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https://www.readbyqxmd.com/read/28819034/when-high-throughput-meets-mechanistic-studies-a-state-of-the-art-approach-in-brugada-syndrome
#1
EDITORIAL
Bettina Heidecker
No abstract text is available yet for this article.
August 18, 2017: Circulation Research
https://www.readbyqxmd.com/read/28810115/current-guidelines-on-syncope
#2
Maheedhar Gedela, Naveen Rajpurohit, Kashif A Shaikh, Muhammad Omar, Scott Pham
Syncope is a very commonly encountered clinical problem in general practice and in the emergency department. In the evaluation of syncope, it is important to identify the specific cause to determine the treatment, to estimate the precise risk to a patient, and to reduce recurrence. Sometimes, making a diagnosis of syncope is difficult, as different mechanisms may often coexist. Syncope causes a significant impact on quality of life due to associated risk of physical injury. In particular, syncope can be a precursor to sudden cardiac death in patients with underlying cardiac disease...
November 2016: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/28800177/exercise-stress-test-reveals-ineligibility-for-subcutaneous-implantable-cardioverter-defibrillator-in-patients-with-brugada-syndrome
#3
Motomi Tachibana, Nobuhiro Nishii, Hiroshi Morita, Koji Nakagawa, Atsuyuki Watanabe, Kazufumi Nakamura, Hiroshi Ito
BACKGROUND: The eligibility of patients with Brugada syndrome (BrS) for implantation of a subcutaneous implantable cardioverter defibrillator (S-ICD) is not well known. This study aimed to clarify the eligibility of BrS patients for S-ICD using electrocardiography (ECG) at rest and during exercise testing. We also analyzed factors associated with ineligibility for S-ICD from standard 12-lead ECG at rest. METHODS: We enrolled 110 consecutive BrS patients who visited Okayama university hospital from December 2015 to December 2016...
August 11, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28797161/masked-inherited-primary-arrhythmia-syndromes-in-sudden-cardiac-death-patients-accompanied-by-coronary-vasospasm
#4
Ki Hong Lee, Hyung Wook Park, Jeong Nam Eun, Jeong Gwan Cho, Nam Sik Yoon, Mi Ran Kim, Yo Han Ku, Hyukjin Park, Seung Hun Lee, Jeong Han Kim, Min Chul Kim, Woo Jin Kim, Hyun Kuk Kim, Jae Yeong Cho, Keun-Ho Park, Doo Sun Sim, Hyun Ju Yoon, Kye Hun Kim, Young Joon Hong, Ju Han Kim, Youngkeun Ahn, Myung Ho Jeong, Jong Chun Park
Background/Aims: Coronary vasospasms are one of the important causes of sudden cardiac death (SCD). Provocation of coronary vasospasms can be useful, though some results may lead to false positives, with patients potentially experiencing recurrent SCD despite appropriate medical treatments. We hypothesized that it is not coronary vasospasms but inherited primary arrhythmia syndromes (IPAS) that underlie the development of SCD. Methods: We analyzed 74 consecutive patients (3...
August 11, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28789916/flecainide-reduces-ventricular-arrhythmias-in-patients-with-genotype-ryr2-positive-catecholaminergic-polymorphic-ventricular-tachycardia
#5
Fernando Wangüemert Pérez, Julio Salvador Hernández Afonso, María Del Val Groba Marco, Eduardo Caballero Dorta, Luis Álvarez Acosta, Oscar Campuzano Larrea, Guillermo Pérez, Josep Brugada Terradellas, Ramón Brugada Terradellas
INTRODUCTION AND OBJECTIVES: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited disease characterized by polymorphic or bidirectional ventricular arrhythmias (VA) triggered by physical or emotional stress in young people with a structurally normal heart. Beta-blockers are the cornerstone of treatment, while flecainide has recently been incorporated into the therapeutic arsenal. The aim of this study was to report our experience with this drug. METHODS: The cohort included 174 genotype-positive CPVT-patients from 7 families...
August 5, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28782696/heritability-in-a-scn5a-mutation-founder-population-with-increased-female-susceptibility-to-non-nocturnal-ventricular-tachyarrhythmia-and-sudden-cardiac-death
#6
Rachel M A Ter Bekke, Aaron Isaacs, Andrei Barysenka, Marije B Hoos, Jan D H Jongbloed, Jan C A Hoorntje, Alfons S M Patelski, Apollonia T J M Helderman-van den Enden, Arthur van den Wijngaard, Monika Stoll, Paul G A Volders
BACKGROUND: Heritable cardiac sodium-channel dysfunction is associated with various arrhythmia syndromes, some predisposing to ventricular fibrillation. Phenotypic diversity among carriers of identical-by-descent mutations is often remarkable, suggesting influences of genetic modifiers. OBJECTIVE: We identified a unique SCN5A-mutation founder population with mixed clinical phenotypes and sudden cardiac death, and investigated the heritability of electromechanical traits besides the SCN5A-mutation effect...
August 3, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28781860/masquerading-bundle-branch-block-obscuring-the-diagnosis-of-brugada-syndrome-an-electrocardiographic-and-vectorcardiographic-study
#7
Isabel V Konopka, Hugo A Garro, Rita B Tepper, Norma Pizzarelli, Mario D Gonzalez, Rafael S Acunzo
We describe the induction of a masquerading bundle branch block in two patients with Brugada syndrome following the administration of Ajmaline. The development of this conduction disturbance prevented the correct electrocardiographic diagnosis. However, the simultaneously obtained vectocardiogram identified both the Brugada pattern and the masquerading bundle branch block.
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28781849/double-jeopardy-long-qt3-and-brugada-syndromes
#8
Amneet Sandhu, Ryan T Borne, Chandara Mam, T Jared Bunch, Ryan G Aleong
Mutations in the SCN5A gene are linked to both the long QT syndrome 3 and Brugada syndrome with few reports describing an overlapping phenotype. We present a unique case and discuss clinical considerations of a patient concurrently exhibiting such conditions with genetic analysis confirming an SCN5A mutation.
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28780562/rationale-and-design-of-a-multicentre-prospective-randomised-controlled-clinical-trial-to-evaluate-the-efficacy-of-the-adipose-graft-transposition-procedure-in-patients-with-a-myocardial-scar-the-agtp-ii-trial
#9
Paloma Gastelurrutia, Carolina Gálvez-Montón, Maria Luisa Cámara, Juan Bustamante-Munguira, Pablo García-Pavia, Pablo Avanzas, J Alberto San Román, Domingo Pascual-Figal, Eduardo de Teresa, Maria G Crespo-Leiro, Nicolás Manito, Julio Núñez, Francisco Fernández-Avilés, Ángel Caballero, Albert Teis, Josep Lupón, Ramón Brugada, Carlos Martín, Jacobo Silva, Ana Revilla-Orodea, Sergio J Cánovas, Jose M Melero, Jose J Cuenca-Castillo, Angel Gonzalez-Pinto, Antoni Bayes-Genis
INTRODUCTION: Cardiac adipose tissue is a source of progenitor cells with regenerative capacity. Studies in rodents demonstrated that the intramyocardial delivery of cells derived from this tissue improves cardiac function after myocardial infarction (MI). We developed a new reparative approach for damaged myocardium that integrates the regenerative properties of cardiac adipose tissue with tissue engineering. In the adipose graft transposition procedure (AGTP), we dissect a vascularised flap of autologous pericardial adipose tissue and position it over the myocardial scarred area...
August 4, 2017: BMJ Open
https://www.readbyqxmd.com/read/28771489/additional-value-of-screening-for-minor-genes-and-copy-number-variants-in-hypertrophic-cardiomyopathy
#10
Irene Mademont-Soler, Jesus Mates, Raquel Yotti, Maria Angeles Espinosa, Alexandra Pérez-Serra, Ana Isabel Fernandez-Avila, Monica Coll, Irene Méndez, Anna Iglesias, Bernat Del Olmo, Helena Riuró, Sofía Cuenca, Catarina Allegue, Oscar Campuzano, Ferran Picó, Carles Ferrer-Costa, Patricia Álvarez, Sergio Castillo, Pablo Garcia-Pavia, Esther Gonzalez-Lopez, Laura Padron-Barthe, Aranzazu Díaz de Bustamante, María Teresa Darnaude, José Ignacio González-Hevia, Josep Brugada, Francisco Fernandez-Aviles, Ramon Brugada
INTRODUCTION: Hypertrophic cardiomyopathy (HCM) is the most prevalent inherited heart disease. Next-generation sequencing (NGS) is the preferred genetic test, but the diagnostic value of screening for minor and candidate genes, and the role of copy number variants (CNVs) deserves further evaluation. METHODS: Three hundred and eighty-seven consecutive unrelated patients with HCM were screened for genetic variants in the 5 most frequent genes (MYBPC3, MYH7, TNNT2, TNNI3 and TPM1) using Sanger sequencing (N = 84) or NGS (N = 303)...
2017: PloS One
https://www.readbyqxmd.com/read/28764236/clinical-significance-of-the-forsaken-avr-in-evaluation-of-tachyarrhythmias-a-reminder
#11
Dharmendra Jain, Pradeep Nigam, Manoj Indurkar, Riyas Chiramkara
INTRODUCTION: Mechanism of a regular, monomorphic Wide QRS Complex Tachycardia (WCT) is an important diagnostic challenge in day to day practice for the clinicians and affects further management and prognosis. Many of the WCT and Narrow Complex Tachycardia (NCT) produce certain characteristic changes in lead aVR by which we can differentiate between them. AIM: The present study was aimed to evaluate tachyarrhythmias in relation to lead aVR and to highlight the clinical significance of lead aVR, "The Neglected Lead"...
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28759457/genetic-causes-of-sudden-cardiac-death-in-children-inherited-arrhythmogenic-diseases
#12
Gaetano Vacanti, Riccardo Maragna, Andrea Mazzanti, Silvia G Priori
PURPOSE OF REVIEW: In this chapter we will discuss the most recent and relevant evidences published in the field of inherited arrhythmogenic disorders, focusing on the so called 'channelopathies' that are associated with sudden cardiac death (SCD) in children: long QT syndrome (LQTS), short QT syndrome (SQTS), Brugada syndrome (BrS), and catecholaminergic polymorphic ventricular tachycardia (CPVT). RECENT FINDINGS: We will discuss the latest diagnostic criteria for channelopathies released by the European Society of Cardiology, the new data on BrS in children and the recent evidence supporting a genotype-specific therapy for LQTS type 3...
July 28, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28751544/the-impact-of-cryoballoon-versus-radiofrequency-ablation-for-paroxysmal-atrial-fibrillation-on-healthcare-utilization-and-costs-an-economic-analysis-from-the-fire-and-ice-trial
#13
K R Julian Chun, Josep Brugada, Arif Elvan, Laszlo Gellér, Matthias Busch, Alberto Barrera, Richard J Schilling, Matthew R Reynolds, Robert B Hokanson, Reece Holbrook, Benedict Brown, Michael Schlüter, Karl-Heinz Kuck
BACKGROUND: This study sought to assess payer costs following cryoballoon or radiofrequency current (RFC) catheter ablation of paroxysmal atrial fibrillation in the randomized FIRE AND ICE trial. METHODS AND RESULTS: A trial period analysis of healthcare costs evaluated the impact of ablation modality (cryoballoon versus RFC) on differences in resource use and associated payer costs. Analyses were based on repeat interventions, rehospitalizations, and cardioversions during the trial, with unit costs based on 3 national healthcare systems (Germany [€], the United Kingdom [£], and the United States [$])...
July 27, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28748141/recording-of-isolated-very-delayed-potentials-on-the-right-ventricular-epicardium-in-a-patient-with-brugada-syndrome
#14
Atsuyuki Watanabe, Hiroshi Morita, Sho Tsushima, Koji Nakagawa, Nobuhiro Nishii, Hiroshi Ito
No abstract text is available yet for this article.
July 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28742119/doestrare-a-statistical-test-to-identify-local-enrichments-in-rare-genomic-variants-associated-with-disease
#15
Elodie Persyn, Matilde Karakachoff, Solena Le Scouarnec, Camille Le Clézio, Dominique Campion, French Exome Consortium, Jean-Jacques Schott, Richard Redon, Lise Bellanger, Christian Dina
Next-generation sequencing technologies made it possible to assay the effect of rare variants on complex diseases. As an extension of the "common disease-common variant" paradigm, rare variant studies are necessary to get a more complete insight into the genetic architecture of human traits. Association studies of these rare variations show new challenges in terms of statistical analysis. Due to their low frequency, rare variants must be tested by groups. This approach is then hindered by the fact that an unknown proportion of the variants could be neutral...
2017: PloS One
https://www.readbyqxmd.com/read/28739038/anesthetic-and-perioperative-management-of-patients-with-brugada-syndrome
#16
REVIEW
Gregory Dendramis, Claudia Paleologo, Giuseppe Sgarito, Umberto Giordano, Roberto Verlato, Adrian Baranchuk, Pedro Brugada
Brugada syndrome (BrS) is an arrhythmogenic disease reported to be one among the leading causes of cardiac death in subjects under the age of 40 years. In these patients, episodes of lethal arrhythmias may be induced by several factors or situations, and for this reason, management during anesthesia and surgery must provide some precautions and drugs restrictions. To date, it is difficult to formulate guidelines for anesthetic management of patients with BrS because of the absence of prospective studies, and there is not a definite recommendation for neither general nor regional anesthesia, and there are no large studies in merit...
June 28, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28737621/baroreflex-activation-therapy-a-new-approach-to-the-management-of-advanced-heart-failure-with-reduced-ejection-fraction
#17
Edoardo Gronda, Darrel Francis, Faiez Zannad, Christian Hamm, Josep Brugada, Emilio Vanoli
: Chronic heart failure is a common clinical condition characterized by persistent excessive sympathetic nervous system activation. The derangement of the sympathetic activity has relevant implications for disease progression and patient survival. Aiming to positively impact patient outcome, autonomic nervous system modulatory therapies have been developed and tested in animal and clinical studies. As a general gross assumption, direct vagal stimulation and baroreflex activation are considered equivalent. This assumption does not take into account the fact that direct cervical vagal nerve stimulation involves activation of both afferent and efferent fibers innervating not only the heart, but the entire visceral system, leading to undesired responses to and from this compartment...
September 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28734879/ventricular-arrhythmias-ablation-in-brugada-syndrome-current-and-future-directions
#18
Carlo Pappone, Josep Brugada
No abstract text is available yet for this article.
July 19, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28734073/differential-calcium-sensitivity-in-nav-1-5-mixed-syndrome-mutants
#19
Mena Abdelsayed, Alban-Elouen Baruteau, Karen Gibbs, Shubhayan Sanatani, Andrew D Krahn, Vincent Probst, Peter C Ruben
INTRODUCTION: Inherited arrhythmias may arise from mutations in the SCN5a gene, which encodes the cardiac voltage-gated sodium channel, NaV 1.5. Mutants in NaV 1.5 result in Brugada Syndrome (BrS1), Long-QT Syndrome (LQT3), or mixed syndromes (an overlap of BrS1/LQT3). Exercise is a potential arrhythmogenic trigger in mixed syndromes. We sought to determine the effects of elevated cytosolic calcium, common during exercise, in mixed syndrome NaV 1.5 mutants. METHODS: We used whole-cell patch-clamp to assess the biophysical properties of NaV 1...
July 22, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28728742/a-clinical-score-model-to-predict-lethal-events-in-young-patients-%C3%A2-19-years-with-the-brugada-syndrome
#20
M Cecilia Gonzalez Corcia, Juan Sieira, Gudrun Pappaert, Carlo de Asmundis, Gian Battista Chierchia, Andrea Sarkozy, Pedro Brugada
Risk stratification in Brugada syndrome in young patients remains challenging. We investigated the clinical characteristics, prognosis, and risk in young patients with the Brugada syndrome. We studied 95 patients with the Brugada syndrome aged ≤19 years. The median age at diagnosis was 12.9 years. The clinical presentation was sudden cardiac death in 7% and syncope in 21%. The remaining 72% were asymptomatic at diagnosis. Electrical abnormalities were present in 36%, including spontaneous type I electrocardiogram (12%), sinus node dysfunction (9%), atrioventricular block (17%), intraventricular conduction delay (16%), and atrial arrhythmias (8%)...
June 15, 2017: American Journal of Cardiology
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