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early repolarization syndrome

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https://www.readbyqxmd.com/read/28436550/epicardial-brugada-syndrome-ablation-unmasking-inferior-j-waves
#1
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
#2
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
#3
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/channelopathies-genetic-testing-and-risk-stratification
#4
Arthur A M Wilde, Ahmad Amin
The cardiac channelopathies are a group of diseases with (disease-) specific electrocardiographic (ECG) characteristics and a disease-specific risk of sudden cardiac death (SCD). This group includes the Long QT Syndromes (LQTS), Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT), Brugada Syndrome (BrS), Short QT Syndromes (SQTS), and Early Repolarization Syndrome (ERS). In the past 2 decades the genetic basis for these disease entities has largely been unraveled and that, together with the identification of the genetic basis of the cardiomyopathies, has paved the way for the complete new field of Cardiogenetics...
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/27968776/the-electrocardiogram-of-the-neonate-and-infant
#12
Konrad Brockmeier, Rewa Nazal, Narayanswami Sreeram
The ECG in children has a number of characteristic differences compared to the ECG of the adult. The transition of the ECG in neonates after birth represents dynamic changes of the circulatory system due to the postnatal adaptation, different physiologic properties of the fetal and neonatal myocardium, the location and orientation of the heart in the chest and influence of body mass during that period and later on in childhood. The complexity of the changes implies a broad variation of ECG changes during the first days and weeks of life, whose interpretation requires expert knowledge...
November 2016: Journal of Electrocardiology
https://www.readbyqxmd.com/read/27965319/high-prevalence-of-early-repolarization-in-the-paediatric-relatives-of-sudden-arrhythmic-death-syndrome-victims-and-in-normal-controls
#13
Anna McCorquodale, Rachel Poulton, Jennifer Hendry, Gabrielle Norrish, Ella Field, Sarah Mead-Regan, Martin Lowe, Juan Pablo Kaski
AIMS: Elevation of the ECG J-point in the inferior and lateral leads (early repolarization) has been described in survivors of ventricular fibrillation (VF) arrest and occurs in adult first-degree relatives of sudden cardiac death (SCD) probands at a frequency significantly greater than in controls, raising the possibility that this could represent an independent risk factor in the aetiology of SCD. However, data on early repolarization in the paediatric population are lacking. This study aimed to assess the prevalence of early repolarization in paediatric first-degree relatives of sudden arrhythmic death syndrome (SADS) victims...
December 13, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/27890733/differences-in-the-onset-mode-of-ventricular-tachyarrhythmia-between-patients-with-j-wave-in-anterior-leads-and-those-with-j-wave-in-inferolateral-leads
#14
Tsukasa Kamakura, Mitsuru Wada, Kohei Ishibashi, Yuko Y Inoue, Koji Miyamoto, Hideo Okamura, Satoshi Nagase, Takashi Noda, Takeshi Aiba, Satoshi Yasuda, Wataru Shimizu, Shiro Kamakura, Kengo Kusano
BACKGROUND: The pathophysiological mechanism of J wave in anterior leads (A-leads) and inferolateral leads (L-leads) remains unclear. OBJECTIVE: We investigated the onset mode and circadian distribution of ventricular tachyarrhythmia (VTA) episodes between patients with early repolarization syndrome (ERS) and Brugada syndrome (BrS). METHODS: The study enrolled 35 patients with ERS and 52 patients with type 1 BrS with spontaneous ventricular fibrillation who were divided into 4 groups: ERS(A+L) (n = 15), patients with ERS who had a non-type 1 Brugada pattern electrocardiogram in any A-leads (second to fourth intercostal spaces) in control and/or after drug provocation tests; ERS(L) (n = 20), patients with ERS with J wave only in L-leads; BrS(A) (n = 24), patients with BrS without J wave in L-leads; and BrS(A+L) (n = 28), patients with BrS with J wave in L-leads...
November 23, 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27885745/exaggerated-reactivity-of-parasympathetic-nerves-is-involved-in-ventricular-fibrillation-in-j-wave-syndrome
#15
Tetsuji Shinohara, Hidekazu Kondo, Toyokazu Otsubo, Akira Fukui, Kunio Yufu, Mikiko Nakagawa, Naohiko Takahashi
INTRODUCTION: Brugada syndrome (BrS) and early repolarization syndrome (ERS) are termed the J-wave syndrome. In most cases of J-wave syndrome, ventricular fibrillation (VF) often occurs around midnight or in the early morning when parasympathetic tone is augmented. OBJECTIVE: The purpose of this study was to clarify the relationship between VF and autonomic nervous activity in patients with J-wave syndrome. METHODS AND RESULTS: We enrolled 28 consecutive patients with J-wave syndrome (20 BrS and 8 ERS) in whom implantable cardioverter defibrillators (ICDs) were implanted between January 2002 and December 2014...
November 25, 2016: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/27830074/sodium-channel-blockade-unmasked-brugada-electrocardiographic-pattern-in-a-patient-with-complete-right-bundle-branch-block-and-early-repolarization-in-the-lateral-leads
#16
Shozo Konishi, Hitoshi Minamiguchi, Yuji Okuyama, Yasushi Sakata
Early repolarization syndrome (ERS) and Brugada syndrome (BrS) share many electrocardiographic and clinical features, and recently have been collectively grouped as J wave syndrome. However, the effects of sodium channel blockers on the J waves differ greatly between ERS and BrS.
November 2016: Clinical Case Reports
https://www.readbyqxmd.com/read/27765749/spontaneous-initiation-of-premature-ventricular-complexes-and-arrhythmias-in-type-2-long-qt-syndrome
#17
Xiaodong Huang, Tae Yun Kim, Gideon Koren, Bum-Rak Choi, Zhilin Qu
The occurrence of early afterdepolarizations (EADs) and increased dispersion of repolarization are two known factors for arrhythmogenesis in long QT syndrome. However, increased dispersion of repolarization tends to suppress EADs due to the source-sink effect, and thus how the two competing factors cause initiation of arrhythmias remains incompletely understood. Here we used optical mapping and computer simulation to investigate the mechanisms underlying spontaneous initiation of arrhythmias in type 2 long QT (LQT2) syndrome...
December 1, 2016: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/27702859/antiarrhythmic-effect-of-vernakalant-in-an-experimental-model-of-long-qt-syndrome
#18
Gerrit Frommeyer, Catharina Clauss, Christian Ellermann, Harilaos Bogossian, Dirk G Dechering, Simon Kochhäuser, Florian Reinke, Christian Pott, Lars Eckardt
AIMS: The antiarrhythmic drug vernakalant exerts antiarrhythmic effects in atrial fibrillation. Recent experimental data suggest interactions with the late sodium current and antiarrhythmic effects in ventricular arrhythmias. We aimed at investigating whether treatment with vernakalant reduces polymorphic ventricular tachycardia (VT) in an experimental model of Long-QT-syndrome (LQTS). METHODS AND RESULTS: Twenty-nine isolated rabbit hearts were assigned to two groups and treated with erythromycin (300 µM, n = 15) or veratridine (0...
October 4, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/27686029/efficacy-and-safety-of-dextrose-insulin-in-unmasking-non-diagnostic-brugada-ecg-patterns
#19
Enrique Velázquez-Rodríguez, Horacio Rodríguez-Piña, Alex Pacheco-Bouthillier, Marcelo Paz Jiménez-Cruz
BACKGROUND: Typical diagnostic, coved-type 1, Brugada ECG patterns fluctuate spontaneously over time with a high proportion of non-diagnostic ECG patterns. Insulin modulates ion transport mechanisms and causes hyperpolarization of the resting potential. We report our experience with unmasking J-ST changes in response to a dextrose-insulin test. METHODS: Nine patients, mean age 40.5±19.4years (range: 15-65years), presented initially with a non-diagnostic ECG pattern, which was suggestive of Brugada syndrome (group I)...
November 2016: Journal of Electrocardiology
https://www.readbyqxmd.com/read/27662776/validation-of-the-vessel-specific-leads-vsls-for-detection-of-acute-ischemia-on-a-dataset-with-non-ischemic-st-segment-deviation
#20
John J Wang, Olle Pahlm, Galen S Wagner, James W Warren, B Milan Horáček, John L Sapp
BACKGROUND: Existing criteria recommended by ACC/ESC for identifying patients with ST elevation myocardial infarction (STEMI) from the 12-lead ECG perform with high specificity (SP) but low sensitivity (SE). In our previous studies, we found that the SE of acute ischemia detection can be markedly improved without any loss of SP by calculating, from the 12-lead ECG, ST deviation in 3 "optimal" vessel-specific leads (VSLs). To further validate the method, we evaluated the SP performance using a dataset with non-ischemic ST-segment changes...
November 2016: Journal of Electrocardiology
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