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Sudden arrhythmic death syndrome

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https://www.readbyqxmd.com/read/28496938/brugada-syndrome-risk-stratification-and-management
#1
REVIEW
Yoshifusa Aizawa
The Brugada syndrome (BrS) is an arrhythmogenic disease associated with an increased risk of ventricular fibrillation and sudden cardiac death. The risk stratification and management of BrS patients, particularly of asymptomatic ones, still remains challenging. A previous history of aborted sudden cardiac death or arrhythmic syncope in the presence of spontaneous type 1 ECG pattern of BrS phenotype appear to be the most reliable predictors of future arrhythmic events. Several other ECG parameters have been proposed for risk stratification...
October 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/28494446/mutation-load-of-multiple-ion-channel-gene-mutations-in-brugada-syndrome
#2
Francesca Gualandi, Fatima Zaraket, Michele Malagù, Giulia Parmeggiani, Cecilia Trabanelli, Sergio Fini, Xiao Dang, Xiaoming Wei, Mingyan Fang, Matteo Bertini, Roberto Ferrari, Alessandra Ferlini
Brugada syndrome is a primary arrhythmic syndrome that accounts for 20% of all sudden cardiac death cases in individuals with a structurally normal heart. Pathogenic variants associated with Brugada syndrome have been identified in over 19 genes, with SCN5A as a pivotal gene accounting for nearly 30% of cases. In contrast to other arrhythmogenic channelopathies (such as long QT syndrome), digenic inheritance has never been reported in Brugada syndrome. Exploring 66 cardiac genes using a new custom next-generation sequencing panel, we identified a double heterozygosity for pathogenic mutations in SCN5A and TRPM4 in a Brugada syndrome patient...
May 12, 2017: Cardiology
https://www.readbyqxmd.com/read/28486839/brugada-syndrome-and-the-story-of-dave
#3
Samira Kashinath Dhamapurkar, Barbara A Wilson, Anita Rose, Gerhard Florschutz
Brugada syndrome (BrS) is a little known genetic condition that causes severe disturbances in cardiac rhythm and may result in sudden unexpected cardiac death in an apparently healthy person. The heart structure is typically normal but there are problems with electrical activity. The syndrome is named after Spanish brothers who are cardiologists, Pedro and Josep Brugada. BrS is the major cause of sudden unexplained death syndrome (SUDS), also known as sudden arrhythmic death syndrome (SADS). Following a description of the syndrome, including its prevalence and incidence, how it is diagnosed and how it can be treated, we consider those who survive a cardiac arrest and what problems they may face...
May 10, 2017: Neuropsychological Rehabilitation
https://www.readbyqxmd.com/read/28484840/epidemiology-of-ventricular-tachyarrhythmia-any-changes-in-the-past-decades
#4
REVIEW
Benjamin Jong-Ming Pang, Martin Stephen Green
Ventricular tachyarrhythmias include potentially lethal episodes of sustained ventricular tachycardia (VT) and ventricular fibrillation (VF) as well as hemodynamically tolerated ventricular ectopic activity. Sustained VT or VF may develop in the setting of acute myocardial infarction or as clinical sequelae of advanced cardiomyopathy. The incidence of these serious arrhythmias is estimated from retrospective and observational studies and registries of sudden cardiac arrest and sudden cardiac death. Over the past few decades, there has been a gradual decline in the incidence of life-threatening ventricular tachyarrhythmias which has been largely driven by upstream treatments for and prevention of coronary artery disease and its sequelae...
May 8, 2017: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/28479512/long-term-prognosis-of-drug-induced-brugada-syndrome
#5
Juan Sieira, Giuseppe Ciconte, Giulio Conte, Carlo de Asmundis, Gian-Battista Chierchia, Giannis Baltogiannis, Giacomo Di Giovanni, Yukio Saitoh, Ruben Casado-Arroyo, Justo Juliá, Mark La Meir, Francis Wellens, Kristel Wauters, Gudrun Pappaert, Pedro Brugada
BACKROUND: Patients with a drug-induced Brugada syndrome (BS) are considered at a lower risk than those with the spontaneous type I pattern. Nevertheless, they can present arrhythmic events. OBJECTIVE: The purpose of this study was to investigate their clinical characteristics, long-term prognosis and risk factors. METHODS: A consecutive cohort of 343 drug-induced BS patients was included and compared to 78 with spontaneous type I pattern...
May 4, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28469493/beyond-the-electrocardiogram-mutations-in-cardiac-ion-channel-genes-underlie-nonarrhythmic-phenotypes
#6
REVIEW
Thomas M Roston, Taylor Cunningham, Anna Lehman, Zachary W Laksman, Andrew D Krahn, Shubhayan Sanatani
Cardiac ion channelopathies are an important cause of sudden death in the young and include long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, idiopathic ventricular fibrillation, and short QT syndrome. Genes that encode ion channels have been implicated in all of these conditions, leading to the widespread implementation of genetic testing for suspected channelopathies. Over the past half-century, researchers have also identified systemic pathologies that extend beyond the arrhythmic phenotype in patients with ion channel gene mutations, including deafness, epilepsy, cardiomyopathy, periodic paralysis, and congenital heart disease...
2017: Clinical Medicine Insights. Cardiology
https://www.readbyqxmd.com/read/28449774/utility-of-post-mortem-genetic-testing-in-cases-of-sudden-arrhythmic-death-syndrome
#7
Najim Lahrouchi, Hariharan Raju, Elisabeth M Lodder, Efstathios Papatheodorou, James S Ware, Michael Papadakis, Rafik Tadros, Della Cole, Jonathan R Skinner, Jackie Crawford, Donald R Love, Chee J Pua, Bee Y Soh, Jaydutt D Bhalshankar, Risha Govind, Jacob Tfelt-Hansen, Bo G Winkel, Christian van der Werf, Yanushi D Wijeyeratne, Greg Mellor, Jan Till, Marta C Cohen, Maria Tome-Esteban, Sanjay Sharma, Arthur A M Wilde, Stuart A Cook, Connie R Bezzina, Mary N Sheppard, Elijah R Behr
BACKGROUND: Sudden arrhythmic death syndrome (SADS) describes a sudden death with negative autopsy and toxicological analysis. Cardiac genetic disease is a likely etiology. OBJECTIVES: This study investigated the clinical utility and combined yield of post-mortem genetic testing (molecular autopsy) in cases of SADS and comprehensive clinical evaluation of surviving relatives. METHODS: We evaluated 302 expertly validated SADS cases with suitable DNA (median age: 24 years; 65% males) who underwent next-generation sequencing using an extended panel of 77 primary electrical disorder and cardiomyopathy genes...
May 2, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28392310/the-role-of-the-autonomic-nervous-system-in-arrhythmias-and-sudden-cardiac-death
#8
REVIEW
Sonia Franciosi, Frances K G Perry, Thomas M Roston, Kathryn R Armstrong, Victoria E Claydon, Shubhayan Sanatani
The autonomic nervous system (ANS) is complex and plays an important role in cardiac arrhythmia pathogenesis. A deeper understanding of the anatomy and development of the ANS has shed light on its involvement in cardiac arrhythmias. Alterations in levels of Sema-3a and NGF, both growth factors involved in innervation patterning during development of the ANS, leads to cardiac arrhythmias. Dysregulation of the ANS, including polymorphisms in genes involved in ANS development, have been implicated in sudden infant death syndrome...
March 31, 2017: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/28382321/electrophysiological-mechanisms-of-long-and-short-qt-syndromes
#9
REVIEW
Gary Tse, Yin Wah Fiona Chan, Wendy Keung, Bryan P Yan
The QT interval on the human electrocardiogram is normally in the order of 450 ms, and reflects the summated durations of action potential (AP) depolarization and repolarization of ventricular myocytes. Both prolongation and shortening in the QT interval have been associated with ventricular tachy-arrhythmias, which predispose affected individuals to sudden cardiac death. In this article, the molecular determinants of the AP duration and the causes of long and short QT syndromes (LQTS and SQTS) are explored...
March 2017: IJC Heart & Vasculature
https://www.readbyqxmd.com/read/28379344/a-score-model-to-predict-risk-of-events-in-patients-with-brugada-syndrome
#10
Juan Sieira, Giulio Conte, Giuseppe Ciconte, Gian-Battista Chierchia, Ruben Casado-Arroyo, Giannis Baltogiannis, Giacomo Di Giovanni, Yukio Saitoh, Justo Juliá, Giacomo Mugnai, Mark La Meir, Francis Wellens, Jens Czapla, Gudrun Pappaert, Carlo de Asmundis, Pedro Brugada
Aims: Risk stratification in Brugada Syndrome (BS) remains challenging. Arrhythmic events can occur life-long and studies with long follow-ups are sparse. The aim of our study was to investigate long-term prognosis and risk stratification of BS patients. Methods and results: A single centre consecutive cohort of 400 BS patients was included and analysed. Mean age was 41.1 years, 78 patients (19.5%) had a spontaneous type I electrocardiogram (ECG). Clinical presentation was aborted sudden cardiac death (SCD) in 20 patients (5...
March 31, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28371698/cardiovascular-causes-of-maternal-sudden-death-sudden-arrhythmic-death-syndrome-is-leading-cause-in-uk
#11
Dimitra Krexi, Mary N Sheppard
OBJECTIVE: This study aims to determine the causes of sudden cardiac death during pregnancy and in the postpartum period and patients' characteristics. There are few studies in the literature. METHODS: Eighty cases of sudden unexpected death due to cardiac causes in relation to pregnancy and postpartum period in a database of 4678 patients were found and examined macroscopically and microscopically. RESULTS: The mean age was 30±7 years with a range from 16 to 43 years...
May 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
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/28339816/post-mortem-toxicology-in-young-sudden-cardiac-death-victims-a-nationwide-cohort-study
#13
Thea Bjune, Bjarke Risgaard, Line Kruckow, Charlotte Glinge, Ole Ingemann-Hansen, Peter Mygind Leth, Kristian Linnet, Jytte Banner, Bo Gregers Winkel, Jacob Tfelt-Hansen
Aims: Several drugs increase the risk of ventricular fibrillation and sudden cardiac death (SCD). We aimed to investigate in detail the toxicological findings of all young SCD throughout Denmark. Methods and results: Deaths in persons aged 1-49 years were included over a 10-year period. Death certificates and autopsy reports were retrieved and read to identify cases of sudden death and establish cause of death. All medico-legal autopsied SCD were included and toxicological reports collected...
February 27, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28316113/the-utility-of-exercise-testing-in-risk-stratification-of-asymptomatic-patients-with-type-1-brugada-pattern
#14
Muthiah Subramanian, Mukund A Prabhu, Madhavankutty Santhakumari Harikrishnan, Saritha S Shekhar, Praveen G Pai, Kumaraswamy Natarajan
INTRODUCTION: Risk stratification of asymptomatic patients with a Brugada type 1 ECG pattern remains an unresolved clinical conundrum. In contrast to provocative pharmacological testing in Brugada syndrome, there is limited data on the role of exercise stress testing as a risk stratification modality. The objective of this study was to evaluate the utility of exercise testing in asymptomatic patients with type 1 Brugada pattern to prognosticate major arrhythmic events (MAE) during follow-up...
March 18, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28315637/trpm4-non-selective-cation-channel-variants-in-long-qt-syndrome
#15
Thomas Hof, Hui Liu, Laurent Sallé, Jean-Jacques Schott, Corinne Ducreux, Gilles Millat, Philippe Chevalier, Vincent Probst, Romain Guinamard, Patrice Bouvagnet
BACKGROUND: Long QT syndrome (LQTS) is an inherited arrhythmic disorder characterized by prolongation of the QT interval, a risk of syncope, and sudden death. There are already a number of causal genes in LQTS, but not all LQTS patients have an identified mutation, which suggests LQTS unknown genes. METHODS: A cohort of 178 LQTS patients, with no mutations in the 3 major LQTS genes (KCNQ1, KCNH2, and SCN5A), was screened for mutations in the transient potential melastatin 4 gene (TRPM4)...
March 18, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28276182/application-of-the-national-early-warning-score-news-as-a-stratification-tool-on-admission-in-an-italian-acute-medical-ward-a-perspective-study
#16
Walter Spagnolli, Marta Rigoni, Emanuele Torri, Susanna Cozzio, Elisa Vettorato, Giandomenico Nollo
AIM: We aimed to assess the performance of the National Early Warning Score (NEWS) as tool for patient risk stratification at admission in an acute Internal Medicine ward and to ensure patient placement in ward areas with the required and most appropriate intensity of care. As secondary objective, we considered NEWS performance in two subgroups of patients: sudden cardiac events (acute coronary syndromes and arrhythmic events), and chronic respiratory insufficiency. METHODS: We conducted a perspective cohort single centre study on 2,677 unselected patients consecutively admitted from July 2013 to March 2015 in the Internal Medicine ward of the hospital of Trento, Italy...
March 2017: International Journal of Clinical Practice
https://www.readbyqxmd.com/read/28258845/noninvasive-ecg-imaging-ecgi-mapping-the-arrhythmic-substrate-of-the-human-heart
#17
Yoram Rudy
This short communication accompanies my presentation at the International Congress on Sudden Cardiac Death held in Prague, March 30-April 1, 2017. It summarizes briefly studies of the cardiac electrophysiological substrate in patients with hereditary arrhythmogenic syndromes - the Long QT and Brugada syndromes - conducted noninvasively, in situ, using Electrocardiographic Imaging (ECGI). The same noninvasive approach was used to map the electrophysiological substrate of a post-infarction myocardial scar and to relate this substrate to the pattern of activation during reentrant ventricular tachycardia...
June 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27996317/cardiac-arrest-with-clozapine-and-olanzapine-revealing-long-qt-syndrome
#18
Edward Woloszyn, Nishant Whig, Eileen Trigoboff, Jeffery J Grace
The authors describe a rare case of "concealed" congenital Long QT Syndrome (LQTS) Type 3 in a patient with treatment resistant schizophrenia and no known personal or family history of cardiac disease. The patient in this Case Report had a hidden genetic condition revealed only following the essential administration of antipsychotics. As a result, this patient experienced an aborted cardiac arrest and a total of five episodes of ventricular tachycardia (VT) requiring cardioversion. Successful control of the VT occurred with an Automatic Internal Defibrillator (AID), judicious use of antipsychotic medications, and anti-arrhythmic medications...
December 20, 2016: Clinical Schizophrenia & related Psychoses
https://www.readbyqxmd.com/read/27992820/gja1-gene-variations-in-sudden-unexplained-nocturnal-death-syndrome-in-the-chinese-han-population
#19
Qiuping Wu, Yeda Wu, Liyong Zhang, Jinxiang Zheng, Shuangbo Tang, Jianding Cheng
Sudden unexplained nocturnal death syndrome (SUNDS) is a conundrum to both forensic pathologists and physicians, more than 80% of which the molecular pathogenesis remains unclear. Reported studies on both clinical and genetic phenotypes suggest SUNDS is related to congenital and acquired arrhythmias. Recent researches have linked the mutations of gene gap junction alpha 1 (GJA1) with arrhythmogenic cardiac disorders. In the present study, we investigate the potential correlation between GJA1 gene variations and the occurrence of SUNDS...
December 9, 2016: Forensic Science International
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
#20
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
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