Ten-year experience in atenolol use and exercise evaluation in children with genetically proven long QT syndrome

Conclusions Atenolol is an effective treatment for genetically proven LQT1 and LQT2 children and adolescents, with good tolerability. In LQT1 patients, QTc intervals at 2–3min into the recovery phase of exercise were significantly prolonged, particularly in patients with transmembrane mutations.
Source: Journal of Arrhythmia - Category: Cardiology Source Type: research

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In conclusion, we identified a novel hERG channel activator HW-0168 that can be used for studying the physiological role of hERG in cardiac myocytes and may be beneficial for treating long QT syndrome.
Source: Journal of Pharmacological Sciences - Category: Drugs & Pharmacology Source Type: research
Conclusions: AED reliably identifies the underlying lethal ventricular arrhythmias in addition to aborting SCD.What is Known:•Although infrequent in children, sudden cardiac death (SCD) is often an unexpected and tragic event. The etiology is diverse and sometimes remains unknown despite extensive investigations.•Automated external defibrillator (AED) is both therapeutic in aborting SCD and diagnostic in identifying the underlying lethal ventricular arrhythmias. However, the diagnostic aspect of AED is under-acknowledged by most medical providers.What is New:•Four of 25 patients (16%) were initially managed ...
Source: European Journal of Pediatrics - Category: Pediatrics Source Type: research
CONCLUSIONS: Supraphysiological oestradiol levels that occur during controlled ovarian hyperstimulation cause prolongation of QTc intervals, but not to a pathological level. Although this prolongation is not significant in healthy individuals, it might increase ventricular arrhythmia risk in patients with congenital long QT syndrome and in patients taking medication that prolongs QT. PMID: 29399762 [PubMed - indexed for MEDLINE]
Source: Kardiologia Polska - Category: Cardiology Authors: Tags: Kardiol Pol Source Type: research
Differential Diagnosis Between Catecholaminergic Polymorphic Ventricular Tachycardia and Long QT Syndrome Type 1 - Modified Schwartz Score. Circ J. 2018 Jun 21;: Authors: Ozawa J, Ohno S, Fujii Y, Makiyama T, Suzuki H, Saitoh A, Horie M Abstract BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) has been often misdiagnosed as long QT syndrome (LQTS) type 1 (LQT1), which phenotypically mimics CPVT but has a relatively better prognosis.Methods and Results:The derivation and validation cohorts consisted of 146 and 21 patients, respectively, all of whom had exercise- or emotion...
Source: Circulation Journal - Category: Cardiology Authors: Tags: Circ J Source Type: research
CONCLUSIONS: Supraphysiological estradiol levels that occur during controlled ovarian stimulation causes prolongation in QTc intervals, but this is not at pathological level. Although this prolongation is not significant in healthy individuals, it might increase ventricular arrhythmia risk in patients with congenital long QT syndrome and in patients taking medication that prolongs QT. PMID: 29399762 [PubMed - as supplied by publisher]
Source: Polish Heart Journal - Category: Cardiology Authors: Tags: Kardiol Pol Source Type: research
This study tested the hypothesis that concomitant sympathetic and parasympathetic stimulation ( “autonomic conflict”) may act as a trigger for arrhythmia in long QT syndrome (LQTS). Studies were performed in isolated innervated rabbit hearts treated with clofilium (100 nmol/L); a potassium channel blocker. The influence of vagus nerve stimulation (VNS) on spontaneous ventricular arrhythm ia was assessed in the absence/presence of sustained noradrenaline perfusion (100 nmol/L) and with sudden adrenergic stress (injections of noradrenaline into the perfusion line).
Source: Journal of Molecular and Cellular Cardiology - Category: Cytology Authors: Source Type: research
Publication date: Available online 18 May 2017 Source:Pharmacology & Therapeutics Author(s): Isik Turker, Tomohiko Ai, Hideki Itoh, Minoru Horie Since the early 1990s, the concept of primary “inherited” arrhythmia syndromes or ion channelopathies has evolved rapidly as a result of revolutionary progresses made in molecular genetics. Alterations in genes coding for membrane proteins such as ion channels or their associated proteins responsible for the generation of cardiac action potentials (AP) have been shown to cause specific malfunctions which eventually lead to cardiac arrhythmias. These arrhythmic dis...
Source: Pharmacology and Therapeutics - Category: Drugs & Pharmacology Source Type: research
This report documents a single center experience with patients who have both genetically confirmed LQTS and CHD, to examine their modes of presentation and factors associated with making the diagnosis of LQTS in this patient population, as well as potential confounding variables that may mask or delay both LQTS diagnosis and initiation of therapy.
Source: The American Journal of Cardiology - Category: Cardiology Authors: Source Type: research
Conclusions— We identify the first disease-causing ANK2 variant localized to the membrane-binding domain resulting in reduced ankyrin-B expression and abnormal localization. Further study is warranted on the potential association of this variant with structural heart disease given the role of ANK2 in targeting and stabilization of key structural and signaling molecules in cardiac cells.
Source: Circulation: Cardiovascular Genetics - Category: Cardiology Authors: Tags: Arrhythmias, Cell Biology/Structural Biology, Clinical Studies, Cardiovascular Disease, Genetics Original Articles Source Type: research
ConclusionsVATS‐LCSD was a safe, and probably effective procedure for patients with hereditary ventricular tachycardia syndrome, with no serious adverse events and with short hospital stay.This article is protected by copyright. All rights reserved
Source: Pacing and Clinical Electrophysiology : PACE - Category: Cardiology Authors: Tags: ELECTROPHYSIOLOGY Source Type: research
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