ArticlePediatric research2012
Genetic determinants of right-ventricular remodeling after tetralogy of Fallot repair.
Article in Pediatric research, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 52 citations in OpenAlex.
- T1 mapping of the right ventricle in congenital heart disease: a focus on pediatrics.Pediatric radiology · 2025Review
- Myocardial Fibrosis in Congenital Heart Disease and the Role of MRI.Radiology. Cardiothoracic imaging · 2023Review
- The right ventricle in tetralogy of Fallot: adaptation to sequential loading.Frontiers in pediatrics · 2023Review
- Circular Network of Coregulated Sphingolipids Dictates Chronic Hypoxia Damage in Patients With Tetralogy of Fallot.Frontiers in cardiovascular medicine · 2021Article
- Can Erythropoietin Reduce Hypoxemic Neurological Damages in Neonates With Congenital Heart Defects?Frontiers in pharmacology · 2021Review
- Hypoxia-inducible factor signaling in pulmonary hypertension.The Journal of clinical investigation · 2020Review
- Gene-environment regulatory circuits of right ventricular pathology in tetralogy of fallot.Journal of molecular medicine (Berlin, Germany) · 2019Article
- Determinants of Left Ventricular Dysfunction and Remodeling in Patients With Corrected Tetralogy of Fallot.Journal of the American Heart Association · 2019Article
- Genetic variants of HIF1α are associated with right ventricular fibrotic load in repaired tetralogy of Fallot patients: a cardiovascular magnetic resonance study.Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance · 2019Article
- Adrenergic receptor genotypes influence postoperative outcomes in infants in the Single-Ventricle Reconstruction Trial.The Journal of thoracic and cardiovascular surgery · 2017Article
- Heart Failure in Pediatric Patients With Congenital Heart Disease.Circulation research · 2017Review
- Cardio-Ankle Vascular Index (CAVI) and Plasma Transforming Growth Factor-β1 (TGF-β1) Level Correlate with Aortopathy in Adults with Repaired Tetralogy of Fallot.Pediatric cardiology · 2017Article
- Increased left ventricular myocardial extracellular volume is associated with longer cardiopulmonary bypass times, biventricular enlargement and reduced exercise tolerance in children after repair of Tetralogy of Fallot.Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance · 2016Article
- Cellular and molecular basis of RV hypertrophy in congenital heart disease.Heart (British Cardiac Society) · 2016Review
- Focal myocardial fibrosis assessed by late gadolinium enhancement cardiovascular magnetic resonance in children and adolescents with dilated cardiomyopathy.Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance · 2015Article
- The impact of pulmonary insufficiency on the right ventricle: a comparison of isolated valvar pulmonary stenosis and tetralogy of fallot.Pediatric cardiology · 2015Article
- New mechanistic and therapeutic targets for pediatric heart failure: report from a National Heart, Lung, and Blood Institute working group.Circulation · 2014Article
- Heart failure in congenital heart disease: the role of genes and hemodynamics.Pflugers Archiv : European journal of physiology · 2014Review
- Diffuse myocardial fibrosis following tetralogy of Fallot repair: a T1 mapping cardiac magnetic resonance study.Pediatric radiology · 2014Article
- Heart failure in congenital heart disease: a confluence of acquired and congenital.Heart failure clinics · 2014Review
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Authors and funding
13 authors at 5 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHypoxia-inducible factor (HIF1A) regulates the myocardial response to hypoxia and hemodynamic load. We investigated the association of HIF1A variants with right-ventricular (RV) remodeling after tetralogy of Fallot (TOF) repair.
methodsChildren with TOF were genotyped for three single-nucleotide polymorphisms in HIF1A. Genotypes were analyzed for association with RV myocardial protein expression and fibrosis at complete repair (n = 42) and RV dilation, fractional area change, and freedom from pulmonary valve/conduit replacement on follow-up.
resultsIn 180 TOF patients, mean age at repair was 1.0 ± 0.8 y with follow-up at 9.0 ± 3.5 y; 82% had moderate to severe pulmonary insufficiency. Freedom from RV reinterventions at 5, 10, and 15 y was 92, 84, and 67%, respectively. Patients with more functioning HIF1A alleles had higher transforming growth factor β1 expression and more fibrosis at initial repair as compared with controls (P < 0.05). During follow-up, patients with more functioning HIF1A alleles showed less RV dilation, better preservation of RV function, and greater freedom from RV reinterventions (P < 0.05). This was confirmed in a replication cohort of 69 patients.
conclusionIn children who have had TOF repair, a lower number of functioning HIF1A alleles was associated with RV dilation and dysfunction, suggesting that hypoxia adaptation in unrepaired TOF may influence RV phenotype after repair.
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