Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
Aims: Hypoplastic coronary artery disease is a rare congenital abnormality that is associated with sudden cardiac death. However, molecular mechanisms responsible for this disease are not clear. The aim of the present study was to assess the role of nitric oxide synthase-3 (NOS3) in the pathogenesis of hypoplastic coronary arteries.
Methods And Results: Wild-type (WT), NOS3(-/-), and a novel cardiac-specific NOS3 overexpression mouse model were employed. Deficiency in NOS3 resulted in coronary artery hypoplasia in foetal mice and spontaneous myocardial infarction in postnatal hearts. Coronary artery diameters, vessel density, and volume were significantly decreased in NOS3(-/-) mice at postnatal day 0. In addition, NOS3(-/-) mice showed a significant increase in the ventricular wall thickness, myocardial volume, and cardiomyocyte cell size compared with WT mice. Lack of NOS3 also down-regulated the expression of Gata4, Wilms tumour-1, vascular endothelial growth factor, basic fibroblast growth factor and erythropoietin, and inhibited migration of epicardial cells. These abnormalities and hypoplastic coronary arteries in the NOS3(-/-) mice were completely rescued by the cardiac-specific overexpression of NOS3.
Conclusion: Nitric oxide synthase-3 is required for coronary artery development and deficiency in NOS3 leads to hypoplastic coronary arteries.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1093/eurheartj/ehs306 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Validity of diagnoses, procedures, and birth records in a Japanese administrative claims database for pediatric patients.
Pediatr Int
September 2025
Department of Clinical Epidemiology and Health Economics, School of Public Health, The University of Tokyo, Tokyo, Japan.
Background: Administrative claims data are used in clinical studies; however, recorded diagnoses and procedures have not been fully validated for pediatric patients. We aimed to examine the validity of recorded information on pediatric patients in the Japanese Diagnosis Procedure Combination (DPC) database, a national inpatient database that includes administrative claims data.
Methods: We validated the DPC data using medical charts as the reference standard.
Natural killer cell subpopulations in the peripheral blood of single ventricle/hypoplastic left heart syndrome patients via single-cell RNA sequencing.
Exp Biol Med (Maywood)
September 2025
The Center for Applied Genomics, Children's Hospital of Philadelphia, Philadelphia, PA, United States.
Natural Killer (NK) cells are integral components of the innate immune system, recognizing and eliminating virus-infected cells. They may play a crucial role in the immune response and contribute to the complications associated with Single Ventricle/Hypoplastic Left Heart Syndrome (SV/HLHS). Utilizing single-cell RNA sequencing (scRNA-seq), NK cells from peripheral blood mononuclear cells (PBMCs) were analyzed in three de-identified SV/HLHS cases and three healthy controls.
View Article and Find Full Text PDFRelationship between anaesthesia strategy and intracardiac hemodynamics during paediatric cardiac catheterisation.
Cardiol Young
September 2025
Department of Pediatrics, Division of Pediatric Critical Care Medicine, Vanderbilt University School of Medicine, Nashville, TN, USA.
Objectives: Describe the hemodynamic implications of anaesthetic choice among children with heart disease undergoing cardiac catheterisation.
Methods: Study 1 was a secondary analysis of data obtained during catheterisation-based hemodynamic assessment of infants with hypoplastic left heart syndrome following Stage 1 palliation, randomised in the Single Ventricle Reconstruction trial. Measured and calculated hemodynamics including pulmonary and systemic vascular resistance indexed to body surface area (PVRi and SVRi respectively) and pulmonary/systemic blood flow (Qp/Qs) were analysed with respect to anaesthetic employed during catheterisation, classified as moderate sedation or general anaesthesia.
Technical Advances and Outcomes of Fetal Atrial Septal Intervention for Restrictive or Intact Atrial Septum.
Circ Cardiovasc Interv
September 2025
Department of Pediatrics, Division of Pediatric Cardiology (B.Y.F., P.D., M.M., C.A.A., S.G., L.F.E., G.S., R.T., H.D., N.A., A.M.Q., S.A.M.), Texas Children's Hospital and Baylor College of Medicine, Houston.
Background: Infants with hypoplastic left heart syndrome with severely restrictive or intact atrial septum (R/IAS) have low survival. In-utero creation of an atrial septal communication has been reported, with high complication rates.
Methods: We performed a retrospective cohort study of fetuses with hypoplastic left heart syndrome, double outlet right ventricular with mitral stenosis/atresia, or mitral valve dysplasia and R/IAS from 2012 to 2024 who underwent evaluation for fetal atrial septal intervention (FASI).
Outcomes of Pregnancies with Absent or Hypoplastic Fetal Nasal Bone: A Retrospective Analysis of Prenatal Findings and Perinatal Outcomes.
Life (Basel)
August 2025
Department of Obstetrics and Gynecology, Division of Obstetrics and Feto-Maternal Medicine, Medical University of Vienna, Spitalgasse 23, 1090 Vienna, Austria.
Hypoplastic or absent fetal nasal bone (NB) is a significant soft marker in the risk assessment for aneuploidies. This study aimed to evaluate prenatal findings and perinatal outcomes in fetuses with absent or hypoplastic NB managed at our center. This retrospective analysis was conducted at the Department of Obstetrics at the Medical University of Vienna and including all cases with an absent or hypoplastic fetal NB between 2004 and 2022.
View Article and Find Full Text PDF