• 1,4-Disubstituted Piperazin-2-Ones as Selective Late Sodium Current Inhibitors with QT Interval Shortening Properties in Isolated Rabbit Hearts
• 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 mild phenotype associated with KCNQ1 p.V205M mediated long QT syndrome in First Nations children of Northern British Columbia: effect of additional variants and considerations for management
• 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
• 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
• Characterizing Associations of QTc Interval with Nocturnal Glycemic Control in Children with Type 1 Diabetes
• 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
• Concentration-QTc Modeling of the DPP-4 Inhibitor HSK7653 in a First-in-Human Study of Chinese Healthy Volunteers
• 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
• Divergent Biochemical Properties and Disparate Impact of Arrhythmogenic Calmodulin Mutations on Zebrafish Cardiac Function
• 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
• Electrophysiological effects of estrogen on hiPSC-derived cardiomyocytes of a patient affected by estrogen-sensitive Long-QT Syndrome Type 2
• 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
• Evaluation of the Effect of Favipiravir on QT Intervals in COVID-19 Patients with and without Diabetes Mellitus
• Expanding the Phenotype of the CACNA1C-Associated Neurological Disorders in Children: Systematic Literature Review and Description of a Novel Mutation
• Extracellular Kir2.1(C122Y) Mutant Upsets Kir2.1-PIP(2) Bonds and Is Arrhythmogenic in Andersen-Tawil Syndrome
• Factors associated with prolonged QTc interval in Iranian population: MASHAD cohort study
• Family long QT syndrome type 2 associated with &lt;em&gt;KCNH2&lt;/em&gt; 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
• Generating a human induced pluripotent stem cell line (XACHi018-A) from a Timothy syndrome infant carrying heterozygous CACNA1C c.1216G>A (p.G406R) mutation
• 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 &gt; 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
• Genetic and clinical characteristics of single and compound types of patients with long QT syndrome type 3
• Genetic characterization of KCNQ1 variants improves risk stratification in type 1 long QT syndrome patients
• I_Ks Activator ML277 Mildly Affects Repolarization and Arrhythmic Outcome in the CAVB Dog Model
• Identifying genomic variant associated with long QT syndrome type 2 in an ecuadorian mestizo individual: a case report
• Identifying Key Binding Interactions Between the Cardiac L-Type Calcium Channel and Calmodulin Using Molecular Dynamics Simulations
• 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
• 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
• 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 KCNQ1 Q234K variant, identified in patients with long QT syndrome and epileptiform activity, induces both gain- and loss-of-function of slowly activating delayed rectifier potassium currents
• Novel trans-2,3-enoyl-CoA reductase-like variant associated with catecholaminergic polymorphic ventricular tachycardia type 3
• Pediatric and Familial Genetic Arrhythmia Syndromes: SCN5A-Related Disorders When It Is Not Long QT Type 3: Clinical Signs and Symptoms
• 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
• Rescue of expression and function of long QT syndrome-causing mutant hERG channels by enhancing channel stability in the plasma membrane
• 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
• Systematic analysis of SCN5A variants associated with inherited cardiac diseases
• 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⁺/Ca²⁺ 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
• Therapeutic Efficacy of Mexiletine for Long QT Syndrome Type 2: Evidence From Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes, Transgenic Rabbits, and Patients
• 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^±)
• Wearable electrocardiogram devices in patients with congenital long QT syndrome: The SMART-QT study