Disease: Long QT syndrome type 3
- <em>ARumenamides</em>: A novel class of potential antiarrhythmic compounds
- 1,4-Disubstituted Piperazin-2-Ones as Selective Late Sodium Current Inhibitors with QT Interval Shortening Properties in Isolated Rabbit Hearts
- A distinct molecular mechanism by which phenytoin rescues a novel long QT 3 variant
- A Heart Failure-Associated <em>SCN5A</em> Splice Variant Leads to a Reduction in Sodium Current Through Coupled-Gating With the Wild-Type Channel
- A Molecularly Detailed Na<sub>V</sub>1.5 Model Reveals a New Class I Antiarrhythmic Target
- A Novel SCN5A Mutation Found in a Familial Case of Long QT Syndrome Complicated by Severe Left Ventricular Dysfunction
- Advancing Cardiovascular Drug Screening Using Human Pluripotent Stem Cell-Derived Cardiomyocytes
- Analysis of the shape of the T-wave in congenital long-QT syndrome type 3 by geometric morphometrics
- Anti-arrhythmic potential of the late sodium current inhibitor GS-458967 in murine Scn5a-1798insD+/- and human SCN5A-1795insD+/- iPSC-derived cardiomyocytes
- Antiarrhythmic Hit to Lead Refinement in a Dish Using Patient-Derived iPSC Cardiomyocytes
- Arrhythmogenic Biophysical Phenotype for SCN5A Mutation S1787N Depends upon Splice Variant Background and Intracellular Acidosis
- Association Between Syncope Trigger Type and Risk of Subsequent Life-Threatening Events in Patients With Long QT Syndrome
- Beneficial effects of chronic mexiletine treatment in a human model of SCN5A overlap syndrome
- Binding characteristics of calpastatin domain L to Na<sub>V</sub>1.5 sodium channel and its IQ motif mutants
- Cardiac Sodium Channel Mutations: Why so Many Phenotypes?
- Clinical Aspects of Type 3 Long-QT Syndrome: An International Multicenter Study
- Clinical Spectrum of <em>SCN5A</em> Channelopathy in Children with Primary Electrical Disease and Structurally Normal Hearts
- Combined mexiletine and flecainide for severe long QT syndrome type 3
- Complex interactions in a novel SCN5A compound mutation associated with long QT and Brugada syndrome: Implications for Na+ channel blocking pharmacotherapy for de novo conduction disease
- Complexity of ranolazine and phenytoin use in an infant with long QT syndrome type 3
- Computational Study on Effect of KCNQ1 P535T Mutation in a Cardiac Ventricular Tissue
- Detection of distant familial relatedness in biobanks for identification of undiagnosed carriers of a Mendelian disease variant: application to Long QT syndrome
- Detection of Na<sub>v</sub>1.5 Conformational Change in Mammalian Cells Using the Noncanonical Amino Acid ANAP
- Did Mutation Type Affect the Efficacy of Mexiletine Observed in Patients With LQTS Type 3?
- Dynamic QT response to cold-water face immersion in long-QT syndrome type 3
- E1784K, the most common Brugada syndrome and long-QT syndrome type 3 mutant, disrupts sodium channel inactivation through two separate mechanisms
- Effects of Allicin on Late Sodium Current Caused by ΔKPQ-SCN5A Mutation in HEK293 Cells
- Effects of amiodarone on rodent ventricular cardiomyocytes: Novel perspectives from a cellular model of Long QT Syndrome Type 3
- Eleclazine exhibits enhanced selectivity for long QT syndrome type 3-associated late Na(+) current
- Embryonic type Na<sup>+</sup> channel β-subunit, SCN3B masks the disease phenotype of Brugada syndrome
- Establishment of human embryonic stem cell WAe009-A-48 carrying a long QT syndrome mutation in SCN5A
- Experimental analysis of the onset mechanism of TdP reported in an LQT3 patient during pharmacological treatment with serotonin-dopamine antagonists against insomnia and nocturnal delirium
- Functional Consequences of the <em>SCN5A</em>-p.Y1977N Mutation within the PY Ubiquitylation Motif: Discrepancy between HEK293 Cells and Transgenic Mice
- Functional modulation of atrio-ventricular conduction by enhanced late sodium current and calcium-dependent mechanisms in Scn5a1798insD/+ mice
- Gene-Specific Therapy With Mexiletine Reduces Arrhythmic Events in Patients With Long QT Syndrome Type 3
- Genetic analysis of cardiac SCN5A Gene in Iranian patients with hereditary cardiac arrhythmias
- Genetic and clinical characteristics of single and compound types of patients with long QT syndrome type 3
- Genetic causes of sudden cardiac death in the young
- Genotype-phenotype dilemma in a case of sudden cardiac death with the E1053K mutation and a deletion in the SCN5A gene
- High incidence of sudden cardiac death in one family with type-3 long QT syndrome: molecular genetics and electrophysiology mechanism analysis
- High Prevalence of Late-Appearing T-Wave in Patients With Long QT Syndrome Type 8
- Human iPS cell model of type 3 long QT syndrome recapitulates drug-based phenotype correction
- Human iPSC-derived cardiomyocytes and pyridyl-phenyl mexiletine analogs
- Hypernatremia and intercalated disc edema synergistically exacerbate long-QT syndrome type 3 phenotype
- I<sub>Ks</sub> Activator ML277 Mildly Affects Repolarization and Arrhythmic Outcome in the CAVB Dog Model
- Impact of pacing frequency in amiodarone interaction with cardiomyocytes near physiological temperature in health and disease conditions
- 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
- Independent validation and clinical implications of the risk prediction model for long QT syndrome (1-2-3-LQTS-Risk)
- Intracellular calcium attenuates late current conducted by mutant human cardiac sodium channels
- Lidocaine attenuation testing: An in vivo investigation of putative LQT3-associated variants in the SCN5A-encoded sodium channel
- Long-term flecainide therapy in type 3 long QT syndrome
- Mechanisms underlying age-associated manifestation of cardiac sodium channel gain-of-function
- Metabolic Maturation Media Improve Physiological Function of Human iPSC-Derived Cardiomyocytes
- Mexiletine
- Mexiletine rescues a mixed biophysical phenotype of the cardiac sodium channel arising from the SCN5A mutation, N406K, found in LQT3 patients
- Modeling incomplete penetrance in long QT syndrome type 3 through ion channel heterogeneity: an in silico population study
- Multifocal ectopic purkinje-related premature contractions and related cardiomyopathy
- Multiple mechanisms underlie increased cardiac late sodium current
- Mutational analysis of SCN5A gene in long QT syndrome
- Outcome by Sex in Patients With Long QT Syndrome With an Implantable Cardioverter Defibrillator
- Phenotypic variability in LQT3 human induced pluripotent stem cell-derived cardiomyocytes and their response to antiarrhythmic pharmacologic therapy: An in silico approach
- Polypharmacy-Induced Long QT Syndrome in a Patient With Newly Diagnosed Acute Myeloid Leukaemia: A Case Report
- Predicting Patient Response to the Antiarrhythmic Mexiletine Based on Genetic Variation
- 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
- Propranolol Attenuates Late Sodium Current in a Long QT Syndrome Type 3-Human Induced Pluripotent Stem Cell Model
- Publisher Correction: Analysis of the shape of the T-wave in congenital long-QT syndrome type 3 by geometric morphometrics
- Purkinje Cells as Sources of Arrhythmias in Long QT Syndrome Type 3
- Purkinje system hyperexcitability and ventricular arrhythmia risk in type 3 long QT syndrome
- QT Dynamics During Exercise in Asymptomatic Children with Long QT Syndrome Type 3
- Ranolazine as an Alternative Therapy to Flecainide for SCN5A V411M Long QT Syndrome Type 3 Patients
- Requirement of β subunit for the reduced voltage-gated Na<sup>+</sup> current of a Brugada syndrome patient having novel double missense mutation (p.A385T/R504T) of <em>SCN5A</em>
- Research progress of the treatment of congenital long QT syndrome type 3
- Revealing the Concealed Nature of Long-QT Type 3 Syndrome
- Role of chronic continuous intravenous lidocaine in the clinical management of patients with malignant type 3 long QT syndrome
- Same Gene, Different Story (a Case Report of Congenital Long QT Syndrome Subtype 8 With a Novel Mutation)
- SCN5A mutations in 442 neonates and children: genotype-phenotype correlation and identification of higher-risk subgroups
- SCN5a overlap syndromes-This episode: Long QT syndrome type 3 meets multifocal ectopic Purkinje-related premature contractions
- SCN5A-1795insD founder variant: a unique Dutch experience spanning 7 decades
- Selective late sodium current inhibitor GS-458967 suppresses Torsades de Pointes by mostly affecting perpetuation but not initiation of the arrhythmia
- Sevoflurane prolonged the QTc interval and increased transmural dispersion of repolarization in a patient with long QT syndrome 3: a case report
- Structure of human Na<sub>v</sub>1.5 reveals the fast inactivation-related segments as a mutational hotspot for the long QT syndrome
- Subcutaneous and transvenous implantable cardioverter defibrillator in high-risk long-QT syndrome type 3 associated with Val411Met mutation in SCN5A
- 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 citrus flavanone hesperetin preferentially inhibits slow-inactivating currents of a long QT syndrome type 3 syndrome Na(+) channel mutation
- The Citrus Flavonoid Hesperetin Has an Inadequate Anti-Arrhythmic Profile in the ΔKPQ Na<sub>V</sub>1.5 Mutant of the Long QT Type 3 Syndrome
- The congenital long QT syndrome Type 3: An update
- The effects of ageing and adrenergic challenge on electrocardiographic phenotypes in a murine model of long QT syndrome type 3
- The efficacy of Ranolazine on E1784K is altered by temperature and calcium
- The role of mexiletine in the management of long QT syndrome
- Therapeutic approaches for Long QT syndrome type 3: an update
- 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
- Triggers, characteristics, and hospital outcome of patients with Takotsubo syndrome: 10 years experience in a large university hospital centre
- 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<sup>±</sup>)
- Variant-specific therapy for long QT syndrome type 3
- When the Gates Swing Open Only: Arrhythmia Mutations That Target the Fast Inactivation Gate of Na<sub>v</sub>1.5
- Whole-exome sequencing identifies a Novel SCN5A mutation (C335R) in a Chinese family with arrhythmia