Disease: Long QT syndrome type 2
- 4-phenylbutyric acid re-trafficking hERG/G572R channel protein by modulating the endoplasmic reticulum stress-associated chaperones and endoplasmic reticulum-associated degradation gene
- 4q25 Microdeletion with Axenfeld-Rieger Syndrome and Developmental Delay
- A biomimetic ion channel shortens the QT interval of type 2 long QT syndrome through efficient transmembrane transport of potassium ions
- A case of long QT syndrome type 2 that developed torsades de pointes two days after the initiation of oral beta-blocker therapy
- A case of long QT syndrome type 2 that developed torsades de pointes two days after the initiation of oral β-blocker therapy
- A Common East Asian aldehyde dehydrogenase 2*2 variant promotes ventricular arrhythmia with chronic light-to-moderate alcohol use in mice
- A Cross-Sectional Study of the Neuropsychiatric Phenotype of CACNA1C-Related Disorder
- A Novel <em>KCNH2</em> S981fs Mutation Identified by Whole-Exome Sequencing Is Associated with Type 2 Long QT Syndrome
- A Novel KCNH2 S981fs Mutation Identified by Whole-Exome Sequencing Is Associated with Type 2 Long QT Syndrome
- A Novel Variant in AKAP9 Gene, a Controversial Gene, in Long QT Syndrome
- A Possible Explanation for the Low Penetrance of Pathogenic KCNE1 Variants in Long QT Syndrome Type 5
- Activation of small conductance Ca(2+) -activated K(+) channels suppresses Ca(2+) transient and action potential alternans in ventricular myocytes
- Activation of small conductance Ca<sup>2+</sup> -activated K<sup>+</sup> channels suppresses Ca<sup>2+</sup> transient and action potential alternans in ventricular myocytes
- An electrophysiological substrate of COVID-19
- An LQT2-related mutation in the voltage-sensing domain is involved in switching the gating polarity of hERG
- Antisense oligonucleotide therapeutic approach for Timothy syndrome
- Assessment of Corrected QT Interval and QT Dispersion in Patients with Uncomplicated Metabolic Syndrome
- Beneficial effects of chronic mexiletine treatment in a human model of SCN5A overlap syndrome
- Breath-Holding Spells
- Breath-Holding Spells
- CACNA1C-Related Channelopathies
- Calmodulin mutation in long QT syndrome 15 associated with congenital heart defects further complicated by a functional 2:1 atrioventricular block: Management from foetal life to postpartum
- Calmodulin Mutations in Human Disease
- Calmodulin variant E140G associated with long QT syndrome impairs CaMKIIdelta autophosphorylation and L-type calcium channel inactivation
- Calmodulin variant E140G associated with long QT syndrome impairs CaMKIIδ autophosphorylation and L-type calcium channel inactivation
- Characterizing Associations of QTc Interval with Nocturnal Glycemic Control in Children with Type 1 Diabetes
- Classic Timothy Syndrome Associated With Bilateral Border Digit Syndactyly: A Case Series
- Clinical interpretation of KCNH2 variants using a robust PS3/BS3 functional patch clamp assay
- Clinical interpretation of KCNH2 variants using a robust PS3/BS3 functional patch-clamp assay
- Cohort study to assess the prevalence of prolonged QT and arrhythmias in critically ill patients during the early phase of the COVID-19 pandemic
- Current updates on arrhythmia within Timothy syndrome: genetics, mechanisms and therapeutics
- Deciphering HERG mutation in long QT syndrome type 2 using antisense oligonucleotide-mediated techniques: Lessons from cystic fibrosis
- Differential Effects of Remdesivir and Lumacaftor on Homomeric and Heteromeric hERG Channels
- Effects of cohort, genotype, variant, and maternal beta-blocker treatment on foetal heart rate predictors of inherited long QT syndrome
- Effects of cohort, genotype, variant, and maternal β-blocker treatment on foetal heart rate predictors of inherited long QT syndrome
- ELUCIDATING THE ROLE OF THE HIS-PURKINJE SYSTEM DURING LONG QT MEDIATED ARRHYTHMIAS
- Enhancing the interpretation of genetic observations in KCNQ1 in unselected populations: relevance to secondary findings
- Enzastaurin cardiotoxicity: QT interval prolongation, negative inotropic responses and negative chronotropic action
- Extracellular Kir2.1(C122Y) Mutant Upsets Kir2.1-PIP(2) Bonds and Is Arrhythmogenic in Andersen-Tawil Syndrome
- Family long QT syndrome type 2 associated with <em>KCNH2</em> gene mutation: aborted sudden cardiac death
- Family long QT syndrome type 2 associated with <em>KCNH2</em> gene mutation: aborted sudden cardiac death
- Fetal Arrhythmia Leading to a Diagnosis of Congenital Long QT Syndrome Type II
- From gene-specific to function-specific risk stratification in long QT syndrome Type 2: implications for clinical management
- Functional and clinical characterization of a novel homozygous KCNH2 missense variant in the pore region of Kv11.1 leading to a viable but severe long-QT syndrome
- Functional characterization and identification of a therapeutic for a novel SCN5A-F1760C variant causing type 3 long QT syndrome refractory to all guideline-directed therapies
- Gene- and variant-specific efficacy of serum/glucocorticoid-regulated kinase 1 inhibition in long QT syndrome types 1 and 2
- Generation of a human embryonic stem cell line WAe009-A-79 carrying a long QT syndrome mutation in KCNQ1
- Generation of a KCNQ1 (c.1032 + 2 T > C) mutant human embryonic stem cell line via CRISPR base editing
- Generation of patient-specific induced pluripotent stem cell lines with Type 2 Long QT Syndrome and the KCNH2 c.379C > T pathogenic variant
- Generation of patient-specific induced pluripotent stem cell lines with Type 2 Long QT Syndrome and the KCNH2 c.379C > T pathogenic variant
- Geno- and phenotypic characteristics and clinical outcomes of <em>CACNA1C</em> gene mutation associated Timothy syndrome, "cardiac only" Timothy syndrome and isolated long QT syndrome 8: A systematic review
- Geno- and phenotypic characteristics and clinical outcomes of CACNA1C gene mutation associated Timothy syndrome, "cardiac only" Timothy syndrome and isolated long QT syndrome 8: A systematic review
- Heart rate-corrected QT interval prolongation is associated with decreased heart rate variability in patients with type 2 diabetes
- I<sub>Ks</sub> Activator ML277 Mildly Affects Repolarization and Arrhythmic Outcome in the CAVB Dog Model
- Impaired Ca(V)1.2 inactivation reduces the efficacy of calcium channel blockers in the treatment of LQT8
- Impaired Ca<sub>V</sub>1.2 inactivation reduces the efficacy of calcium channel blockers in the treatment of LQT8
- In Vivo Base Editing of <em>Scn5a</em> Rescues Type 3 Long QT Syndrome in Mice
- In Vivo Base Editing of Scn5a Rescues Type 3 Long QT Syndrome in Mice
- Increased Ca(V)1.2 late current by a CACNA1C p.R412M variant causes an atypical Timothy syndrome without syndactyly
- Increased Ca<sub>V</sub>1.2 late current by a CACNA1C p.R412M variant causes an atypical Timothy syndrome without syndactyly
- Inducible over-expression of cardiac Nos1ap causes short QT syndrome in transgenic mice
- Injectable contraceptive Depo-Provera induces erratic beating patterns in patient-specific induced pluripotent stem cell-derived cardiomyocytes with long QT syndrome type 2
- Integrated Approach Including Docking, MD Simulations, and Network Analysis Highlights the Action Mechanism of the Cardiac hERG Activator RPR260243
- International Cohort of Neonatal Timothy Syndrome
- Isolated non-immune mediated second-degree atrioventricular block in fetus: natural history and predictive factors for spontaneous recovery
- KCNH2 mutation c.3099_3112del causes congenital long QT syndrome type 2 with gender differences
- KCNH2A561V Heterozygous Mutation Inhibits KCNH2 Protein Expression via The Activation of UPR Mediated by ATF6
- Life-threatening cardiac arrhythmia and sudden death during electronic gaming: An international case series and systematic review
- Long QT syndrome-associated calmodulin variants disrupt the activity of the slowly activating delayed rectifier potassium channel
- Mechanisms of fever-induced QT prolongation and torsades de pointes in patients with KCNH2 mutation
- Modeling incomplete penetrance in long QT syndrome type 3 through ion channel heterogeneity: an in silico population study
- Modeling long QT syndrome type 2 on-a-chip via in-depth assessment of isogenic gene-edited 3D cardiac tissues
- Non-missense variants of KCNH2 show better outcomes in type 2 long QT syndrome
- Novel Calmodulin Variant p.E46K Associated With Severe Catecholaminergic Polymorphic Ventricular Tachycardia Produces Robust Arrhythmogenicity in Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes
- Novel trans-2,3-enoyl-CoA reductase-like variant associated with catecholaminergic polymorphic ventricular tachycardia type 3
- Prediction of anesthetic torsadogenicity using a human ventricular cell model
- Pregnancy Management in a Patient with Long QT Syndrome Type 2 (LQT2) carrier of the variant KCNH2: Trp100X
- Prescription and Dispensation of QT-Prolonging Medications in Individuals Receiving Hemodialysis
- Prolongation of the heart rate-corrected QT interval is associated with cardiovascular diseases: Systematic review and meta-analysis
- Proof of concept for monoclonal antibody therapy in a cellular model of acquired long QT syndrome type 3
- Proof-of-concept for monoclonal antibody therapy in a cellular model of acquired long QT syndrome type 3
- Risk of Cardiac Lesion with Chronic and Acute Use of Loperamide-An Integrative Review
- SCN5A-1795insD founder variant: a unique Dutch experience spanning 7 decades
- Sex-specific repolarization heterogeneity in mouse left ventricle: Optical mapping combined with mathematical modeling predict the contribution of specific ionic currents
- Spatiotemporal repolarization dispersion before and after exercise in patients with long QT syndrome type 1 versus controls: probing into the arrhythmia substrate
- Sustained heart rate-corrected QT prolongation during recovery from hypoglycaemia in people with type 1 diabetes, independently of recovery to hyperglycaemia or euglycaemia
- Targeted activation of hERG channels rescues electrical instability induced by the hERG R56Q+/- Long QT Syndrome variant
- Targeted activation of human ether-a-go-go-related gene channels rescues electrical instability induced by the R56Q+/- long QT syndrome variant
- The Antiarrhythmic Action of the Na(+)/Ca(2+) Exchanger Inhibitor SEA0400 on Drug-Induced Long QT Syndrome Depends on the Severity of Proarrhythmic Conditions in Anesthetized Atrioventricular Block Rabbits
- The Antiarrhythmic Action of the Na<sup>+</sup>/Ca<sup>2+</sup> Exchanger Inhibitor SEA0400 on Drug-Induced Long QT Syndrome Depends on the Severity of Proarrhythmic Conditions in Anesthetized Atrioventricular Block Rabbits
- The Impact of Chronic Disease on the Corrected QT (QTc) Value in Women in a British Columbia First Nations Population
- The Lancet Commission to reduce the global burden of sudden cardiac death: a call for multidisciplinary action
- Torsade de pointes secondary to long QT syndrome after intragastric balloon placement. A rare but severe complication
- Type 3 long QT syndrome: Is the effectiveness of treatment with beta-blockers population-specific?
- Understanding Calmodulin Variants Affecting Calcium-Dependent Inactivation of L-Type Calcium Channels through Whole-Cell Simulation of the Cardiac Ventricular Myocyte
- Use of subcutaneous cardioverter-defibrillator. First cases reported from National Institute of Cardiology Ignacio Chávez-Mexico
- Using Ribonucleoprotein-based CRISPR/Cas9 to Edit Single Nucleotide on Human Induced Pluripotent Stem Cells to Model Type 3 Long QT Syndrome (SCN5A())
- Using Ribonucleoprotein-based CRISPR/Cas9 to Edit Single Nucleotide on Human Induced Pluripotent Stem Cells to Model Type 3 Long QT Syndrome (SCN5A<sup>±</sup>)
- Wearable electrocardiogram devices in patients with congenital long QT syndrome: The SMART-QT study
- When the Gates Swing Open Only: Arrhythmia Mutations That Target the Fast Inactivation Gate of Na<sub>v</sub>1.5