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Sudden arrhythmic death syndrome

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https://www.readbyqxmd.com/read/28339816/post-mortem-toxicology-in-young-sudden-cardiac-death-victims-a-nationwide-cohort-study
#1
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
#2
Muthiah Subramanian, Mukund A Prabhu, M S Harikrishnan, Saritha S Shekhar, Praveen G Pai, K U 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
#3
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
#4
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 9, 2017: International Journal of Clinical Practice
https://www.readbyqxmd.com/read/28258845/noninvasive-ecg-imaging-ecgi-mapping-the-arrhythmic-substrate-of-the-human-heart
#5
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...
February 27, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27996317/cardiac-arrest-with-clozapine-and-olanzapine-revealing-long-qt-syndrome
#6
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
#7
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
#8
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/27925329/fragmented-qrs-is-a-novel-risk-factor-for-ventricular-arrhythmic-events-after-receiving-cardiac-resynchronization-therapy-in-nonischemic-cardiomyopathy
#9
Miyako Igarashi, Hiroshi Tada, Hiro Yamasaki, Kenji Kuroki, Tomoko Ishizu, Yoshihiro Seo, Takeshi Machino, Nobuyuki Murakoshi, Yukio Sekiguchi, Yuichi Noguchi, Akihiko Nogami, Kazutaka Aonuma
INTRODUCTION: A fragmented QRS (fQRS) is reported to be associated with a poor prognosis or sudden cardiac death (SCD) in patients with Brugada syndrome or ischemic heart disease. However, no studies have clarified the impact of the presence of an fQRS on SCD or ventricular arrhythmic events in patients receiving cardiac resynchronization therapy (CRT). This study aimed to clarify this point in patients with nonischemic cardiomyopathy. METHODS AND RESULTS: This study included 137 heart failure patients with nonischemic cardiomyopathy who received CRT (NYHA functional class: II/III/IV = 25/84/28)...
December 7, 2016: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/27909531/brugada-syndrome-risk-stratification-and-management
#10
REVIEW
Konstantinos P Letsas Md Fesc, Stamatis Georgopoulos Md, Konstantinos Vlachos Md, Nikolaos Karamichalakis Md, Ioannis Liatakis Md, Panagiotis Korantzopoulos Md PhD, Tong Liu Md PhD, Michael Efremidis Md, Antonios Sideris Md
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...
August 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27803673/electrophysiological-mechanisms-of-brugada-syndrome-insights-from-pre-clinical-and-clinical-studies
#11
REVIEW
Gary Tse, Tong Liu, Ka H C Li, Victoria Laxton, Yin W F Chan, Wendy Keung, Ronald A Li, Bryan P Yan
Brugada syndrome (BrS), is a primary electrical disorder predisposing affected individuals to sudden cardiac death via the development of ventricular tachycardia and fibrillation (VT/VF). Originally, BrS was linked to mutations in the SCN5A, which encodes for the cardiac Na(+) channel. To date, variants in 19 genes have been implicated in this condition, with 11, 5, 3, and 1 genes affecting the Na(+), K(+), Ca(2+), and funny currents, respectively. Diagnosis of BrS is based on ECG criteria of coved- or saddle-shaped ST segment elevation and/or T-wave inversion with or without drug challenge...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27761159/molecular-autopsy-in-victims-of-inherited-arrhythmias
#12
REVIEW
Christopher Semsarian, Jodie Ingles
Sudden cardiac death (SCD) is a rare but devastating complication of a number of underlying cardiovascular diseases. While coronary artery disease and acute myocardial infarction are the most common causes of SCD in older populations, inherited cardiac disorders comprise a substantial proportion of SCD cases aged less than 40 years. Inherited cardiac disorders include primary inherited arrhythmogenic disorders such as familial long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and inherited cardiomyopathies, most commonly hypertrophic cardiomyopathy (HCM)...
October 2016: Journal of Arrhythmia
https://www.readbyqxmd.com/read/27759343/early-repolarisation-changes-in-ecg-are-they-benign-or-malignant
#13
Oruganti Sai Satish, K Sashikanth Srivastav
Till recently, ST segment elevation in the absence of conduction abnormalities or chest pain occurring particularly in young bradycardia individuals has been considered a normal variant called early repolarisation (ER). However, recent studies suggest a more worrisome picture as patients with history of idiopathic ventricular fibrillation showed increased prevalence of ER in ECG. ER is an ECG pattern characterised by elevation of the QRS-ST junction (J point) ≥ 2 mv from baseline in the inferior (II, III, aVF) or lateral (I, aVL, V4-V6) leads manifested as QRS slurring or notching...
July 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27738063/brugada-syndrome-in-the-young-an-assessment-of-risk-factors-predicting-future-events
#14
Maria Cecilia Gonzalez Corcia, Juan Sieira, Andrea Sarkozy, Carlo de Asmundis, Gian-Battista Chierchia, Jaime Hernandez Ojeda, Gudrun Pappaert, Pedro Brugada
AIMS: To investigate the clinical characteristics, prognoses, and presence of risk factors in young patients with Brugada syndrome (BS). METHODS AND RESULTS: A consecutive cohort of 128 young BS patients (≤25 years old at diagnosis) was analysed. Eighty-eight patients (69%) were asymptomatic, whereas 40 (31%) presented with clinical manifestations of BS. Markers of prognosis and risk were identified upon comparison of these two groups. A history of malignant syncope was strong predictors of ventricular arrhythmic events...
October 13, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/27692676/sudden-death-and-cardiac-arrest-without-phenotype-the-utility-of-genetic-testing
#15
REVIEW
Yanushi D Wijeyeratne, Elijah R Behr
Approximately 4% of sudden cardiac deaths are unexplained [the sudden arrhythmic death syndrome (SADS)], and up to 6-10% of survivors of cardiac arrest do not have an identifiable cardiac abnormality after comprehensive clinical evaluation [idiopathic ventricular fibrillation (IVF)]. Genetic testing may be able to play a role in diagnostics and can be targeted to an underlying phenotype present in family members following clinical evaluation. Alternatively, post-mortem genetic testing (the "molecular autopsy") may diagnose the underlying cause if a clearly pathogenic rare variant is found...
August 31, 2016: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27641793/incidence-and-predictors-of-sudden-arrhythmic-death-or-ventricular-tachyarrhythmias-after-acute-coronary-syndrome-an-asian-perspective
#16
Jo-Jo Hai, Edward Tam, Pak-Hei Chan, Chu-Pak Lau, Chung-Wah Siu, Hung-Fat Tse
BACKGROUND: Current data on the risk of sudden arrhythmic death (SAD) and ventricular tachyarrhythmias (VTs) after acute coronary syndrome (ACS) in the Asian population are limited. OBJECTIVE: The purpose of this study was to investigate the incidence and predictors of SAD or VT after ACS in a contemporary cohort of Chinese patients in the era of early revascularization. METHODS: Consecutive patients admitted to our unit for ACS from 2010 to 2015 were retrospectively reviewed...
January 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27498076/inhibition-of-late-sodium-current-attenuates-ionic-arrhythmia-mechanism-in-ventricular-myocytes-expressing-lamina-n195k-mutation
#17
Yogananda S Markandeya, Tadashi Tsubouchi, Timothy A Hacker, Matthew R Wolff, Luiz Belardinelli, Ravi C Balijepalli
BACKGROUND: Lamin A and C are nuclear filament proteins encoded by the LMNA gene. Mutations in the LMNA gene cause many congenital diseases known as laminopathies, including Emery-Dreifuss muscular dystrophy, Hutchinson-Gilford progeria syndrome, and familial dilated cardiomyopathy (DCM) with conduction disease. A missense mutation (N195K) in the A-type lamins results in familial DCM and sudden arrhythmic death. OBJECTIVE: The purpose of this study was to investigate the ion current mechanism of arrhythmia and DCM caused by the LaminA-N195K variant...
November 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27437140/monogenec-arrhythmic-syndromes-from-molecular-and-genetic-aspects-to-bedside
#18
Golukhova E Z, Gromova O I, Shomahov R A, Bulaeva N I, Bockeria L A
The abrupt cessation of effective cardiac function that is generally due to heart rhythm disorders can cause sudden and unexpected death at any age and is referred to as a syndrome called "sudden cardiac death" (SCD). Annually, about 400,000 cases of SCD occur in the United States alone. Less than 5% of the resuscitation techniques are effective. The prevalence of SCD in a population rises with age according to the prevalence of coronary artery disease, which is the most common cause of sudden cardiac arrest...
April 2016: Acta Naturae
https://www.readbyqxmd.com/read/27378596/predictors-of-ventricular-arrhythmias-and-sudden-death-in-a-qu%C3%A3-bec-cohort-with-brugada-syndrome
#19
Lena Rivard, Antoine Roux, Isabelle Nault, Jean Champagne, Jean-François Roux, Rafik Tadros, Mario Talajic, Julia Cadrin-Tourigny, Azadeh Shohoudi, Blandine Mondésert, Denis Roy, Laurent Macle, Jason Andrade, Katia Dyrda, Marc Dubuc, Peter G Guerra, Jean-François Sarrazin, Bernard Thibault, Paul Khairy
BACKGROUND: Patients with Brugada syndrome (BrS) are at risk for ventricular arrhythmias (VAs) and sudden death. Identification of high-risk individuals beyond those with syncope or resuscitated sudden death remains a major challenge. METHODS: We assessed the value of clinical, electrophysiological, and electrocardiographic (ECG) features, including depolarization and repolarization metrics, in predicting arrhythmic events and sudden death in consecutive patients with BrS diagnosed between 2002 and 2013 in Quebec, Canada...
March 31, 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/27303672/next-generation-sequencing-in-post-mortem-genetic-testing-of-young-sudden-cardiac-death-cases
#20
REVIEW
Najim Lahrouchi, Elijah R Behr, Connie R Bezzina
Sudden cardiac death (SCD) in the young (<40 years) occurs in the setting of a variety of rare inherited cardiac disorders and is a disastrous event for family members. Establishing the cause of SCD is important as it permits the pre-symptomatic identification of relatives at risk of SCD. Sudden arrhythmic death syndrome (SADS) is defined as SCD in the setting of negative autopsy findings and toxicological analysis. In such cases, reaching a diagnosis is even more challenging and post-mortem genetic testing can crucially contribute to the identification of the underlying cause of death...
2016: Frontiers in Cardiovascular Medicine
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