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Sudden arrhythmic death syndrome

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https://www.readbyqxmd.com/read/29761670/arrhythmic-events-in-brugada-syndrome-a-nationwide-israeli-survey-of-the-clinical-characteristics-treatment-and-long-term-follow-up-israbru-vf
#1
Eran Leshem, Michael Rahkovich, Anna Mazo, Mahmoud Suleiman, Miri Blich, Avishag Laish-Farkash, Yuval Konstantino, Rami Fogelman, Boris Strasberg, Michael Geist, Israel Chetboun, Moshe Swissa, Michael Ilan, Aharon Glick, Yoav Michowitz, Raphael Rosso, Michael Glikson, Bernard Belhassen
BACKGROUND: Limited information exists about detailed clinical characteristics and management of the small subset of Brugada syndrome (BrS) patients who had an arrhythmic event (AE). OBJECTIVES: To conduct the first nationwide survey focused on BrS patients with documented AE. METHODS: Israeli electrophysiology units participated if they had treated BrS patients who had cardiac arrest (CA) (lethal/aborted; group 1) or experienced appropriate therapy for tachyarrhythmias after prophylactic implantable cardioverter defibrillator (ICD) implantation (group 2)...
May 2018: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29758173/lack-of-genotype-phenotype-correlation-in-families-who-had-brugada-syndrome-and-sudden-arrhythmic-death-syndrome-with-reported-pathogenic-scn1b-variants
#2
Belinda Gray, Can Hasdemir, Jodie Ingles, Takeshi Aiba, Naomasa Makita, Vincent Probst, Arthur A M Wilde, Ruth Newbury-Ecob, Mary N Sheppard, Christopher Semsarian, Raymond W Sy, Elijah R Behr
BACKGROUND: There is limited evidence that Brugada syndrome (BrS) is due to SCN1B variants (BrS5). This gene may be inappropriately included in routine genetic testing panels for BrS or sudden arrhythmic death syndrome (SADS). OBJECTIVE: We sought to characterize the genotype-phenotype correlation in families who had BrS and SADS with reportedly pathogenic SCN1B variants and to review their pathogenicity. METHODS: Families with BrS and SADS were assessed from 6 inherited arrhythmia centers worldwide, and a comprehensive literature review was performed...
May 3, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29748178/prognostic-significance-of-the-sodium-channel-blocker-test-in-patients-with-brugada-syndrome
#3
Akira Ueoka, Hiroshi Morita, Atsuyuki Watanabe, Yoshimasa Morimoto, Satoshi Kawada, Motomi Tachibana, Masakazu Miyamoto, Koji Nakagawa, Nobuhiro Nishii, Hiroshi Ito
BACKGROUND: A drug provocation test using a sodium channel blocker (SCB) can unmask a type 1 ECG pattern in patients with Brugada syndrome. However, the prognostic value of the results of an SCB challenge is limited in patients with non-type 1 ECG. We investigated the associations of future risk for ventricular fibrillation with SCB-induced ECG changes and ventricular tachyarrhythmias (VTAs). METHODS AND RESULTS: We administered intravenous pilsicainide to 245 consecutive patients with Brugada syndrome (181 patients with spontaneous type 1 ECG, 64 patients with non-type 1 ECG)...
May 10, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29709101/genotype-phenotype-relationship-and-risk-stratification-in-loss-of-function-scn5a-mutation-carriers
#4
Tomas Robyns, Dieter Nuyens, Bert Vandenberk, Cuno Kuiperi, Anniek Corveleyn, Jeroen Breckpot, Christophe Garweg, Joris Ector, Rik Willems
INTRODUCTION: Loss-of-function (LoF) mutations in the SCN5A gene cause multiple phenotypes including Brugada Syndrome (BrS) and a diffuse cardiac conduction defect. Markers of increased risk for sudden cardiac death (SCD) in LoF SCN5A mutation carriers are ill defined. We hypothesized that late potentials and fragmented QRS would be more prevalent in SCN5A mutation carriers compared to SCN5A-negative BrS patients and evaluated risk markers for SCD in SCN5A mutation carriers. METHODS: We included all SCN5A loss-of-function mutation carriers and SCN5A-negative BrS patients from our center...
April 30, 2018: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/29649615/fever-related-arrhythmic-events-in-the-multicenter-survey-on-arrhythmic-events-in-brugada-syndrome-sabrus
#5
Yoav Michowitz, Anat Milman, Georgia Sarquella-Brugada, Antoine Andorin, Jean Champagne, Pieter G Postema, Ruben Casado-Arroyo, Eran Leshem, Jimmy Jm Juang, Carla Giustetto, Jacob Tfelt-Hansen, Yanushi D Wijeyeratne, Christian Veltmann, Domenico Corrado, Sung-Hwan Kim, Pietro Delise, Shingo Maeda, Jean-Baptiste Gourraud, Frederic Sacher, Philippe Mabo, Yoshihide Takahashi, Tsukasa Kamakura, Takeshi Aiba, Giulio Conte, Aviram Hochstadt, Yuka Mizusawa, Michael Rahkovich, Elena Arbelo, Zhengrong Huang, Isabelle Denjoy, Carlo Napolitano, Ramon Brugada, Leonardo Calo, Silvia G Priori, Masahiko Takagi, 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: The literature on fever related arrhythmic events (AE) in Brugada syndrome (BrS) is currently limited to few case reports and small series. OBJECTIVE: The current study aims to describe the characteristics of fever-related AE in a large cohort of BrS patients. METHODS: SABRUS is a multicenter study on 678 BrS patients with first AE documented at time of aborted cardiac arrest (ACA) (n=426) or after prophylactic ICD implantation (n=252)...
April 9, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29574456/modeling-short-qt-syndrome-using-human-induced-pluripotent-stem-cell-derived-cardiomyocytes
#6
Ibrahim El-Battrawy, Huan Lan, Lukas Cyganek, Zhihan Zhao, Xin Li, Fanis Buljubasic, Siegfried Lang, Gökhan Yücel, Katherine Sattler, Wolfram-Hubertus Zimmermann, Jochen Utikal, Thomas Wieland, Ursula Ravens, Martin Borggrefe, Xiao-Bo Zhou, Ibrahim Akin
BACKGROUND: Short QT syndrome (SQTS), a disorder associated with characteristic ECG QT-segment abbreviation, predisposes affected patients to sudden cardiac death. Despite some progress in assessing the organ-level pathophysiology and genetic changes of the disorder, the understanding of the human cellular phenotype and discovering of an optimal therapy has lagged because of a lack of appropriate human cellular models of the disorder. The objective of this study was to establish a cellular model of SQTS using human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs)...
March 24, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29574140/copy-number-variations-of-scn5a-in-brugada-syndrome-scn5a-cnvs-in-brs
#7
Keiko Sonoda, Seiko Ohno, Junichi Ozawa, Mamoru Hayano, Tetsuhisa Hattori, Atsushi Kobori, Mitsuhiko Yahata, Isao Aburadani, Seiichi Watanabe, Yuichi Matsumoto, Takeru Makiyama, Minoru Horie
BACKGROUND: Loss-of-function mutations in SCN5A are associated in ∼20% of Brugada syndrome (BrS) patients. Copy number variations (CNVs) have been shown to be associated with several inherited arrhythmia syndromes. OBJECTIVE: We aimed to investigate SCN5A CNVs among BrS probands. METHODS: The study cohort consisted of 151 BrS probands who were symptomatic or had a family history of BrS, sudden death, syncope or arrhythmic diseases. We performed sequence analysis of SCN5A by Sanger method...
March 21, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29550106/meta-analysis-of-t-peak-t-end-and-t-peak-t-end-qt-ratio-for-risk-stratification-in-congenital-long-qt-syndrome
#8
Gary Tse, Mengqi Gong, Lei Meng, Cheuk Wai Wong, Stamatis Georgopoulos, George Bazoukis, Martin C S Wong, Konstantinos P Letsas, Vassilios S Vassiliou, Yunlong Xia, Adrian M Baranchuk, Gan-Xin Yan, Tong Liu
BACKGROUND AND OBJECTIVES: Congenital long QT syndrome (LQTS) predisposes affected individuals to ventricular tachycardia/fibrillation (VF/VF), potentially resulting in sudden cardiac death. The Tpeak -Tend interval and the Tpeak -Tend /QT ratio, electrocardiographic markers of dispersion of ventricular repolarization, were proposed for risk stratification but their predictive values in LQTS have been controversial. A systematic review and meta-analysis was conducted to examine the value of Tpeak -Tend intervals and Tpeak -Tend /QT ratios in predicting arrhythmic and mortality outcomes in congenital LQTS...
March 6, 2018: Journal of Electrocardiology
https://www.readbyqxmd.com/read/29544603/the-diagnostic-yield-of-brugada-syndrome-after-sudden-death-with-normal-autopsy
#9
Michael Papadakis, Efstathios Papatheodorou, Greg Mellor, Hariharan Raju, Rachel Bastiaenen, Yanushi Wijeyeratne, Sara Wasim, Bode Ensam, Gherardo Finocchiaro, Belinda Gray, Aneil Malhotra, Andrew D'Silva, Nina Edwards, Della Cole, Virginia Attard, Velislav N Batchvarov, Maria Tome-Esteban, Tessa Homfray, Mary N Sheppard, Sanjay Sharma, Elijah R Behr
BACKGROUND: Familial evaluation after a sudden death with negative autopsy (sudden arrhythmic death syndrome; SADS) may identify relatives at risk of fatal arrhythmias. OBJECTIVES: This study aimed to assess the impact of systematic ajmaline provocation testing using high right precordial leads (RPLs) on the diagnostic yield of Brugada syndrome (BrS) in a large cohort of SADS families. METHODS: Three hundred three SADS families (911 relatives) underwent evaluation with resting electrocardiogram using conventional and high RPLs, echocardiography, exercise, and 24-h electrocardiogram monitor...
March 20, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29454138/prognostic-implications-of-early-monomorphic-and-non-monomorphic-tachyarrhythmias-in-patients-discharged-with-acute-coronary-syndrome
#10
Jo-Jo Hai, Ka-Chun Un, Chun-Ka Wong, Ka-Lam Wong, Zhe-Yu Zhang, Pak-Hei Chan, Chu-Pak Lau, Chung-Wah Siu, Hung-Fat Tse
BACKGROUND: The prognostic implication of early ventricular tachyarrhythmias (VTs) after acute coronary syndrome (ACS) remains unclear. OBJECTIVE: We sought to investigate the clinical outcomes of early monomorphic and non-monomorphic VTs that occur within 48 hours in patients after ACS. METHODS: We retrospectively reviewed the clinical outcomes of 2033 (mean age 67.0 ± 13.4 years; 73.1% men) consecutive patients who presented with ACS from 2004 to 2015...
February 14, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29397883/relation-of-the-brugada-phenocopy-to-hyperkalemia-from-the-international-registry-on-brugada-phenocopy
#11
Grace Xu, Byron H Gottschalk, Daniel D Anselm, David G Benditt, Ankit Maheshwari, Shiva Sreenivasan, Raed Abu Shama, Gregory Dendramis, Héctor Barajas-Martínez, José Manuel Rubio Campal, Sam G Aznaurov, Adrian Baranchuk
Brugada phenocopies (BrPs) are clinical entities that differ in etiology from true congenital Brugada syndrome but have identical electrocardiographic (ECG) patterns. Hyperkalemia is known to be one of the causes of BrP. The aim of this study was to determine the clinical characteristics and evolution of hyperkalemia-induced BrP. Data from 27 cases of hyperkalemia-induced BrP were collected from the International Registry at www.brugadaphenocopy.com. Data were extracted from publications. Of the 27 patients included in the analysis, 18 (67%) were male; mean age was 53 ± 15 years (range 31 to 89)...
March 15, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29372449/unsafe-drug-use-and-arrhythmic-events-in-brugada-patients-with-icd-results-of-a-long-term-follow-up
#12
Diogo de Almeida Fernandes, Natália António, Marta Madeira, Pedro Sousa, Miguel Ventura, João Cristóvão, José Nascimento, Luís Elvas, Lino Gonçalves, Guilherme Mariano Pego
PURPOSE: Brugada syndrome is a hereditary disease linked with an increased risk of sudden death that may require an implantable cardioverter-defibrillator (ICD) in order to halt the arrhythmic events. The aim of this study was to identify possible triggers for appropriate ICD therapies in patients with Brugada syndrome, focusing on their past and current therapeutic profiles. METHODS: Thirty patients with high-risk Brugada syndrome, with ICD implanted at the Coimbra Hospital and University Center, were enrolled...
February 2018: Cardiovascular Drugs and Therapy
https://www.readbyqxmd.com/read/29325976/profile-of-patients-with-brugada-syndrome-presenting-with-their-first-documented-arrhythmic-event-data-from-the-survey-on-arrhythmic-events-in-brugada-syndrome
#13
Anat Milman, Antoine Andorin, Jean-Baptiste Gourraud, Pieter G Postema, Frederic Sacher, Philippe Mabo, Sung-Hwan Kim, Jimmy J M 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 patients with Brugada syndrome (BrS) presenting their first arrhythmic event (AE) after prophylactic implantation of an implantable cardioverter-defibrillator (ICD) is limited. OBJECTIVES: The objectives of this study were (1) to compare clinical, electrocardiographic, electrophysiological, and genetic profiles of patients who exhibited their first documented AE as aborted cardiac arrest (group A) with profiles of those in whom the AE was documented after prophylactic ICD implantation (group B) and (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
#14
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...
March 2018: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/29290967/modelling-the-effects-of-chloroquine-on-kcnj2-linked-short-qt-syndrome
#15
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 ( I K1 ) 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
#16
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-qt-syndrome
#17
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
#18
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
#19
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
#20
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...
January 15, 2018: American Journal of Cardiology
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