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https://www.readbyqxmd.com/read/28534204/-ventricular-arrhythmias-in-ion-channel-diseases
#1
REVIEW
Christian Wolpert, Mara Vogel, Christian Nagel, Claudia Herrera-Siklody, Norman Rüb
In patients with ion channel disease the predominant arrhythmias are polymorphic ventricular tachycardias (VT), torsade de pointes tachycardia and ventricular fibrillation (VF). In only extremely rare cases is very rapid monomorphic ventricular tachycardia observed. This is why implantable cardioverter-defibrillators (ICDs) should always be programmed for treatment of VF only with high detection rates to avoid inappropriate discharges. In idiopathic VF and catecholaminergic polymorphic ventricular tachycardia (CPVT), no baseline electrocardiographic abnormalities can be detected, whereas in Brugada syndrome, long QT syndrome, early repolarisation syndrome and Anderson-Tawil syndrome alterations of the baseline ECG are very important to identify patients at risk...
May 22, 2017: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/28528724/twelve-lead-ambulatory-electrocardiographic-monitoring-in-brugada-syndrome-potential-diagnostic-and-prognostic-implications
#2
Belinda Gray, Adrienne Kirby, Peter Kabunga, Saul B Freedman, Laura Yeates, Ajita Kanthan, Caroline Medi, Anthony Keech, Christopher Semsarian, Raymond W Sy
BACKGROUND: Patients with Brugada syndrome (BrS) are diagnosed and risk stratified on the basis of a spontaneous or drug-induced type 1 electrocardiographic (ECG) pattern, often at single time points not accounting for variation throughout the day. OBJECTIVES: The purpose of this study was to prospectively assess the overall burden of type 1 Brugada ECG changes using 12-lead 24-hour Holter monitoring and evaluate association with cardiac events. METHODS: From July 1, 2013 to December 31, 2015, patients with BrS were recruited from 3 Australian centers and the Australian Genetic Heart Disease Registry...
June 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28527921/drug-induced-fatal-arrhythmias-acquired-long-qt-and-brugada-syndromes
#3
REVIEW
Isik Turker, Tomohiko Ai, Hideki Itoh, Minoru Horie
Since the early 1990s, the concept of primary "inherited" arrhythmia syndromes or ion channelopathies has evolved rapidly as a result of revolutionary progresses made in molecular genetics. Alterations in genes coding for membrane proteins such as ion channels or their associated proteins responsible for the generation of cardiac action potentials (AP) have been shown to cause specific malfunctions which eventually lead to cardiac arrhythmias. These arrhythmic disorders include congenital long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, short QT syndrome, progressive cardiac conduction disease, etc...
May 17, 2017: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28523371/impact-of-electrocardiogram-screening-during-drug-challenge-test-for-the-prediction-of-t-wave-oversensing-by-a-subcutaneous-implantable-cardioverter-defibrillator-in-patients-with-brugada-syndrome
#4
Tsukasa Kamakura, Mitsuru Wada, Kohei Ishibashi, Yuko Y Inoue, Koji Miyamoto, Hideo Okamura, Satoshi Nagase, Takashi Noda, Takeshi Aiba, Satoshi Yasuda, Kengo Kusano
Screening tests conducted at rest may be inadequate for the prediction of the T-wave oversensing (TWOS) in subcutaneous implantable cardioverter defibrillator (S-ICD) candidates with Brugada syndrome (BrS) because of the dynamic nature of electrocardiogram (ECG) morphology. We evaluated the utility of ECG screening during drug challenge (DC) for prediction of TWOS in BrS patients implanted with an S-ICD. The study enrolled 6 consecutive BrS patients implanted with an S-ICD. In addition to baseline ECG screening, pre-implant screening during DC using a sodium channel blocker was performed in all patients...
May 18, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28521022/management-of-untreatable-ventricular-arrhythmias-during-pharmacologic-challenges-with-sodium-channel-blockers-for-suspected-brugada-syndrome
#5
Stefano Poli, Mauro Toniolo, Massimo Maiani, Davide Zanuttini, Luca Rebellato, Igor Vendramin, Ermanno Dametto, Guglielmo Bernardi, Flavio Bassi, Carlo Napolitano, Ugolino Livi, Alessandro Proclemer
Pharmacologic challenge with sodium channel blockers is part of the diagnostic workout in patients with suspected Brugada syndrome. The test is overall considered safe but both ajmaline and flecainide detain well known pro-arrhythmic properties. Moreover, the treatment of patients with life-threatening arrhythmias during these diagnostic procedures is not well defined. Current consensus guidelines suggest to adopt cautious protocols interrupting the sodium channel blockers as soon as any ECG alteration appears...
May 17, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28500178/electrical-substrate-elimination-in-135-consecutive-patients-with-brugada-syndrome
#6
Carlo Pappone, Josep Brugada, Gabriele Vicedomini, Giuseppe Ciconte, Francesco Manguso, Massimo Saviano, Raffaele Vitale, Amarild Cuko, Luigi Giannelli, Zarko Calovic, Manuel Conti, Paolo Pozzi, Andrea Natalizia, Simonetta Crisà, Valeria Borrelli, Ramon Brugada, Georgia Sarquella-Brugada, Marco Guazzi, Alessandro Frigiola, Lorenzo Menicanti, Vincenzo Santinelli
BACKGROUND: There is emerging evidence that localization and elimination of abnormal electric activity in the epicardial right ventricular outflow tract may be beneficial in patients with Brugada syndrome. METHODS AND RESULTS: A total of 135 symptomatic Brugada syndrome patients having implantable cardiac defibrillator were enrolled: 63 (group 1) having documented ventricular tachycardia (VT)/ventricular fibrillation (VF) and Brugada syndrome-related symptoms, and 72 (group 2) having inducible VT/VF without ECG documentation at the time of symptoms...
May 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28496938/brugada-syndrome-risk-stratification-and-management
#7
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/28472282/right-ventricular-outflow-tract-high-density-endocardial-unipolar-voltage-mapping-in-patients-with-brugada-syndrome-evidence-for-electroanatomical-abnormalities
#8
Konstantinos P Letsas, Michael Efremidis, Konstantinos Vlachos, Stamatis Georgopoulos, Nikolaos Karamichalakis, Dimitrios Asvestas, Kosmas Valkanas, Panagiotis Korantzopoulos, Tong Liu, Antonios Sideris
Aims: Epicardial structural abnormalities at the right ventricular outflow tract (RVOT) may provide the arrhythmia substrate in Brugada syndrome (BrS). Electroanatomical endocardial unipolar voltage mapping is an emerging tool that accurately identifies epicardial abnormalities in different clinical settings. This study investigated whether endocardial unipolar voltage mapping of the RVOT detects electroanatomical abnormalities in patients with BrS. Methods and results: Ten asymptomatic patients (8 males, 34...
May 2, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28469493/beyond-the-electrocardiogram-mutations-in-cardiac-ion-channel-genes-underlie-nonarrhythmic-phenotypes
#9
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/28436550/epicardial-brugada-syndrome-ablation-unmasking-inferior-j-waves
#10
Adam Lee, Heidi Kohler, Daniel Wright, Haris M Haqqani
Patients with Brugada syndrome are at risk of life threatening ventricular arrhythmias. Epicardial substrate ablation for Brugada syndrome has been described as a means of controlling these arrhythmias and recent reports describe elimination of the Brugada phenotype with ablation. We describe a unique case in which a patient developed inferior J waves with an early repolarization-type electrocardiogram following successful epicardial infundibular substrate ablation (which eliminated the Brugada syndrome ECG on ajmaline challenge)...
April 24, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28389016/myotonic-dystrophy-and-brugada-syndrome-a-common-pathophysiologic-pathway
#11
REVIEW
Andrés Ricardo Pérez-Riera, Adrian Baranchuk, Li Zhang, Raimundo Barbosa-Barros, Luiz Carlos de Abreu, Pedro Brugada
Type 1 myotonic dystrophy (DM1) is a hereditary neuromuscular disease affecting multiple organs in human adults. Here we report a 42-year-old man diagnosed with DM1. Having a history of progressive muscular weakness and gradual loss of visual acuity, he was referred to us by his ophthalmologist for risk assessment of undergoing cataract surgery. Cardiology workup revealed type 1 Brugada ECG pattern, positive late potentials and inducible ventricular fibrillation in an electrophysiology study. Literature review revealed that those ECG changes may be observed in DM1, suggesting that DM1 and Brugada syndrome may share a common pathophysiologic pathway...
March 14, 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/28381391/brugada-phenocopy-induced-by-propafenone-overdose-and-successful-treatment-a-case-report
#12
Mehmet Emre Arı, Filiz Ekici
BACKGROUND: Brugada syndrome is an inherited arrhythmogenic disease that may cause sudden cardiac death due to ventricular fibrillation in young adults. Brugada syndrome caused by propafenone intoxication has been noted rarely in the literature. We reported a rare case, Brugada-phenocopy due to propafenone intoxication and its treatment. CASE REPORT: A fifteen-year-old girl having a seizure was brought to the emergency room. She took 1.5 g propafenone (Rythmol, Abbott, Chicago, IL, USA) for suicidal intention...
April 6, 2017: Balkan Medical Journal
https://www.readbyqxmd.com/read/28379344/a-score-model-to-predict-risk-of-events-in-patients-with-brugada-syndrome
#13
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/28367844/surface-electrocardiogram-screening-for-subcutaneous-implantable-cardioverter-defibrillators-in-japanese-patients-with-and-without-brugada-syndrome
#14
Mihoko Kawabata, Masahiko Goya, Takeshi Sasaki, Shingo Maeda, Atsuhiko Yagishita, Yasuhiro Shirai, Masakazu Kaneko, Shinya Shiohira, Mitsuaki Isobe, Kenzo Hirao
BACKGROUND: Subcutaneous implantable cardioverter-defibrillators (S-ICD) could eliminate lead-associated complications. We assessed the prevalence of S-ICD ineligibility in conventional ICD recipients and compared it in patients with and without Brugada syndrome (BrS).Methods and Results:Consecutive patients with a transvenous ICD without an indication for antibradycardia pacing were assessed. A patient was considered eligible for S-ICD if the ECG satisfied the screening template, both supine and standing, in ≥1 lead...
March 28, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28364267/utility-of-12-lead-and-signal-averaged-holter-electrocardiograms-after-pilsicainide-provocation-for-risk-stratification-in-brugada-syndrome
#15
Jun Kakihara, Masahiko Takagi, Yusuke Hayashi, Hiroaki Tatsumi, Atsushi Doi, Minoru Yoshiyama
Non-invasive risk stratification for ventricular fibrillation (VF) in Brugada syndrome (BrS) has not been fully evaluated. The aim of this study was to assess the utility of signal-averaged Holter electrocardiogram (Holter SAECG) and 12-lead Holter electrocardiogram (Holter ECG) after a pilsicainide provocation test for non-invasive risk stratification in BrS. We enrolled 30 consecutive patients with BrS [divided into 2 groups: the VF group, those with a previous history of VF (n = 10); and the non-VF group, those without a history of VF (n = 20)] and 10 control subjects without type 1 ECG...
March 31, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28363711/prevalence-of-electrocardiographic-patterns-associated-with-sudden-cardiac-death-in-the-spanish-population-aged-40-years-or-older-results-of-the-ofrece-study
#16
Paula Awamleh García, Joaquín Jesús Alonso Martín, Catherine Graupner Abad, Rosa María Jiménez Hernández, Alejandro Curcio Ruigómez, Pedro Talavera Calle, Carmen Cristóbal Varela, José Serrano Antolín, Javier Muñiz, Juan José Gómez Doblas, Eulalia Roig
INTRODUCTION AND OBJECTIVES: Some electrocardiographic patterns are associated with an increased risk of sudden cardiac death due to ventricular arrhythmias. There is no information on the prevalence of these patterns in the general population in Spain. The objective of this study was to analyze the prevalence of these patterns and associated clinical and epidemiological factors. METHODS: This subanalysis of the OFRECE study selected a representative sample of the Spanish population aged ≥ 40 years...
March 28, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28343764/temporary-removal-channelopathies-genetic-testing-and-risk-stratification
#17
Arthur A M Wilde, Ahmad Amin
The publisher regrets that this article has been temporarily removed. A replacement will appear as soon as possible in which the reason for the removal of the article will be specified, or the article will be reinstated. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.
March 18, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28341781/genotype-phenotype-correlation-of-scn5a-mutation-for-the-clinical-and-electrocardiographic-characteristics-of-probands-with-brugada-syndrome-a-japanese-multicenter-registry
#18
Kenichiro Yamagata, Minoru Horie, Takeshi Aiba, Satoshi Ogawa, Yoshifusa Aizawa, Toru Ohe, Masakazu Yamagishi, Naomasa Makita, Harumizu Sakurada, Toshihiro Tanaka, Akihiko Shimizu, Nobuhisa Hagiwara, Ryoji Kishi, Yukiko Nakano, Masahiko Takagi, Takeru Makiyama, Seiko Ohno, Keiichi Fukuda, Hiroshi Watanabe, Hiroshi Morita, Kenshi Hayashi, Kengo Kusano, Shiro Kamakura, Satoshi Yasuda, Hisao Ogawa, Yoshihiro Miyamoto, Jamie D Kapplinger, Michael J Ackerman, Wataru Shimizu
Background -The genotype-phenotype correlation of SCN5A mutations as a predictor of cardiac events in Brugada syndrome remains controversial. We aimed to establish a registry limited to probands, with a long follow-up period, so that the genotype-phenotype correlation of SCN5A mutations in Brugada syndrome can be examined without patient selection bias. Methods -This multicenter registry enrolled 415 probands (n=403; men, 97%; age, 46±14 years) diagnosed with Brugada syndrome whose SCN5A gene was analyzed for mutations...
March 24, 2017: Circulation
https://www.readbyqxmd.com/read/28340026/high-rate-of-subcutaneous-implantable-cardioverter-defibrillator-sensing-screening-failure-in-patients-with-brugada-syndrome-a-comparison-with-other-inherited-primary-arrhythmia-syndromes
#19
Giulio Conte, Mihoko Kawabata, Carlo de Asmundis, Erika Taravelli, Francesco Petracca, Diego Ruggiero, Maria Luce Caputo, François Regoli, Gian-Battista Chierchia, Alessandra Chiodini, Alessandro Del Bufalo, Tiziano Moccetti, Masahiko Goya, Kenzo Hirao, Alessandro Vicentini, Gaetano M De Ferrari, Pedro Brugada, Angelo Auricchio
Aims: Subcutaneous implantable cardioverter-defibrillator (S-ICD) can avoid important complications associated with transvenous leads in patients with inherited primary arrhythmia syndromes, who do not need pacing therapy. Few data are available on the percentage of patients with inherited arrhythmia syndromes eligible for S-ICD implantation. Aim of this study was to analyse the eligibility for S-ICD in a series of patients with Brugada syndrome (BrS), and to compare it with patients with other channelopathies...
March 7, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28339570/frequency-of-different-electrocardiographic-abnormalities-in-a-large-cohort-of-spanish-workers
#20
Jorge Rodríguez-Capitán, Ana Fernández-Meseguer, José Manuel García-Pinilla, Eva Calvo-Bonacho, Manuel Jiménez-Navarro, Teresa García-Margallo, Fernando Cabrera-Bueno, Ignacio Echeverria-Lucotti, Juan José Gómez-Doblas, Eduardo De Teresa-Galván
Aims: Our aim was to describe the electrocardiographic findings of a large sample of Spanish workers from several different employment sectors. Methods and results: Between May 2008 and November 2010, 13 495 consecutive 12-lead resting electrocardiograms (ECGs) were obtained during health examinations of working adults aged 16-74 years in 5 cities in different regions of Spain. Of those, 13 179 ECGs suitable for interpretation were included in this study. All tracings were classified by the same cardiologist, according to the Minnesota Code criteria...
October 6, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
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