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Sudden arrhythmic death syndrome

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https://www.readbyqxmd.com/read/29325976/profile-of-brugada-syndrome-patients-presenting-with-their-first-documented-arrhythmic-event-data-from-the-survey-on-arrhythmic-events-in-brugada-syndrome-sabrus
#1
Anat Milman, Antoine Andorin, Jean-Baptiste Gourraud, Pieter G Postema, Frederic Sacher, Philippe Mabo, Sung-Hwan Kim, Jimmy Jm Juang, Shingo Maeda, Yoshihide Takahashi, Tsukasa Kamakura, Takeshi Aiba, Giulio Conte, Georgia Sarquella-Brugada, Eran Leshem, Michael Rahkovich, Aviram Hochstadt, Yuka Mizusawa, Elena Arbelo, Zhengrong Huang, Isabelle Denjoy, Carla Giustetto, Yanushi D Wijeyeratne, Carlo Napolitano, Yoav Michowitz, Ramon Brugada, Ruben Casado-Arroyo, Jean Champagne, Leonardo Calo, Jacob Tfelt-Hansen, Silvia G Priori, Masahiko Takagi, Christian Veltmann, Pietro Delise, Domenico Corrado, Elijah R Behr, Fiorenzo Gaita, Gan-Xin Yan, Josep Brugada, Antoine Leenhardt, Arthur A M Wilde, Pedro Brugada, Kengo F Kusano, Kenzo Hirao, Gi-Byoung Nam, Vincent Probst, Bernard Belhassen
BACKGROUND: Detailed information on the profile of Brugada syndrome (BrS) patients presenting their first arrhythmic event (AE) after prophylactic implantation of a cardioverter defibrillator (ICD) is limited. OBJECTIVES: 1) To compare clinical, electrocardiographic, electrophysiologic and genetic profiles of patients who exhibited their first documented AE as aborted cardiac arrest (CA) (group A) with those in whom the AE was documented after prophylactic ICD implantation (group B); 2) To characterize group B patients' profile using the Class II indications for ICD implantation established by HRS/EHRA/APHRS Expert Consensus Statement in 2013...
January 8, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29319343/obesity-and-sudden-cardiac-death-in-the-young-clinical-and-pathological-insights-from-a-large-national-registry
#2
Gherardo Finocchiaro, Michael Papadakis, Harshil Dhutia, Della Cole, Elijah R Behr, Maite Tome, Sanjay Sharma, Mary N Sheppard
Aims Obesity is an increasing public health problem and a risk factor for cardiovascular diseases. The aim of the study was to determine the main features and aetiologies in a large cohort of sudden cardiac deaths that occurred in obese subjects. Methods Between 1994 and 2014, 3684 consecutive cases of unexpected sudden cardiac death were referred to our cardiac pathology centre. This study was confined to young individuals (age ≤ 35 years) for whom information about body mass index was available and consisted of 1033 cases...
January 1, 2018: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/29290967/modelling-the-effects-of-chloroquine-on-kcnj2-linked-short-qt-syndrome
#3
Cunjin Luo, Kuanquan Wang, Henggui Zhang
A gain-of-function KCNJ2 D172N mutation in KCNJ2-encoded Kir2.1 channels underlies one form of short QT syndrome (SQT3), which is associated with increased susceptibility to arrhythmias and sudden death. Anti-malarial drug chloroquine was reported as an effective inhibitor of Kir2.1 channels. Using biophysically-detailed human ventricle computer models, this study assessed the effects of chloroquine on SQT3. The ten Tusscher et al. model of human ventricular cell action potential was modified to recapitulate functional changes in the inward rectifier K+ current (IK1) due to heterozygous and homozygous forms of the D172N mutation...
December 5, 2017: Oncotarget
https://www.readbyqxmd.com/read/29258620/refractory-ventricular-fibrillations-after-surgical-repair-of-atrial-septal-defects-in-a-patient-with-cacna1c-gene-mutation-case-report
#4
Ai Kojima, Fumiaki Shikata, Toru Okamura, Takashi Higaki, Seiko Ohno, Minoru Horie, Shunji Uchita, Yujiro Kawanishi, Kenji Namiguchi, Takumi Yasugi, Hironori Izutani
BACKGROUND: Congenital long QT syndrome (LQTS) can cause ventricular arrhythmic events with syncope and sudden death resulting from malignant torsades de pointes (TdP) followed by ventricular fibrillations (VFs). However, the syndrome is often overlooked prior to the development of arrhythmic events in patients with congenital heart diseases demonstrating right bundle branch block on electrocardiogram (ECG). We present a case of an adult patient with congenital heart disease who developed VFs postoperatively, potentially due to his mutation in a LQTS related gene, which was not identified on preoperative assessment due to incomplete evaluation of his family history...
December 19, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29241489/hydroquinidine-prevents-life-threatening-arrhythmic-events-in-patients-with-short%C3%A2-qt%C3%A2-syndrome
#5
Andrea Mazzanti, Riccardo Maragna, Gaetano Vacanti, Anna Kostopoulou, Maira Marino, Nicola Monteforte, Raffaella Bloise, Katherine Underwood, Valentina Tibollo, Eleonora Pagan, Carlo Napolitano, Riccardo Bellazzi, Vincenzo Bagnardi, Silvia G Priori
BACKGROUND: Short QT syndrome (SQTS) is a rare and life-threatening arrhythmogenic syndrome characterized by abbreviated repolarization. Hydroquinidine (HQ) prolongs the QT interval in SQTS patients, although whether it reduces cardiac events is currently unknown. OBJECTIVES: This study investigated whether long-term treatment with HQ reduces the occurrence of life-threatening arrhythmic events (LAE) (cardiac arrest or sudden cardiac death) in SQTS patients. METHODS: In this cohort study on consecutive SQTS patients, 2 analyses were performed: 1) a matched-period analysis for the occurrence of LAE in 17 SQTS patients who received long-term HQ; and 2) a comparison of the annual incidence of LAE off- and on-HQ in 16 SQTS patients who survived a cardiac arrest...
December 19, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29186479/febrile-seizures-prior-to-sudden-cardiac-death-a-danish-nationwide-study
#6
Niels Kjær Stampe, Charlotte Glinge, Reza Jabbari, Thea Bjune, Bjarke Risgaard, Jacob Tfelt-Hansen, Bo Gregers Winkel
Aims: Febrile seizure (FS) is a common disorder affecting 2-5% of children up to 5 years of age. The aim of this study was to determine whether FS in early childhood are over-represented in young adults dying from sudden cardiac death (SCD). Methods and results: We included all deaths (n = 4595) nationwide and through review of all death certificates, we identified 245 SCD in Danes aged 1-30 years in 2000-09. Through the usage of nationwide registries, we identified all persons admitted with first FS among SCD cases (14/245; 5...
November 23, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29176389/contemporary-genetic-testing-in-inherited-cardiac-disease-tools-ethical-issues-and-clinical-applications
#7
Francesca Girolami, Giulia Frisso, Matteo Benelli, Lia Crotti, Maria Iascone, Ruggiero Mango, Cristina Mazzaccara, Kalliope Pilichou, Eloisa Arbustini, Benedetta Tomberli, Giuseppe Limongelli, Cristina Basso, Iacopo Olivotto
: Inherited cardiac diseases comprise a wide and heterogeneous spectrum of diseases of the heart, including the cardiomyopathies and the arrhythmic diseases in structurally normal hearts, that is, channelopathies. With a combined estimated prevalence of 3% in the general population, these conditions represent a relevant epidemiological entity worldwide, and are a major cause of cardiac morbidity and mortality in the young. The extraordinary progress achieved in molecular genetics over the last three decades has unveiled the complex molecular basis of many familial cardiac conditions, paving the way for routine use of gene testing in clinical practice...
January 2018: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/29174490/risk-of-cardiac-events-associated-with-antidepressant-therapy-in-patients-with-long-qt-syndrome
#8
Meng Wang, Barbara Szepietowska, Bronislava Polonsky, Scott McNitt, Arthur J Moss, Wojciech Zareba, David S Auerbach
Patients with long QT syndrome (LQTS) are at a high risk of cardiac events. Many patients with LQTS are treated with antidepressant drugs (ADs). We investigated the LQTS genotype-specific risk of recurrent cardiac arrhythmic events (CAEs) associated with AD therapy. The study included 59 LQT1 and 72 LQT2 patients from the Rochester-based LQTS Registry with corrected QT (QTc) prolongation and a history of AD therapy. Using multivariate Anderson-Gill models, we estimated the LQTS genotype-specific risk of recurrent CAEs (ventricular tachyarrhythmias, aborted cardiac arrest, or sudden cardiac death) associated with time-dependent ADs...
November 13, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29145303/epicardial-radiofrequency-catheter-ablation-of-brugada-syndrome-with-electrical-storm-during-ventricular-fibrillation-a-case-report
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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-%C3%A2-risk-stratification-and-management-of-asymptomatic-individuals-with-brugada-syndrome-a-systematic-review
#16
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...
December 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
#17
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
#18
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
#19
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
#20
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
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