Studying Long QT Syndrome Caused by < em > NAA10 < /em > Genetic Variants Using Patient-Derived Induced Pluripotent Stem Cells
Circulation. 2023 Nov 14;148(20):1598-1601. doi: 10.1161/CIRCULATIONAHA.122.061864. Epub 2023 Nov 13.NO ABSTRACTPMID:37956223 | DOI:10.1161/CIRCULATIONAHA.122.061864 (Source: Circulation)
Source: Circulation - November 13, 2023 Category: Cardiology Authors: Nadjet Belbachir Yiyang Wu Mengcheng Shen Sophia L Zhang Joe Z Zhang Chun Liu Bjorn C Knollmann Gholson J Lyon Ning Ma Joseph C Wu Source Type: research

Type 3 long QT syndrome: is the effectiveness of treatment with beta-blockers population-specific?
Efficacy of beta blocker treatment in type 3 long QT syndrome (LQT3) remains debated. (Source: Heart Rhythm)
Source: Heart Rhythm - November 11, 2023 Category: Cardiology Authors: Alexis Hermida, Jean-Baptiste Gourraud, Isabelle Denjoy, V éronique Fressart, Florence Kyndt, Alice Maltret, Diala Khraiche, Didier Klug, Philippe Mabo, Frédéric Sacher, Philippe Maury, Pierre Winum, Pascal Defaye, Gael Clerici, Dominique Babuty, Yedid Source Type: research

Proof-of-concept for monoclonal antibody therapy in a cellular model of acquired long QT syndrome type 3
Am J Physiol Heart Circ Physiol. 2023 Nov 10. doi: 10.1152/ajpheart.00628.2023. Online ahead of print.ABSTRACTLong QT syndrome (LQTS) type 3 although less common than the first two forms, differs in that arrhythmic events are less likely triggered by adrenergic stimuli and are more often lethal. Effective pharmacological treatment is challenged by inter-individual differences, mutation dependence and adverse effects, translating into an increased use of invasive measures (implantable cardioverter-defibrillator, sympathetic denervation) in LQTS type 3 patients. Previous studies have demonstrated the therapeutic potential of...
Source: American Journal of Physiology. Heart and Circulatory Physiology - November 10, 2023 Category: Physiology Authors: Lenke Kis Jin Li Source Type: research

Proof-of-concept for monoclonal antibody therapy in a cellular model of acquired long QT syndrome type 3
Am J Physiol Heart Circ Physiol. 2023 Nov 10. doi: 10.1152/ajpheart.00628.2023. Online ahead of print.ABSTRACTLong QT syndrome (LQTS) type 3 although less common than the first two forms, differs in that arrhythmic events are less likely triggered by adrenergic stimuli and are more often lethal. Effective pharmacological treatment is challenged by inter-individual differences, mutation dependence and adverse effects, translating into an increased use of invasive measures (implantable cardioverter-defibrillator, sympathetic denervation) in LQTS type 3 patients. Previous studies have demonstrated the therapeutic potential of...
Source: American Journal of Physiology. Heart and Circulatory Physiology - November 10, 2023 Category: Physiology Authors: Lenke Kis Jin Li Source Type: research

Proof-of-concept for monoclonal antibody therapy in a cellular model of acquired long QT syndrome type 3
Am J Physiol Heart Circ Physiol. 2023 Nov 10. doi: 10.1152/ajpheart.00628.2023. Online ahead of print.ABSTRACTLong QT syndrome (LQTS) type 3 although less common than the first two forms, differs in that arrhythmic events are less likely triggered by adrenergic stimuli and are more often lethal. Effective pharmacological treatment is challenged by inter-individual differences, mutation dependence and adverse effects, translating into an increased use of invasive measures (implantable cardioverter-defibrillator, sympathetic denervation) in LQTS type 3 patients. Previous studies have demonstrated the therapeutic potential of...
Source: American Journal of Physiology. Heart and Circulatory Physiology - November 10, 2023 Category: Physiology Authors: Lenke Kis Jin Li Source Type: research

Proof-of-concept for monoclonal antibody therapy in a cellular model of acquired long QT syndrome type 3
Am J Physiol Heart Circ Physiol. 2023 Nov 10. doi: 10.1152/ajpheart.00628.2023. Online ahead of print.ABSTRACTLong QT syndrome (LQTS) type 3 although less common than the first two forms, differs in that arrhythmic events are less likely triggered by adrenergic stimuli and are more often lethal. Effective pharmacological treatment is challenged by inter-individual differences, mutation dependence and adverse effects, translating into an increased use of invasive measures (implantable cardioverter-defibrillator, sympathetic denervation) in LQTS type 3 patients. Previous studies have demonstrated the therapeutic potential of...
Source: American Journal of Physiology. Heart and Circulatory Physiology - November 10, 2023 Category: Physiology Authors: Lenke Kis Jin Li Source Type: research

Proof-of-concept for monoclonal antibody therapy in a cellular model of acquired long QT syndrome type 3
Am J Physiol Heart Circ Physiol. 2023 Nov 10. doi: 10.1152/ajpheart.00628.2023. Online ahead of print.ABSTRACTLong QT syndrome (LQTS) type 3 although less common than the first two forms, differs in that arrhythmic events are less likely triggered by adrenergic stimuli and are more often lethal. Effective pharmacological treatment is challenged by inter-individual differences, mutation dependence and adverse effects, translating into an increased use of invasive measures (implantable cardioverter-defibrillator, sympathetic denervation) in LQTS type 3 patients. Previous studies have demonstrated the therapeutic potential of...
Source: American Journal of Physiology. Heart and Circulatory Physiology - November 10, 2023 Category: Physiology Authors: Lenke Kis Jin Li Source Type: research

Proof-of-concept for monoclonal antibody therapy in a cellular model of acquired long QT syndrome type 3
Am J Physiol Heart Circ Physiol. 2023 Nov 10. doi: 10.1152/ajpheart.00628.2023. Online ahead of print.ABSTRACTLong QT syndrome (LQTS) type 3 although less common than the first two forms, differs in that arrhythmic events are less likely triggered by adrenergic stimuli and are more often lethal. Effective pharmacological treatment is challenged by inter-individual differences, mutation dependence and adverse effects, translating into an increased use of invasive measures (implantable cardioverter-defibrillator, sympathetic denervation) in LQTS type 3 patients. Previous studies have demonstrated the therapeutic potential of...
Source: American Journal of Physiology. Heart and Circulatory Physiology - November 10, 2023 Category: Physiology Authors: Lenke Kis Jin Li Source Type: research

Case report of a child with long QT syndrome type 14 caused by CALM1 gene mutation and literature review
ConclusionsThis patient had multiple episodes of convulsions or syncope after emotional stimulation or activity, with intermittent prolongation of the QTc on routine ECG, marked prolongation of the QTc after exercise, and T-wave alternans, which differed from the LQT14 phenotype caused by the previous CALM1 mutation. (Source: Molecular Genetics & Genomic Medicine)
Source: Molecular Genetics & Genomic Medicine - November 1, 2023 Category: Genetics & Stem Cells Authors: Qiqing Sun, Zhenhua Xie, Fangjie Wang, Jun Guo, Xiaochen Yan Tags: CLINICAL REPORT Source Type: research

Case report of a child with long QT syndrome type 14 caused by CALM1 gene mutation and literature review
CONCLUSIONS: This patient had multiple episodes of convulsions or syncope after emotional stimulation or activity, with intermittent prolongation of the QTc on routine ECG, marked prolongation of the QTc after exercise, and T-wave alternans, which differed from the LQT14 phenotype caused by the previous CALM1 mutation.PMID:37905352 | DOI:10.1002/mgg3.2287 (Source: Molecular Medicine)
Source: Molecular Medicine - October 31, 2023 Category: Molecular Biology Authors: Qiqing Sun Zhenhua Xie Fangjie Wang Jun Guo Xiaochen Yan Source Type: research

Automated quantification of QT-intervals by an algorithm: A validation study in patients with COPD
Conclusion: Clinically relevant QTc-prolongations were accurately identified by the automated algorithm. The implementation of this tool in hospital sleep laboratories may identify asymptomatic patients with long-QT at risk for malignant arrhythmia, allowing them to consult a cardiologist before an eventual cardiac event. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Kohlbrenner, D., Bisang, M., Aeschbacher, S. S., Heusser, E., Ulrich, S., Bloch, K. E., Furian, M. Tags: Monitoring airway disease Source Type: research

Mexiletine: Antiarrhythmic mechanisms, emerging clinical applications and mortality
AbstractMexiletine, a class Ib antiarrhythmic drug, exhibits its major antiarrhythmic effect via inhibition of the fast and late Na+ currents in myocardial tissues that are dependent on the opening of Na+ channels for their excitation. Through a comprehensive examination of mexiletine's therapeutic benefits and potential risks, we aim to provide valuable insights that reinforce its role as a vital therapeutic option for patients with ventricular arrhythmias, long QT syndrome, and other heart rhythm disorders. This review will highlight the current understandings of the antiarrhythmic effects and rationales for recent off-l...
Source: Pacing and Clinical Electrophysiology : PACE - October 17, 2023 Category: Cardiology Authors: Farah Olleik, Mohammed Hussein Kamareddine, Jenna Spears, Gary Tse, Tong Liu, Gan ‐Xin Yan Tags: REVIEW Source Type: research

Spatio-temporal repolarization dispersion before and after exercise in patients with long QT syndrome type 1 vs controls - probing into the arrhythmia substrate
In conclusion, while temporal dispersion of repolarization increased significantly more after exercise in LQT1 patients, there were no signs of exercise-induced increase in global dispersion of action potential duration and morphology. The arrhythmia substrate/mechanism in LQT1 warrants further study.PMID:37773058 | DOI:10.1152/ajpheart.00335.2023 (Source: American Journal of Physiology. Heart and Circulatory Physiology)
Source: American Journal of Physiology. Heart and Circulatory Physiology - September 29, 2023 Category: Physiology Authors: Pia Dahlberg Karl-Jonas Axelsson Annika Rydberg Gunilla Lundahl Lennart Gransberg Lennart Bergfeldt Source Type: research

Impact of antipsychotics and antidepressants drugs on long QT syndrome induction related to hERG channel dysfunction: A systematic review
Biochem Biophys Res Commun. 2023 Sep 23;681:90-96. doi: 10.1016/j.bbrc.2023.09.043. Online ahead of print.ABSTRACTPURPOSE: QT prolongation is one of the main unwanted cardiac effects caused by drugs, such as anti-psychotics and anti-depressants, inducing mainly via hERG channel dysfunction. The precise and underlying mechanism of adverse effects on hERG channel are still indecisive, but these effects limit their use in patients with cardiac risk factors. The aim of this review was studying mechanism of Long-term QT syndrome induction via hERG channel dysfunction by these Drugs.METHOD: Search was performed in PubMed, and Sc...
Source: Biochemical and Biophysical Research communications - September 29, 2023 Category: Biochemistry Authors: Marzieh Nemati Zahra Hosseinzadeh Fatemeh Nemati Bahareh Ebrahimi Source Type: research

Impact of antipsychotics and antidepressants drugs on long QT syndrome induction related to hERG channel dysfunction: A systematic review
Biochem Biophys Res Commun. 2023 Sep 23;681:90-96. doi: 10.1016/j.bbrc.2023.09.043. Online ahead of print.ABSTRACTPURPOSE: QT prolongation is one of the main unwanted cardiac effects caused by drugs, such as anti-psychotics and anti-depressants, inducing mainly via hERG channel dysfunction. The precise and underlying mechanism of adverse effects on hERG channel are still indecisive, but these effects limit their use in patients with cardiac risk factors. The aim of this review was studying mechanism of Long-term QT syndrome induction via hERG channel dysfunction by these Drugs.METHOD: Search was performed in PubMed, and Sc...
Source: Cell Research - September 29, 2023 Category: Cytology Authors: Marzieh Nemati Zahra Hosseinzadeh Fatemeh Nemati Bahareh Ebrahimi Source Type: research