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Sudden arrhythmic death syndrome

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https://www.readbyqxmd.com/read/29145303/epicardial-radiofrequency-catheter-ablation-of-brugada-syndrome-with-electrical-storm-during-ventricular-fibrillation-a-case-report
#1
Shufen Jiang, Xiaomeng Yin, Chang Dong, Yunlong Xia, Jinqiu Liu
RATIONALE: Brugada syndrome (BrS) is characterized by ST segment elevation at the J point ≥2 mm in the right precordial electrocardiogram (ECG) leads, in the absence of structural heart disease, electrolyte disturbances, or ischemia. It is a well-described cause of sudden death in young patients, especially in the age of between 30 and 40 years old. Here, we reported an unusual case of electrical storm (ES) of ventricular fibrillation (VF) caused by BrS with complete right bundle-branch block (CRBBB) in a 75-year-old male patient...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29097318/systematic-review-for-the-2017-aha-acc-hrs-guideline-for-management-of-patients-with-ventricular-arrhythmias-and-the-prevention-of-sudden-cardiac-death-a-report-of-the-american-college-of-cardiology-american-heart-association-task-force-on-clinical-practice
#2
Fred M Kusumoto, Kent R Bailey, Ahmad Sami Chaouki, Abhishek J Deshmukh, Sandeep Gautam, Robert J Kim, Daniel B Kramer, Litsa K Lambrakos, Naseer H Nasser, Dan Sorajja
BACKGROUND: Although large randomized clinical trials have found that primary prevention use of an implantable cardioverter-defibrillator (ICD) improves survival in patients with cardiomyopathy and heart failure symptoms, patients who receive ICDs in practice are often older and have more comorbidities than patients who were enrolled in the clinical trials. In addition, there is a debate among clinicians on the usefulness of electrophysiological study for risk stratification of asymptomatic patients with Brugada syndrome...
October 25, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29097297/systematic-review-for-the-2017-aha-acc-hrs-guideline-for-management-of-patients-with-ventricular-arrhythmias-and-the-prevention-of-sudden-cardiac-death-a-report-of-the-american-college-of-cardiology-american-heart-association-task-force-on-clinical-practice
#3
Fred M Kusumoto, Kent R Bailey, Ahmad Sami Chaouki, Abhishek J Deshmukh, Sandeep Gautam, Robert J Kim, Daniel B Kramer, Litsa K Lambrakos, Naseer H Nasser, Dan Sorajja
BACKGROUND: Although large randomized clinical trials have found that primary prevention use of an implantable cardioverter-defibrillator (ICD) improves survival in patients with cardiomyopathy and heart failure symptoms, patients who receive ICDs in practice are often older and have more comorbidities than patients who were enrolled in the clinical trials. In addition, there is a debate among clinicians on the usefulness of electrophysiological study for risk stratification of asymptomatic patients with Brugada syndrome...
October 24, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29081906/brugada-type-1-electrocardiogram-should-we-treat-the-electrocardiogram-or-the-patient
#4
REVIEW
Pietro Delise, Giuseppe Allocca, Nadir Sitta
Patients with a Brugada type 1 electrocardiogram (ECG) pattern may suffer sudden cardiac death (SCD). Recognized risk factors are spontaneous type 1 ECG and syncope of presumed arrhythmic origin. Familial sudden cardiac death (f-SCD) is not a recognized independent risk factor. Finally, positive electrophysiologic study (+EPS) has a controversial prognostic value. Current ESC guidelines recommend implantable cardioverter defibrillator (ICD) implantation in patients with a Brugada type 1 ECG pattern if they have suffered a previous resuscitated cardiac arrest (class I recommendation) or if they have syncope of presumed cardiac origin (class IIa recommendation)...
September 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/29072257/therapeutic-effects-of-a-taurine-magnesium-coordination-compound-on-experimental-models-of-type-2-short-qt-syndrome
#5
Meng-Yao An, Kai Sun, Yan Li, Ying-Ying Pan, Yong-Qiang Yin, Yi Kang, Tao Sun, Hong Wu, Wei-Zhen Gao, Jian-Shi Lou
Short QT syndrome (SQTS) is a genetic arrhythmogenic disease that can cause malignant arrhythmia and sudden cardiac death. The current therapies for SQTS have application restrictions. We previously found that Mg· (NH2CH2CH2SO3)2· H2O, a taurine-magnesium coordination compound (TMCC) exerted anti-arrhythmic effects with low toxicity. In this study we established 3 different models to assess the potential anti-arrhythmic effects of TMCC on type 2 short QT syndrome (SQT2). In Langendorff guinea pig-perfused hearts, perfusion of pinacidil (20 μmol/L) significantly shortened the QT interval and QTpeak and increased rTp-Te (P<0...
October 26, 2017: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/29030919/baseline-fragmented-qrs-increases-the-risk-of-major-arrhythmic-events-in-brugada-syndrome-systematic-review-and-meta-analysis
#6
Pattara Rattanawong, Tanawan Riangwiwat, Narut Prasitlumkum, Nath Limpruttidham, Napatt Kanjanahattakij, Pakawat Chongsathidkiet, Wasawat Vutthikraivit, Eugene H Chung
BACKGROUND: Fragmented QRS reflects disturbances in the myocardium predisposing the heart to ventricular tachyarrhythmias. Recent studies suggest that fragmented QRS (fQRS) is associated with major arrhythmic events in Brugada syndrome. However, a systematic review and meta-analysis of the literature has not been done. We assessed the association between fQRS and major arrhythmic events in Brugada syndrome by a systematic review of the literature and a meta-analysis. METHODS: We comprehensively searched the databases of MEDLINE and EMBASE from inception to May 2017...
October 14, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/29024690/sodium-channel-current-loss-of-function-in-induced-pluripotent-stem-cell-derived-cardiomyocytes-from-a-brugada-syndrome-patient
#7
Elisabet Selga, Franziska Sendfeld, Rebecca Martinez-Moreno, Claire N Medine, Olga Tura-Ceide, Sir Ian Wilmut, Guillermo J Pérez, Fabiana S Scornik, Ramon Brugada, Nicholas L Mills
Brugada syndrome predisposes to sudden death due to disruption of normal cardiac ion channel function, yet our understanding of the underlying cellular mechanisms is incomplete. Commonly used heterologous expression models lack many characteristics of native cardiomyocytes and, in particular, the individual genetic background of a patient. Patient-specific induced pluripotent stem (iPS) cell-derived cardiomyocytes (iPS-CM) may uncover cellular phenotypical characteristics not observed in heterologous models...
October 9, 2017: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/28994463/prognosis-risk-stratification-and-management-of-asymptomatic-individuals-with-brugada-syndrome-a-systematic-review
#8
REVIEW
Konstantinos P Letsas, Dimitrios Asvestas, Adrian Baranchuk, Tong Liu, Stamatis Georgopoulos, Michael Efremidis, Panagiotis Korantzopoulos, George Bazoukis, Gary Tse, Antonios Sideris, Masahiko Takagi, Joachim R Ehrlich
Brugada syndrome (BrS) is a primary electrical disease associated with increased risk of sudden cardiac death due to polymorphic ventricular arrhythmias. The prognosis, risk stratification and management of asymptomatic individuals remain the most controversial issues in BrS. Furthermore, the decision to manage asymptomatic patients with an implantable cardioverter defibrillator should be made after weighing the potential individual risk of future arrhythmic events against the risk of complications associated with the implant and follow-up of patients living with such devices, and the accompanying impairment of the quality of life...
October 10, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28984287/the%C3%A2-lifevest-wearable-defibrillator-for-noninvasive-prevention-of-sudden-arrhythmic-death-syndrome-the%C3%A2-first-polish-case-of-an-adequate-lifevest-intervention
#9
Michał Chudzik, Łukasz Szyda, Bożena Urbanek, Jerzy K Wranicz, Jarosław D Kasprzak
No abstract text is available yet for this article.
September 29, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/28965168/cardiac-arrhythmias-related-to-sodium-channel-dysfunction
#10
Eleonora Savio-Galimberti, Mariana Argenziano, Charles Antzelevitch
The voltage-gated cardiac sodium channel (Nav1.5) is a mega-complex comprised of a pore-forming α subunit and 4 ancillary β-subunits together with numerous protein partners. Genetic defects in the form of rare variants in one or more sodium channel-related genes can cause a loss- or gain-of-function of sodium channel current (INa) leading to the manifestation of various disease phenotypes, including Brugada syndrome, long QT syndrome, progressive cardiac conduction disease, sick sinus syndrome, multifocal ectopic Purkinje-related premature contractions, and atrial fibrillation...
October 1, 2017: Handbook of Experimental Pharmacology
https://www.readbyqxmd.com/read/28955245/meta-analysis-of-fragmented-qrs-as-an-electrocardiographic-predictor-for-arrhythmic-events-in-patients-with-brugada-syndrome
#11
Lei Meng, Konstantinos P Letsas, Adrian Baranchuk, Qingmiao Shao, Gary Tse, Nixiao Zhang, Zhiwei Zhang, Dan Hu, Guangping Li, Tong Liu
Fragmented QRS (fQRS) is an electrocardiographic marker related to ventricular fibrillation (VF) and sudden cardiac death (SCD) in various clinical settings. Current data regarding the prognostic significance of fQRS in Brugada syndrome (BrS) are contradictory. This meta-analysis aimed to evaluate the presence of fQRS as a risk stratification tool in BrS. Electronic databases (PubMed, EMBASE, and Cochrane Library) were searched until May 2016. Eight observational studies accumulating data on 1,637 BrS patients (mean age: 47 ± 11 years) were included in this meta-analysis...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28948234/mechanisms-underlying-epicardial-radiofrequency-ablation-to-suppress-arrhythmogenesis-in-experimental-models-of-brugada-syndrome
#12
Bence Patocskai, Namsik Yoon, Charles Antzelevitch
OBJECTIVES: This study sought to test the hypothesis that elimination of sites of abnormal repolarization, via epicardial RFA, suppresses the electrocardiographic and arrhythmic manifestations of BrS. BACKGROUND: Brugada syndrome (BrS) is associated with ventricular tachycardia and ventricular fibrillation leading to sudden cardiac death. Nademanee et al. reported that radiofrequency ablation (RFA) of right ventricular outflow tract epicardium significantly reduced the electrocardiogram and arrhythmic manifestations of BrS...
April 2017: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/28873959/sudden-cardiac-death-among-the-young-in-sweden-from-2000-to-2010-an-autopsy-based-study
#13
Aase Wisten, Peter Krantz, Eva-Lena Stattin
Aims: To study the incidence and aetiology of sudden cardiac death (SCD) in 1- to 35-year-olds in Sweden from 2000 to 2010. Methods and results: We used the database of the Swedish National Board of Forensic Medicine and the Swedish Cause of Death Registry and identified SCD cases by review of forensic files and death certificates. We identified 552 individuals with SCD in 1- to 35-year-olds; 156 (28%) were women. In 393 (71%), a forensic autopsy had been performed; in 131 (24%), a clinical autopsy had been performed; in 28 (5%) with no autopsy, a cardiac disease was diagnosed before death...
August 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28814790/in-silico-investigation-of-a-kcnq1-mutation-associated-with-short-qt-syndrome
#14
Ismail Adeniran, Dominic G Whittaker, Aziza El Harchi, Jules C Hancox, Henggui Zhang
Short QT syndrome (SQTS) is a rare condition characterized by abnormally 'short' QT intervals on the ECG and increased susceptibility to cardiac arrhythmias and sudden death. This simulation study investigated arrhythmia dynamics in multi-scale human ventricle models associated with the SQT2-related V307L KCNQ1 'gain-of-function' mutation, which increases slow-delayed rectifier potassium current (IKs). A Markov chain (MC) model recapitulating wild type (WT) and V307L mutant IKs kinetics was incorporated into a model of the human ventricular action potential (AP) for investigation of QT interval changes and arrhythmia substrates...
August 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28728742/a-clinical-score-model-to-predict-lethal-events-in-young-patients-%C3%A2-19-years-with-the-brugada-syndrome
#15
M Cecilia Gonzalez Corcia, Juan Sieira, Gudrun Pappaert, Carlo de Asmundis, Gian Battista Chierchia, Andrea Sarkozy, Pedro Brugada
Risk stratification in Brugada syndrome in young patients remains challenging. We investigated the clinical characteristics, prognosis, and risk in young patients with the Brugada syndrome. We studied 95 patients with the Brugada syndrome aged ≤19 years. The median age at diagnosis was 12.9 years. The clinical presentation was sudden cardiac death in 7% and syncope in 21%. The remaining 72% were asymptomatic at diagnosis. Electrical abnormalities were present in 36%, including spontaneous type I electrocardiogram (12%), sinus node dysfunction (9%), atrioventricular block (17%), intraventricular conduction delay (16%), and atrial arrhythmias (8%)...
September 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28685702/a-focus-on-pharmacological-management-of-catecholaminergic-polymorphic-ventricular-tachycardia
#16
Claudio Barbanti, Alice Maltret, Daniel Sidi
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a channelopathy characterized by adrenergic mediated ventricular arrhythmia. Untreated CPVT is a malignant syndrome with more than 50% of arrhythmic event and up to 25% of fatal or near-fatal cardiac event at 8 years follow-up. Prevention of sudden cardiac death starts with exclusion of competitive sports. Beta blockers (BB) are the cornerstone pharmacological therapy for prevention of cardiac event in CPVT patients. Dose of BB should be the highest tolerable, preferably nadolol...
July 7, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28668989/sudden-unexplained-death-in-alcohol-misuse-sudam-patients-have-different-characteristics-to-those-who-died-from-sudden-arrhythmic-death-syndrome-sads
#17
Tracy Sorkin, Mary N Sheppard
There is growing awareness of sudden unexplained death in alcohol misuse (SUDAM) in which there is no obvious cause of death, no evidence of acute alcohol toxicity or alcoholic ketoacidosis, and the heart is morphologically normal. This study describes the characteristics of a cohort with SUDAM from a tertiary cardiovascular referral center and compares the findings with those of individuals who died from sudden arrhythmic death syndrome (SADS). Cases in this retrospective cross-sectional study were identified from a database of referrals to our center spanning approximately 40 years...
July 1, 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/28645806/brugada-syndrome-a-general-cardiologist-s-perspective
#18
REVIEW
Marija M Polovina, Milica Vukicevic, Bojan Banko, Gregory Y H Lip, Tatjana S Potpara
Brugada syndrome (BrS) is one of the commonest inherited primary arrhythmia syndromes typically presenting with arrhythmic syncope or sudden cardiac death (SCD) due to polymorphic ventricular tachycardia and ventricular fibrillation precipitated by vagotonia or fever in apparently healthy adults, less frequently in children. The prevalence of the syndrome (0.01%-0.3%) varies among regions and ethnicities, being the highest in Southeast Asia. BrS is diagnosed by the "coved type" ST-segment elevation≥2mm followed by a negative T-wave in ≥1 of the right precordial leads V1-V2...
October 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/28606196/prescribing-an-automated-external-defibrillator-for-children-at-increased-risk-of-sudden-arrhythmic-death
#19
Karen A McLeod, Eileen Fern, Fiona Clements, Ruth McGowan
BACKGROUND: Automated external defibrillators can be life-saving in out-of-hospital cardiac arrest. OBJECTIVE: Our aim was to review our experience of prescribing automated external defibrillators for children at increased risk of sudden arrhythmic death. METHODS: We reviewed all automated external defibrillators issued by the Scottish Paediatric Cardiac Electrophysiology Service from 2005 to 2015. All parents were given resuscitation training according to the Paediatric Resuscitation Guidelines, including the use of the automated external defibrillator...
September 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28592292/effects-of-amiodarone-on-short-qt-syndrome-variant-3-in-human-ventricles-a-simulation-study
#20
Cunjin Luo, Kuanquan Wang, Henggui Zhang
BACKGROUND: Short QT syndrome (SQTS) is a newly identified clinical disorder associated with atrial and/or ventricular arrhythmias and increased risk of sudden cardiac death (SCD). The SQTS variant 3 is linked to D172N mutation to the KCNJ2 gene that causes a gain-of-function to the inward rectifier potassium channel current (I K1), which shortens the ventricular action potential duration (APD) and effective refractory period (ERP). Pro-arrhythmogenic effects of SQTS have been characterized, but less is known about the possible pharmacological treatment of SQTS...
June 7, 2017: Biomedical Engineering Online
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