ArticleThe Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation2014
Gene expression and β-adrenergic signaling are altered in hypoplastic left heart syndrome.
Article in The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 38 citations in OpenAlex.
- Probenecid Improves Cardiac Function in Subjects with a Fontan Circulation and Augments Cardiomyocyte Calcium Homeostasis.Pediatric cardiology · 2020Trial
- Pediatric heart failure: Current approach and treatment.JHLT open · 2025Article
- Cellular and Transcriptional Landscape of Human Hypoplastic Left Heart Syndrome.Research square · 2025Article
- Hypoplastic left heart syndrome (HLHS) becomes of age: Assessing the young adult with HLHS including the neoaorta/aortic arch.International journal of cardiology. Congenital heart disease · 2025Review
- Cell-based therapy to boost right ventricular function and cardiovascular performance in hypoplastic left heart syndrome: Current approaches and future directions.Seminars in perinatology · 2023Review
- Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome.Journal of cardiovascular development and disease · 2022Article
- Current state of the art in hypoplastic left heart syndrome.Frontiers in cardiovascular medicine · 2022Review
- Altered metabolic and inflammatory transcriptomics after cardiac surgery in neonates with congenital heart disease.Scientific reports · 2021Observational
- Cardiovascular Drug Therapy during Interstage After Hybrid Approach: A Single-Center Experience in 51 Newborns with Hypoplastic Left Heart.Paediatric drugs · 2021Observational
- Physiology of the Right Ventricle Across the Lifespan.Frontiers in physiology · 2021Review
- Important Considerations in Pediatric Heart Failure.Current cardiology reports · 2020Review
- Heart failure in single right ventricle congenital heart disease: physiological and molecular considerations.American journal of physiology. Heart and circulatory physiology · 2020Review
- HuR regulates phospholamban expression in isoproterenol-induced cardiac remodelling.Cardiovascular research · 2020Article
- Alteration of cardiolipin biosynthesis and remodeling in single right ventricle congenital heart disease.American journal of physiology. Heart and circulatory physiology · 2020Article
- Pediatric Heart Failure: An Evolving Public Health Concern.The Journal of pediatrics · 2020Article
- Control of cytokinesis by β-adrenergic receptors indicates an approach for regulating cardiomyocyte endowment.Science translational medicine · 2019Article
- Hypoplastic left heart syndrome: From bedside to bench and back.Journal of molecular and cellular cardiology · 2019Review
- Elamipretide Improves Mitochondrial Function in the Failing Human Heart.JACC. Basic to translational science · 2019Article
- Phosphodiesterase-5 Is Elevated in Failing Single Ventricle Myocardium and Affects Cardiomyocyte Remodeling In Vitro.Circulation. Heart failure · 2018Article
- Histone deacetylase adaptation in single ventricle heart disease and a young animal model of right ventricular hypertrophy.Pediatric research · 2017Article
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Authors and funding
12 authors at 3 institutions in 1 country.
Funding
Abstract
backgroundThe purpose of the current study was to define the myocellular changes and adaptation of the β-adrenergic receptor (β-AR) system that occur in the systemic right ventricle (RV) of children with hypoplastic left heart syndrome (HLHS).
methodsExplanted hearts from children with HLHS and non-failing controls were used for this study. HLHS patients were divided into 2 groups: "compensated" (C-HLHS), infants listed for primary transplant with normal RV function and absence of heart failure symptoms, and "decompensated" (D-HLHS), patients listed for transplant after failed surgical palliation with RV failure and/or refractory protein-losing enteropathy or plastic bronchitis.
resultsCompared with non-failing control RVs, the HLHS RV demonstrated decreased sarcoplasmic reticulum calcium-adenosine triphosphatase 2a and α-myosin heavy chain (MHC) gene expression, decreased total β-AR due to down-regulation of β1-AR, preserved cyclic adenosine monophosphate levels, and increased calcium/calmodulin-dependent protein kinase II (CaMKII) activity. There was increased atrial natriuretic peptide expression only in the C-HLHS group. Unique to those in the D-HLHS group was increased β-MHC and decreased α-MHC protein expression (MHC isoform switching), increased adenylyl cyclase 5 expression, and increased phosphorylation of the CaMK target site on phospholamban, threonine 17.
conclusionsThe HLHS RV has an abnormal myocardial gene expression pattern, downregulation of β1-AR, preserved cyclic adenosine monophosphate levels, and increased CaMKII activity compared with the non-failing control RV. There is MHC isoform switching, increased adenylyl cyclase 5, and increased phosphorylation of phospholamban threonine 17 only in the D-HLHS group. Although abnormal gene expression and changes in the β-AR system precede clinically evident ventricular failure in HLHS, additional unique adaptations occur in those with HLHS and failed surgical palliation.
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