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early repolarization syndrome

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https://www.readbyqxmd.com/read/28685701/the-role-of-quinidine-in-the-pharmacological-therapy-of-ventricular-arrhythmias-quinidine
#1
Milica Bajcetic, Teodora Vidonja Uzelac, Aleksandra Kezic, Milica Bajcetic
BACKGROUND: Historically, quinidine was the first medicine used in the therapy of heart arrhythmias. Studies in the early 20th century identified quinidine, a diastereomer of the antimalarial quinine, as the most potent of the antiarrhythmic substances extracted from the cinchona plant. Quinidine is used by the 1920s, as an antiarrhythmic agent to maintain sinus rhythm after conversion from atrial flutter or atrial fibrillation and to prevent recurrence of ventricular tachycardia or ventricular fibrillation...
July 7, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28685698/medical-therapy-for-long-qt-syndrome
#2
George Adamos, Nicoletta Iacovidou, Theodoros Xanthos
Long QT syndrome (LQTS) is an arrhythmogenic disorder characterized by repolarization abnormalities with a propensity to cause life threatening cardiac events. The first manifestation of the syndrome may be sudden death, therefore, early diagnosis and therapy is of great importance. LQTS can be both congenital and acquired. The latter is most commonly seen in hospitalized patients and such individuals have an easily recognizable and reversible precipitating factor (electrolyte disturbances, certain drugs etc...
July 7, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28619993/low-extracellular-potassium-prolongs-repolarization-and-evokes-early-afterdepolarization-in-human-induced-pluripotent-stem-cell-derived-cardiomyocytes
#3
Jukka Kuusela, Kim Larsson, Disheet Shah, Chandra Prajapati, Katriina Aalto-Setälä
Long QT syndrome (LQTS) is characterized by a prolonged QT-interval on electrocardiogram and by increased risk of sudden death. One of the most common and potentially life-threatening electrolyte disturbances is hypokalemia, characterized by low concentrations of K(+) Using a multielectrode array platform and current clamp technique, we investigated the effect of low extracellular K(+) concentration ([K(+)]Ex) on the electrophysiological properties of hiPSC-derived cardiomyocytes (CMs) generated from a healthy control subject (WT) and from two symptomatic patients with type 1 of LQTS carrying G589D (LQT1A) or IVS7-2A>G mutation (LQT1B) in KCNQ1 The baseline prolongations of field potential durations (FPDs) and action potential durations (APDs) were longer in LQT1-CMs than in WT-CMs...
June 15, 2017: Biology Open
https://www.readbyqxmd.com/read/28606656/divergent-antiarrhythmic-effects-of-resveratrol-and-piceatannol-in-a-whole-heart-model-of-long-qt-syndrome
#4
Christian Ellermann, Julian Wolfes, Simon Kochhäuser, Dirk G Dechering, Florian Reinke, Kristina Wasmer, Lars Eckardt, Gerrit Frommeyer
BACKGROUND: The polyphenol resveratrol and its metabolite piceatannol have beneficial health effects including antiarrhythmic properties in ischemia/reperfusion. The objective of this study was to determine potential antiarrhythmic effects in acquired long-QT-syndrome (LQTS). METHODS AND RESULTS: 26 rabbit hearts were isolated and Langendorff-perfused. The IKr-blocker sotalol (100μM) was infused to mimic LQTS-2. Hearts were assigned to two groups. Sotalol significantly prolonged action potential duration (APD90) and QT-interval in both groups (group 1:APD90: +18ms, p<0...
June 3, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28569435/the-new-kids-on-the-block-of-arrhythmogenic-disorders-short-qt-syndrome-and-early-repolarization
#5
REVIEW
Andrea Mazzanti, Katherine Underwood, Dmitriy Nevelev, Shanna Kofman, Silvia G Priori
Short QT syndrome (SQTS) is one of the rarest inheritable cardiac channelopathies, characterized by an accelerated cardiac repolarization, which is also the substrate for the development of life-threatening ventricular arrhythmias. Up to this date, fewer than 200 SQTS cases have been reported in the literature worldwide. Patients with SQTS may experience a cardiac arrest as early as in the neonatal period or as late as 80 years old. The cumulative probability of experiencing a cardiac arrest by the fifth decade of life approaches 40%, highlighting the importance of early recognition and management...
June 1, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28541470/progressive-increase-of-the-tpeak-tend-interval-is-associated-with-ischaemia-induced-ventricular-fibrillation-in-a-porcine-myocardial-infarction-model
#6
Jan E Azarov, Marina M Demidova, Sasha Koul, Jesper van der Pals, David Erlinge, Pyotr G Platonov
Aims: Repolarization indices of ECG have been widely assessed as predictors of ventricular arrhythmias. However, little is known of the dynamic changes of these parameters during continuous monitoring in acute ischaemic episodes. The objective of the study was to evaluate repolarization-related predictors of ventricular fibrillation (VF) during progression of experimental myocardial infarction. Methods and results: Myocardial infarction was induced in 27 pigs by 40-min balloon inflation in the left anterior descending coronary artery, and 12-lead ECG was continuously recorded...
May 24, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28493952/a-mutation-in-the-cacna1c-gene-leads-to-early-repolarization-syndrome-with-incomplete-penetrance-a-chinese-family-study
#7
Xin Liu, Yang Shen, Jinyan Xie, Huihui Bao, Qing Cao, Rong Wan, Xiaoming Xu, Hui Zhou, Lin Huang, Zhenyan Xu, Wengen Zhu, Jinzhu Hu, Xiaoshu Cheng, Kui Hong
BACKGROUND: Early repolarization syndrome (ERS) may be a near-Mendelian or an oligogenic disease; however, no direct evidence has been provided to support this theory. METHODS AND RESULTS: We described a large Chinese family with nocturnal sudden cardiac death induced by ERS in most of the young male adults. One missense mutation (p.Q1916R) was found in the major subunit of the L-type calcium channel gene CACNA1C by the direct sequencing of candidate genes. A concomitant gain-of-function variant in the sodium channel gene SCN5A (p...
2017: PloS One
https://www.readbyqxmd.com/read/28491706/radiofrequency-catheter-ablation-for-treatment-of-premature-ventricular-contractions-triggering-ventricular-fibrillation-from-the-right-ventricular-outflow-tract-in-a-patient-with-early-repolarization-syndrome
#8
Jun Kakihara, Masahiko Takagi, Yusuke Hayashi, Hiroaki Tatsumi, Atsushi Doi, Minoru Yoshiyama
No abstract text is available yet for this article.
July 2016: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28490369/erratum-to-%C3%A2-novel-trigenic-cacna1c-des-mypn-mutations-in-a-family-of-hypertrophic-cardiomyopathy-with-early-repolarization-and-short-qt-syndrome
#9
Yanhong Chen, Hector Barajas-Martinez, Dongxiao Zhu, Xihui Wang, Chonghao Chen, Ruijuan Zhuang, Jingjing Shi, Xueming Wu, Yijia Tao, Weidong Jin, Xiaoyan Wang, Dan Hu
No abstract text is available yet for this article.
May 11, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28436550/epicardial-brugada-syndrome-ablation-unmasking-inferior-j-waves
#10
Adam Lee, Heidi Kohler, Daniel Wright, Haris M Haqqani
Patients with Brugada syndrome are at risk of life threatening ventricular arrhythmias. Epicardial substrate ablation for Brugada syndrome has been described as a means of controlling these arrhythmias and recent reports describe elimination of the Brugada phenotype with ablation. We describe a unique case in which a patient developed inferior J waves with an early repolarization-type electrocardiogram following successful epicardial infundibular substrate ablation (which eliminated the Brugada syndrome ECG on ajmaline challenge)...
April 24, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28427417/novel-trigenic-cacna1c-des-mypn-mutations-in-a-family-of-hypertrophic-cardiomyopathy-with-early-repolarization-and-short-qt-syndrome
#11
Yanhong Chen, Hector Barajas-Martinez, Dongxiao Zhu, Xihui Wang, Chonghao Chen, Ruijuan Zhuang, Jingjing Shi, Xueming Wu, Yijia Tao, Weidong Jin, Xiaoyan Wang, Dan Hu
BACKGROUND: Hypertrophic cardiomyopathy (HCM) patients with early repolarization (ER) pattern are at higher risk of ventricular arrhythmia, yet the genetic background of this situation has not been well investigated. Here we report novel trigenic mutations detected in a Chinese family of obstructive HCM with ER and short QT syndrome (SQTS). METHODS: Proband and family members underwent detailed medical assessments. DNAs were extracted from peripheral blood leukocytes for genetic screening with next generation method...
April 20, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28367598/early-repolarization-in-normal-adolescents-is-common
#12
Humera Ahmed, Richard J Czosek, David S Spar, Timothy K Knilans, Jeffrey B Anderson
In adults with structurally normal hearts, the early repolarization pattern (ERP) on electrocardiogram (ECG) may be associated with an increased risk of sudden cardiac death. The prevalence and significance of the ERP in children is unknown. This study examines the prevalence of the ERP, the population in which it is found, and whether there exists any correlation with increased LV mass or family history of significant cardiac events. This was a secondary review of data obtained from healthy adolescents undergoing a limited ECG and transthoracic echocardiogram (TTE) as part of a cardiac screening study...
April 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28343764/temporary-removal-channelopathies-genetic-testing-and-risk-stratification
#13
Arthur A M Wilde, Ahmad Amin
The publisher regrets that this article has been temporarily removed. A replacement will appear as soon as possible in which the reason for the removal of the article will be specified, or the article will be reinstated. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.
March 18, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28341588/targeted-next-generation-sequencing-of-51-genes-involved-in-primary-electrical-disease
#14
Dorien Proost, Johan Saenen, Geert Vandeweyer, Annelies Rotthier, Maaike Alaerts, Emeline M Van Craenenbroeck, Joachim Van Crombruggen, Geert Mortier, Wim Wuyts, Christiaan Vrints, Jurgen Del Favero, Bart Loeys, Lut Van Laer
Primary electrical disease (PED) is characterized by cardiac arrhythmias, which can lead to sudden cardiac death in the absence of detectable structural heart disease. PED encompasses a diversity of inherited syndromes, predominantly Brugada syndrome, early repolarization syndrome, long QT syndrome, short QT syndrome, arrhythmogenic right ventricular cardiomyopathy, and catecholaminergic polymorphic ventricular tachycardia. To overcome the diagnostic challenges imposed by the clinical and genetic heterogeneity of PED, we developed a targeted gene panel for next-generation sequencing of 51 PED genes...
May 2017: Journal of Molecular Diagnostics: JMD
https://www.readbyqxmd.com/read/28340026/high-rate-of-subcutaneous-implantable-cardioverter-defibrillator-sensing-screening-failure-in-patients-with-brugada-syndrome-a-comparison-with-other-inherited-primary-arrhythmia-syndromes
#15
Giulio Conte, Mihoko Kawabata, Carlo de Asmundis, Erika Taravelli, Francesco Petracca, Diego Ruggiero, Maria Luce Caputo, François Regoli, Gian-Battista Chierchia, Alessandra Chiodini, Alessandro Del Bufalo, Tiziano Moccetti, Masahiko Goya, Kenzo Hirao, Alessandro Vicentini, Gaetano M De Ferrari, Pedro Brugada, Angelo Auricchio
Aims: Subcutaneous implantable cardioverter-defibrillator (S-ICD) can avoid important complications associated with transvenous leads in patients with inherited primary arrhythmia syndromes, who do not need pacing therapy. Few data are available on the percentage of patients with inherited arrhythmia syndromes eligible for S-ICD implantation. Aim of this study was to analyse the eligibility for S-ICD in a series of patients with Brugada syndrome (BrS), and to compare it with patients with other channelopathies...
March 7, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28274576/long-term-follow-up-of-probands-with-brugada-syndrome
#16
Carlo de Asmundis, Giacomo Mugnai, Gian-Battista Chierchia, Juan Sieira, Giulio Conte, Moises Rodriguez-Mañero, Gudrun Pappaert, Jens Czapla, Jan Nijs, Mark La Meir, Ruben Casado, Erwin Ströker, Valentina De Regibus, Pedro Brugada
This study analyzes the natural history of a large cohort of probands with Brugada syndrome (BrS) to assess the predictive value of different clinical and electrocardiographic parameters for the development of ventricular fibrillation (VF) or sudden cardiac death (SCD) during a long-term follow-up. Baseline characteristics of 289 consecutive probands (203 men; mean age 45 ± 16 years) with a Brugada type 1 electrocardiogram were analyzed. After a mean follow-up of 10.1 ± 4.6 years, 29 malignant arrhythmias occurred...
May 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28184198/local-left-ventricular-epicardial-j-waves-and-late-potentials-in-brugada-syndrome-patients-with-inferolateral-early-repolarization-pattern
#17
Satoshi Nagase, Masamichi Tanaka, Hiroshi Morita, Koji Nakagawa, Tadashi Wada, Masato Murakami, Nobuhiro Nishii, Kazufumi Nakamura, Hiroshi Ito, Tohru Ohe, Kengo F Kusano
Background: Brugada syndrome (BrS) is characterized by J-point or ST-segment elevation on electrocardiograms (ECGs) and increased risk of ventricular fibrillation (VF). In BrS, epicardial depolarization abnormality with delayed potential on the right ventricular outflow tract is reportedly the predominant mechanism underlying VF. Yet VF occurrence is also associated with early repolarization (ER) pattern in the inferolateral ECG leads, which may represent the inferior and/or left lateral ventricular myocardium...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28087596/a-meta-analysis-on-the-prognostic-significance-of-inferolateral-early-repolarization-pattern-in-brugada-syndrome
#18
Stamatis Georgopoulos, Konstantinos P Letsas, Tong Liu, Maria Kalafateli, Panagiotis Korantzopoulos, Gerd Bürkle, Konstantinos Vlachos, Georgios Giannopoulos, Michael Efremidis, Spyridon Deftereos, Antonios Sideris, Masahiko Takagi, Gan-Xin Yan, Joachim R Ehrlich
AIMS: The early repolarization (ER) pattern has been linked to an increased risk for arrhythmic death in various clinical settings. There are limited and conflicting data regarding the prognostic significance of ER pattern in Brugada syndrome (BS). The aim of this meta-analysis was to provide a detailed analysis of the currently available studies regarding the arrhythmic risk in patients with BS and ER pattern. METHODS AND RESULTS: Current databases were searched until May 2015...
January 13, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28077816/incidence-of-atrial-tachyarrhythmias-in-patients-with-early-repolarization-syndrome
#19
Ki Won Hwang, Gi-Byoung Nam, Junhee Han, Yong Giun Kim, Hyung Oh Choi, Jun Kim, Kee-Joon Choi, You-Ho Kim
Atrial tachyarrhythmias (ATAs) occur in a significant proportion of Brugada syndrome (BrS) patients and are often an important cause of inappropriate shocks. The aim of this retrospective study was to evaluate the incidence of ATAs and ATA-induced inappropriate shocks in early repolarization syndrome (ERS) patients as compared to BrS patients.We analyzed data from 20 consecutive patients who were diagnosed with ERS and compared them with patients diagnosed with BrS (n = 31). Clinical and ICD interrogation data were collected and analyzed for all events with ICD shocks...
February 7, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28000227/variants-of-brugada-syndrome-and-early-repolarization-syndrome-an-expanded-concept-of-j-wave-syndrome
#20
Sung-Hwan Kim, Gi-Byoung Nam, Sung-Cheol Yun, Hyung Oh Choi, Kee-Joon Choi, Boyoung Joung, Hui-Nam Pak, Moon-Hyoung Lee, Sung Soon Kim, Seung-Jung Park, Young Keun On, June Soo Kim, Il-Young Oh, Eue-Keun Choi, Seil Oh, Yun-Shik Choi, Jong Il Choi, Sang Weon Park, Young-Hoon Kim, Yong-Seog Oh, Man Young Lee, Hong Euy Lim, Young-Soo Lee, Yongkeun Cho, Jun Kim, Kyoung-Suk Rhee, Dong-Il Lee, Dae Kyoung Cho, You-Ho Kim
BACKGROUND: The role of J-waves in the pathogenesis of ventricular fibrillation (VF) occurring in structurally normal hearts is important. METHODS: We evaluated 127 patients who received an implantable cardioverter-defibrillator (ICD) for Brugada syndrome (BS, n = 53), early repolarization syndrome (ERS, n = 24), and patients with unknown or deferred diagnosis (n = 50). Electrocardiography (ECG), clinical characteristics, and ICD data were analyzed. RESULTS: J-waves were found in 27/50 patients with VF of unknown/deferred diagnosis...
February 2017: Pacing and Clinical Electrophysiology: PACE
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