytic trafficking, is associated with congenital heart defects when mutated.Genes & diseases · 2026Article
- In Vitro Models to Study Fibroelastic Remodeling of the Endocardium in Congenital Heart Disease.Pediatric cardiology · 2026Article
- Decoding Long Noncoding RNAs in Cardiac Development and Congenital Heart Disease: Recent Advances and Clinical Perspectives.Circulation. Genomic and precision medicine · 2026Review
- Preconditioned endothelialized grafts reduce stenosis and improve vascular remodeling in a rat pulmonary artery model.Acta biomaterialia · 2026Article
- Transcriptional dynamics of the murine heart during perinatal development at single-cell resolution.Biology open · 2026Article
- Multimodal Nomogram for the Prenatal Risk Assessment of Hypoplastic Left Heart Syndrome Using Self-Supervised Learning.Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine · 2026Article
- Coronary endothelial cells receive Notch1 signal during heart development.Biochemistry and biophysics reports · 2026Article
- ALPK3 regulates cardiomyocyte apoptosis through the Notch1 signaling pathway.Molecular genetics and genomics : MGG · 2026Article
- Machine learning-guided risk stratification for long QT syndrome genetic variants with hiPSC-derived cardiomyocytes.Cardiovascular research · 2026Article
- Hypoplastic Left Heart Syndrome Cardiomyocytes Exhibit Intrinsic Stress Vulnerabilities and Augmented Stress Responses in vitro.Stem cell reviews and reports · 2026Article
- Shear Preconditioning Restores Endothelial Function of HLHS Patient-Derived iPSC-ECs for Successful Conduit Implantation.Arteriosclerosis, thrombosis, and vascular biology · 2026Article
- Genetic drivers of congenital cardiac fibrosis.Communications biology · 2026Review
- Is There a Unified Etiology of Hypoplastic Left Heart Syndrome? Evaluating Genetic, Structural, and Hemodynamic Models of Disease Initiation.Pathophysiology : the official journal of the International Society for Pathophysiology · 2026Review
- Modeling early human heart development using an iPSC-based 3D bioprinted model of embryonic heart tube.Nature communications · 2026Article
- Single-cell omics uncovers novel pathological mechanisms and therapeutic targets for congenital heart diseases: insights from integrated intercellular communication analysis.Stem cell research & therapy · 2026Review
- Vascularized Cardiac Tissue Engineering: From Advances in Biofabrication to Translational Applications.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Review
- Animal models of hypoplastic left heart syndrome: genetic and anatomical approaches.Pediatric research · 2026Review
- Ventricular assist device unloading reverses microvascular senescence in single ventricle disease.Nature cardiovascular research · 2026Article
- Ets1-regulated endothelial-secreted factors promote compact myocardial growth and contribute to the pathogenesis of ventricular non-compaction.Cardiovascular research · 2026Article
- Defects inFrontiers in genetics · 2026Article
54 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
23 authors at 9 institutions in 3 countries.
Funding
Abstract
Hypoplastic left heart syndrome (HLHS) is a complex congenital heart disease characterized by abnormalities in the left ventricle, associated valves, and ascending aorta. Studies have shown intrinsic myocardial defects but do not sufficiently explain developmental defects in the endocardial-derived cardiac valve, septum, and vasculature. Here, we identify a developmentally impaired endocardial population in HLHS through single-cell RNA profiling of hiPSC-derived endocardium and human fetal heart tissue with an underdeveloped left ventricle. Intrinsic endocardial defects contribute to abnormal endothelial-to-mesenchymal transition, NOTCH signaling, and extracellular matrix organization, key factors in valve formation. Endocardial abnormalities cause reduced cardiomyocyte proliferation and maturation by disrupting fibronectin-integrin signaling, consistent with recently described de novo HLHS mutations associated with abnormal endocardial gene and fibronectin regulation. Together, these results reveal a critical role for endocardium in HLHS etiology and provide a rationale for considering endocardial function in regenerative strategies.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.