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("ECG" OR "EKG") AND "Long QT Syndrome"

Alaa Alahmadi, Alan Davies, Markel Vigo, Caroline Jay
OBJECTIVE: The study sought to quantify a layperson's ability to detect drug-induced QT interval prolongation on an electrocardiogram (ECG) and determine whether the presentation of the trace affects such detection. MATERIALS AND METHODS: Thirty layperson participants took part in a psychophysical and eye-tracking experiment. Following training, participants completed 21 experimental trials, in which each trial consisted of 2 ECGs (a baseline and a comparison stimulus, both with a heart rate of 60 beats/min)...
March 8, 2019: Journal of the American Medical Informatics Association: JAMIA
Ryan Wereski, Richard Dobson, Edward J Newman, Derek Connelly
Diagnosis of inherited arrhythmia syndromes, including long-QT syndrome (LQTS), is challenging; however, early detection and initiation of therapies can reduce otherwise high rates of mortality. Two months following the birth of her first child a previously well 21-year-old female experienced four episodes of transient loss of consciousness (TLOC). The history was atypical for seizures but a video electroencephalogram (EEG) captured an episode with abnormal bifrontal epileptic discharge. She was commenced on levetiracetam...
March 2019: Journal of the Royal College of Physicians of Edinburgh
János Tomcsányi, Kristóf Tomcsányi
Authors report the case of a patient with drug-induced long QT syndrome. This case highlights the importance of ECG signs of LQTS that may lead to torsade de pointes tachycardia. The patient received the QT prolonging moxifloxacine and the QT remained long even after the offending drug was discontinued. Orv Hetil. 2018; 159(39): 1607-1610.
September 2018: Orvosi Hetilap
Dafni Charisopoulou, George Koulaouzidis, Annika Rydberg, Henein Y Michael
BACKGROUND: Electromechanical (EM) coupling heterogeneity is significant in long QT syndrome (LQTS), particularly in symptomatic patients; EM window (EMW) has been proposed as an indicator of interaction and a better predictor of arrhythmia than QTc. AIM: To investigate the dynamic response of EMW to exercise in LQTS and its predictive value of arrhythmia. METHODS: Forty-seven LQTS carriers (45±15 years, 20 with arrhythmic events) and 35 controls underwent exercise echocardiogram...
December 9, 2018: Clinical Cardiology
Guoliang Li, Li Zhang
Congenital long QT syndrome (LQTS) is a hereditary cardiac disorder characterized by QT-interval prolongation and T-wave abnormalities on electrocardiogram (ECG), and is associated with an increased risk of torsade de pointes and sudden cardiac death. Beta-blocker medication is effective in most patients except those with a very slow heart rate. Increased late sodium currents (INa-L) can result in bradycardia-dependent QT prolongation. Mexiletine, an inhibitor of INa-L, is not only effective in treating type-3 LQTS, but also shows the promise in managing LQTS patients of other genotypes with markedly prolonged QT interval at slow heart rates...
November 2018: Journal of Electrocardiology
Ben J M Hermans, Job Stoks, Frank C Bennis, Arja S Vink, Ainara Garde, Arthur A M Wilde, Laurent Pison, Pieter G Postema, Tammo Delhaas
Aims: Diagnosing long QT syndrome (LQTS) is challenging due to a considerable overlap of the QTc-interval between LQTS patients and healthy controls. The aim of this study was to investigate the added value of T-wave morphology markers obtained from 12-lead electrocardiograms (ECGs) in diagnosing LQTS in a large cohort of gene-positive LQTS patients and gene-negative family members using a support vector machine. Methods and results: A retrospective study was performed including 688 digital 12-lead ECGs recorded from genotype-positive LQTS patients and genotype-negative relatives at their first visit...
November 1, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Y D Li, Maimaitimin Maimaitiabudula, X H Zhou, Y M Lu, J H Zhang, Q Xing, B P Tang
Objective: Present study analyzed the association betwen the postassium voltage-gated channel KQT-like subfamily member 1 gene (KCNQ1) mutation and the clinical and the electrocardiographic features in 2 pedigrees with congenital long QT syndrome type 1 (LQT1) in Xinjiang Uygur Autonomous Region. Methods: Three family members were diagnosed as LQT1 patients in 2 Uygur congenital LQT1 families, these 3 LQT1 patients served as long QT group, 24 Uygur healthy volunteers served as control group. Electrocardiogram (ECG) and the gene detection were applied to compare the ECG and molecular genetic features between the long QT group and control group, and to explore the relationship between the KCNQ1 gene mutation and the clinical and the electrocardiographic features in these 2 families with congenital long QT syndrome type 1...
November 24, 2018: Zhonghua Xin Xue Guan Bing za Zhi
Jonathan R Skinner, Annika Winbo, Dominic Abrams, Jitendra Vohra, Arthur A Wilde
Forty per cent (40%) of sudden unexpected natural deaths in people under 35 years of age are associated with a negative autopsy, and the cardiac ion channelopathies are the prime suspects in such cases. Long QT syndrome (LQTS), Brugada syndrome (BrS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are the most commonly identified with genetic testing. The cellular action potential driving the heart cycle is shaped by a specific series of depolarising and repolarising ion currents mediated by ion channels...
October 4, 2018: Heart, Lung & Circulation
Kun Li, Jing Yang, Wenjia Guo, Tingting Lv, Jihong Guo, Jianfeng Li, Ping Zhang
Long QT syndrome is a rare but potentially lethal cardiac channelopathy. The primary aim of the study was to investigate the long-term effects of video-assisted thoracoscopic (VATS) left cardiac sympathetic denervation (LCSD) in Chinese patients with long QT syndrome.VATS-LCSD was performed in eight Chinese patients with LQTS. Twelve-lead ECGs and 24-hour Holter monitoring ECGs were recorded before and after surgery. The medical charts were reviewed to obtain patient data, and the patients who had been lost to follow-up were contacted through telephone...
October 25, 2018: International Heart Journal
Charlotte Gibbs, Jacob Thalamus, Kristian Tveten, Øyvind L Busk, Jan Hysing, Kristina H Haugaa, Øystein L Holla
Background Congenital long- QT syndrome ( LQTS ) is a genetic disorder characterized by prolongation of the corrected QT interval ( QT c) on an ECG . The aim of the present study was to estimate the prevalence of pathogenic and likely pathogenic sequence variants in patients who had at least 1 ECG with a QT c ≥500 ms. Methods and Results Telemark Hospital Trust is a community hospital within the Norwegian national health system, serving ≈173 000 inhabitants. We searched the ECG database at Telemark Hospital Trust, Norway, from January 2004 to December 2014, and identified 1531 patients with at least 1 ECG with a QT c ≥500 ms...
August 21, 2018: Journal of the American Heart Association
Małgorzata Szperl, Urszula Kozicka, Agnieszka Kosiec, Piotr Kukla, Marta Roszczynko, Elżbieta Katarzyna Biernacka
Congenital long QT syndrome (LQTS) is a primary cardiac channelopathy. Genetic testing has not only diagnostic but also prognostic and therapeutic implications. At present, 15 genes have been associated with the disease, with most mutations located in 3 major LQTS-susceptibility genes. During a routine genetic screening for KCNQ1, KCNH2 and SCN5A genes in index cases with LQTS, seven novel variants in KCNH2 and SCN5A genes were found. Genotype-phenotype correlations were analysed in these patients and their families...
November 2018: Journal of Applied Genetics
Victoria Scott-Warren, Anju Bendon, Iain A Bruce, Lise Henderson, Jacques Diacono
Congenital long QT syndrome (cLQTS) is an inherited cardiac ion channelopathy characterized by a long corrected-QT interval on the ECG, associated with a risk of syncope and sudden death as a result of arrhythmias. The archetypal arrhythmia associated with cLQTS is torsade de pointes which may degenerate into ventricular fibrillation. Children with Jervell and Lange-Neilsen syndrome have the combination of cLQTS and congenital sensorineural deafness and may present for cochlear implantation (CI). Sympathetic stimulation and administration of QT-prolonging medications may trigger arrhythmias in children with cLQTS and thus the perioperative period is a time of increased risk of adverse events, with deaths reported in the CI literature...
November 2018: Cochlear Implants International
Nikhil Ahluwalia, Hariharan Raju
PURPOSE OF REVIEW: Pre-participation athlete screening has led to the referral of asymptomatic athletes with a prolonged QT interval warranting their evaluation for long QT syndrome (LQTS). Establishing a diagnosis of LQTS can be difficult, particularly in asymptomatic athletes presenting with a prolonged QTc < 500 ms. This review examines the evaluatory pathway to ascertain the common pitfalls leading to mis- or overdiagnosis. We discuss the advanced ECG-based tools and consider their application in the diagnostic process...
August 27, 2018: Current Treatment Options in Cardiovascular Medicine
Sherif Gouda, Mohammed Q Saif, Mohammed Shabana, Sameh Salama, Ahmed Eldamaty
BACKGROUND: Jervell and Lange-Nielsen syndrome is an autosomal recessive form of long QT syndrome (LQTS), clinically manifested by long QT interval and bilateral sensorineural hearing loss (SNHL) with the highest prevalence in Norway and Sweden. No data are available about the prevalence of such syndrome in Egypt. OBJECTIVES: This study aimed to assess by electrocardiogram (ECG) the prevalence of LQTS among Egyptian children with SNHL. METHODS: One thousand and twelve patients, aged ≤ 10 years (mean age 5...
August 22, 2018: Pacing and Clinical Electrophysiology: PACE
Daisuke Hazeki, Yumiko Ninomiya, Kentaro Ueno, Masao Yoshinaga
BACKGROUND: While the prevalence of short QT syndrome (SQTS) in children and adolescents is low, early detection is important because SQTS can cause life-threatening arrhythmia. The aim of this study was to determine the tentative screening criteria for short QT interval in children and adolescents. Methods and Results: A total of 75,040 digitally stored electrocardiograms (ECG) of participants in a school-based ECG screening program were obtained between 2009 and 2013 in Kagoshima, Japan...
September 25, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
Yoshihiro Tanaka, Kenshi Hayashi, Noboru Fujino, Tetsuo Konno, Hayato Tada, Chiaki Nakanishi, Akihiko Hodatsu, Toyonobu Tsuda, Yoji Nagata, Ryota Teramoto, Shohei Yoshida, Akihiro Nomura, Masa-Aki Kawashiri, Masakazu Yamagishi
Heterologous expression systems play a vital role in the characterization of potassium voltage-gated channel subfamily H member 2 (KCNH2) gene mutations, such as E637K which is associated with long QT syndrome type 2 (LQT2). In vivo assays using zebrafish provide a means for testing genetic variants of cardiac disease; however, limited information on the role of the E637K mutation is available from in vivo systems and their utility has yet to be fully exploited in the context of LQT2. We sought to evaluate the ability of the E637K mutant channel to restore normal repolarization in larval zebrafish with a human KCNH2 orthologue, kcnh2a-knockdown...
July 25, 2018: Heart and Vessels
David Ziupa, Marius Menza, Susanne Koppermann, Robin Moss, Julia Beck, Gerlind Franke, Stefanie Perez Feliz, Michael Brunner, Sonja Mayer, Heiko Bugger, Gideon Koren, Manfred Zehender, Bernd A Jung, Gunnar Seemann, Daniela Foell, Christoph Bode, Katja E Odening
BACKGROUND: Prolonged repolarization is the hallmark of long QT syndrome (LQTS), which is associated with subclinical mechanical dysfunction. We aimed at elucidating mechanical cardiac function in LQTS type 1 (loss of IKs ) and its modification upon further prolongation of the action potential (AP) by IKr -blockade (E-4031). METHODS: Transgenic LQT1 and wild type (WT) rabbits (n = 12/10) were subjected to tissue phase mapping MRI, ECG, and epicardial AP recording...
July 9, 2018: International Journal of Cardiology
Pyotr G Platonov, Scott McNitt, Bronislava Polonsky, Spencer Z Rosero, Valentina Kutyifa, Allison Huang, Arthur J Moss, Wojciech Zareba
BACKGROUND: Long-QT (LQT) syndrome mutation carriers have higher risk of cardiac events than unaffected family members even in the absence of QTc prolongation. Changes in T-wave morphology may reflect penetrance of LQT syndrome mutations. We aimed to assess whether T-wave morphology may improve risk stratification of LQT2 mutation carriers with normal QTc interval. METHODS: LQT2 mutation carriers with QTc <460 ms in men and <470 ms in women (n=154) were compared with unaffected family members (n=1007)...
July 2018: Circulation. Arrhythmia and Electrophysiology
Umile Giuseppe Longo, Laura Risi Ambrogioni, Mauro Ciuffreda, Nicola Maffulli, Vincenzo Denaro
Introduction: Sudden cardiac death (SCD) of young athletes during competition or training is a tragic event. The long QT syndrome (LQTS) is an arrythmogenic disorder characterized by prolonged ventricular repolarization leading to torsade de pointes evident at electrocardiogram (ECG). Implantable cardioverter defibrillator is an option to revert ventricular fibrillation to sinus rhythm, although the implantation may result in denial of sports participations to the athlete. The authors reviewed the current literature on LQTS in young athletes, to clarify the role of different screening technologies to prevent SCD...
September 1, 2018: British Medical Bulletin
Siddharth Narayan Gadage
Long QT syndrome (LQTS) is a congenital disorder characterized by prolongation of QT interval in the electrocardiogram (ECG) and a propensity to develop ventricular arrhythmias, which may lead to syncope, cardiac arrest or sudden death. T-wave alternans (TWA), a phenomenon of beat-to-beat variability in the repolarization phase of the ventricles, has been closely associated with an increased risk of ventricular tachyarrhythmic events (VTE) and sudden cardiac death (SCD).
May 2018: Annals of Pediatric Cardiology
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