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https://www.readbyqxmd.com/read/28718687/do-sodium-channel-proteolytic-fragments-regulate-sodium-channel-expression
#1
Donatus O Onwuli, Laia Yañez-Bisbe, Mellina Pinsach-Abuin, Anna Tarradas, Ramon Brugada, John Greenman, Sara Pagans, Pedro Beltran-Alvarez
The cardiac voltage-gated sodium channel (gene: SCN5A, protein: NaV1.5) is responsible for the sodium current that initiates the cardiomyocyte action potential. Research into the mechanisms of SCN5A gene expression has gained momentum over the last few years. We have recently described the transcriptional regulation of SCN5A by GATA4 transcription factor. In this addendum to our study, we report our observations that 1) the linker between domains I and II (LDI-DII) of NaV1.5 contains a nuclear localisation signal (residues 474-481) that is necessary to localise LDI-DII into the nucleus, and 2) nuclear LDI-DII activates the SCN5A promoter in gene reporter assays using cardiac-like H9c2 cells...
July 18, 2017: Channels
https://www.readbyqxmd.com/read/28709698/revisiting-the-sensitivity-of-sodium-channel-blocker-testing-in-brugada-syndrome-using-obligate-transmittance
#2
EDITORIAL
Rafik Tadros, Arthur A M Wilde
No abstract text is available yet for this article.
July 5, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28705229/brugada-syndrome-in-a-patient-with-amyotrophic-lateral-sclerosis-a-case-report
#3
Anusha Battineni, Rohit Gummi, Naresh Mullaguri, Raghav Govindarajan
BACKGROUND: Amyotrophic lateral sclerosis is a fatal neuromuscular disorder characterized by progressive death of the upper and lower motor neurons in the central nervous system. Patients with this disease die mostly as a result of respiratory failure; however, owing to prolonged survival through assisted ventilation, cardiovascular causes are increasingly responsible for mortality. We report what is to the best of our knowledge the first case of type 2 Brugada syndrome causing ventricular tachyarrhythmia and cardiac arrest in a patient with upper limb onset amyotrophic lateral sclerosis...
July 14, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28703223/unmasking-the-molecular-link-between-arrhythmogenic-cardiomyopathy-and-brugada-syndrome
#4
REVIEW
Javier Moncayo-Arlandi, Ramon Brugada
The intercalated discs that connect cardiomyocytes control cell-to-cell adhesion and communication. Several macromolecular structures (desmosomes, fascia adherens junctions, gap junctions, and sodium-channel complexes) coexist in, and confer their mechanical and electrical properties to, the intercalated disc. Traditionally, each structure was assumed to have a unique function in the intercalated disc. However, growing evidence suggests that these complexes act together in intercellular communication and adhesion, forming a single structural and functional entity - the connexome...
July 13, 2017: Nature Reviews. Cardiology
https://www.readbyqxmd.com/read/28698050/-brugada-pattern-unmasked-by-propafenone-in-an-84-years-old-man
#5
Enrique Asensio Lafuente
No abstract text is available yet for this article.
July 8, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/28691889/osborn-wave-mimicking-brugada-sign
#6
János Tomcsányi, Hrisula Arabadzisz, Kristóf Tomcsányi
No abstract text is available yet for this article.
July 10, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28690312/wolff-parkinson-white-syndrome-with-ventricular-hypertrophy-in-a-brazilian-family
#7
Lenises de Paula van der Steld, Oscar Campuzano, Alexandra Pérez-Serra, Mabel Moura de Barros Zamorano, Selma Sousa Matos, Ramon Brugada
BACKGROUND PRKAG2 syndrome diagnosis is already well-defined as Wolff-Parkinson-White syndrome (WPW), ventricular hypertrophy (VH) due to glycogen accumulation, and conduction system disease (CSD). Because of its rarity, there is a lack of literature focused on the treatment. The present study aimed to describe appropriate strategies for the treatment of affected family members with PRKAG2 syndrome with a long follow-up period. CASE REPORT We studied 60 selected individuals from 84 family members (32 males, 53...
July 10, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28685701/the-role-of-quinidine-in-the-pharmacological-therapy-of-ventricular-arrhythmias-quinidine
#8
Milica Bajcetic, Teodora Vidonja Uzelac, Aleksandra Kezic, Milica Bajcetic
BACKGROUND: Historically, quinidine was the first medicine used in the therapy of heart arrhythmias. Studies in the early 20th century identified quinidine, a diastereomer of the antimalarial quinine, as the most potent of the antiarrhythmic substances extracted from the cinchona plant. Quinidine is used by the 1920s, as an antiarrhythmic agent to maintain sinus rhythm after conversion from atrial flutter or atrial fibrillation and to prevent recurrence of ventricular tachycardia or ventricular fibrillation...
July 7, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28685636/comparisons-of-clinical-impacts-on-individuals-with-brugada-electrocardiographic-patterns-defined-by-ishne-criteria-or-ehra-hrs-aphrs-criteria-a-nationwide-community-based-study
#9
Ching-Yu Julius Chen, Jyh-Ming Jimmy Juang, Ying-Hsiang Chen, I-Chien Wu, Chih-Cheng Hsu, Ray-Chin Wu, Kwo-Ching Chen, Wen-Jin Liaw, Tsung-Lung Tsai, Lian-Yu Lin, Juey-Jen Hwang, Li-Ting Ho, Chih-Chien Yu, Jen-Kuang Lee, Cho-Kai Wu, Shih-Fan Sherri Yeh, Dun-Hui Yang, I-Shou Chang, Ling-Ping Lai, Fu-Tien Chiang, Jiunn-Lee Lin, Chao Agnes Hsiung
INTRODUCTION: Identifying Brugada electrocardiographic pattern (BrP) early is crucial to prevent sudden cardiac death. Two different diagnostic criteria proposed by International Society for Holter and Noninvasive Electrocardiography (ISHNE) and Heart Rhythm Society/European Heart Rhythm Association/Asia-Pacific Heart Rhythm Society (HRS/EHRA/APHRS) were widely used in clinical practice. The difference in prevalence and prognosis of BrP by applying the two different criteria was never studied before...
July 18, 2017: Annals of Medicine
https://www.readbyqxmd.com/read/28685200/role-of-the-burden-of-premature-atrial-contractions-during-the-blanking-period-following-second-generation-cryoballoon-ablation-in-predicting-late-recurrences-of-atrial-arrhythmias
#10
Hugo-Enrique Coutiño, Juan-Pablo Abugattas, Moisés Levinstein, Giacomo Mugnai, Darragh Moran, Valentina De Regibus, Erwin Ströker, Ken Takarada, Rajin Choudhury, Luis Marroquín, Francesca Salghetti, Ian-Emiray Lusoc, Saverio Iacopino, Juan Sieira, Carlo de Asmundis, Pedro Brugada, Gian-Battista Chierchia
PURPOSE: Recent data suggests that high burden of premature atrial complexes after pulmonary vein isolation predicts recurrences of atrial arrhythmias. The present study sought to assess the role of premature atrial complexes burden in predicting atrial arrhythmias recurrences in patients with atrial fibrillation (AF) who have undergone second-generation cryoballoon ablation (CB-Adv). METHODS: Consecutive patients with drug-resistant paroxysmal atrial fibrillation who underwent pulmonary vein isolation using CB-Adv technology as the index procedure were retrospectively included...
July 6, 2017: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/28666944/sodium-channel-blocker-challenge-in-the-familial-screening-of-brugada-syndrome-safety-and-predictors-of-positivity
#11
Dylan Therasse, Frederic Sacher, Bertrand Petit, Dominique Babuty, Philippe Mabo, Raphael Martins, Laurence Jesel, Philippe Maury, Jean Luc Pasquie, Jacques Mansourati, Jean Marc Dupuis, Florence Kyndt, Aurélie Thollet, Beatrice Guyomarch, Julien Barc, Jean Jacques Schott, Herve Le Marec, Richard Redon, Vincent Probst, Jean-Baptiste Gourraud
BACKGROUND: Sodium-channel blocker challenge (SCBC) is frequently performed to unmask Brugada syndrome. OBJECTIVE: We aim to identify predictors of positivity and complications of SCBC in the setting of familial screening. METHODS: All consecutive patients from 2000 to 2014 who benefit from a sodium-channel blocker and belong to a family with at least 2 subjects affected by the syndrome were enrolled and followed prospectively. Data were reviewed by 2 physicians blinded to the clinical and genetic status...
June 27, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28666943/risk-of-arrhythmic-events-in-drug-induced-brugada-syndrome
#12
EDITORIAL
Najim Lahrouchi, Mario Talajic, Rafik Tadros
No abstract text is available yet for this article.
June 27, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28663046/long-term-vagal-stimulation-for-heart-failure-eighteen-month-results-from-the-neural-cardiac-therapy-for-heart-failure-nectar-hf-trial
#13
Gaetano M De Ferrari, Craig Stolen, Anton E Tuinenburg, D Jay Wright, Josep Brugada, Christian Butter, Helmut Klein, Petr Neuzil, Cornelis Botman, Maria Angeles Castel, Antonio D'Onofrio, Gert J de Borst, Scott Solomon, Kenneth M Stein, Bernd Schubert, Kevin Stalsberg, Nicholas Wold, Stephen Ruble, Faiez Zannad
BACKGROUND: The NECTAR-HF study evaluated safety and feasibility of vagal nerve stimulation (VNS) for the treatment of heart failure patients. The first six-month randomized phase of the study did not show improvement in left ventricular remodelling in response to VNS. This study reports the 18-month results and provides novel findings aiming to understand the lack of efficacy of VNS, including a new technique assessing the effects of VNS. METHODS: Ninety-six patients were randomized 2:1 to active or inactive VNS for 6months, thereafter VNS was activated for all patients...
June 10, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28660543/therapeutic-strategies-targeting-inherited-cardiomyopathies
#14
REVIEW
Kenneth Varian, W H Wilson Tang
PURPOSE OF REVIEW: Cardiomyopathies due to genetic mutations are a heterogeneous group of disorders that comprise diseases of contractility, myocardial relaxation, and arrhythmias. Our goal here is to discuss a limited list of genetically inherited cardiomyopathies and the specific therapeutic strategies used to treat them. RECENT FINDINGS: Research into the molecular pathophysiology of the development of these cardiomyopathies is leading to the development of novel treatment approaches...
June 28, 2017: Current Heart Failure Reports
https://www.readbyqxmd.com/read/28657502/brugada-like-coved-type-electrocardiogram-pattern-induced-by-apical-right-ventricular-pacemaker-stimulation
#15
Natale Daniele Brunetti, Francesco Santoro, Pier Luigi Pellegrino, Girolamo D'Arienzo, Matteo Di Biase
No abstract text is available yet for this article.
June 28, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28652678/pectus-excavatum-right-ventricular-compromise-with-orthostatic-syndrome-and-brugada-phenocopy
#16
Eftychios Siniorakis, Spyridon Arvanitakis, Panagiotis Tzevelekos, Stamatia Panta, Apostolos Balanis, Fotini Aivalioti, Sotiria Limberi
Pectus excavatum (PEXT) consists of an overgrowth of the chondral region with posterior displacement of the inferior part of the sternum, resulting in a concave chest deformity. Characteristic clinical and imaging findings may occur, depending on the compression that right cardiac chambers suffer, when squeezed between the sternum and the column vertebrae.
July 2017: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/28645806/brugada-syndrome-a-general-cardiologist-s-perspective
#17
REVIEW
Marija M Polovina, Milica Vukicevic, Bojan Banko, Gregory Y H Lip, Tatjana S Potpara
Brugada syndrome (BrS) is one of the commonest inherited primary arrhythmia syndromes typically presenting with arrhythmic syncope or sudden cardiac death (SCD) due to polymorphic ventricular tachycardia and ventricular fibrillation precipitated by vagotonia or fever in apparently healthy adults, less frequently in children. The prevalence of the syndrome (0.01%-0.3%) varies among regions and ethnicities, being the highest in Southeast Asia. BrS is diagnosed by the "coved type" ST-segment elevation≥2mm followed by a negative T-wave in ≥1 of the right precordial leads V1-V2...
June 20, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/28637782/the-brugada-syndrome-susceptibility-gene-hey2-modulates-cardiac-transmural-ion-channel-patterning-and-electrical-heterogeneity
#18
Christiaan C Veerman, Svitlana Podliesna, Rafik Tadros, Elisabeth M Lodder, Isabella Mengarelli, Berend de Jonge, Leander Beekman, Julien Barc, Ronald Wilders, Arthur A Wilde, Bastiaan J Boukens, Ruben Coronel, Arie Verkerk, Carol Ann Remme, Connie R Bezzina
Rationale: Genome-wide association studies previously identified an association of rs9388451 at chromosome 6q22.3 (near HEY2) with Brugada syndrome (BrS). The causal gene and underlying mechanism remain unresolved. Objective: We used an integrative approach entailing transcriptomic studies in human hearts and electrophysiological studies in Hey2 heterozygous knockout mice (Hey2(+/-) ) to dissect the underpinnings of the 6q22.31 association with BrS. Methods and Results: We queried expression quantitative trait locus (eQTL) data acquired in 190 human left ventricular (LV) samples from the Genotype-Tissue Expression (GTEx) consortium for cis-eQTL effects of rs9388451 which revealed an association between BrS risk allele dosage and HEY2 expression (β=+0...
June 21, 2017: Circulation Research
https://www.readbyqxmd.com/read/28625928/second-generation-cryoballoon-ablation-in-the-setting-of-left-common-pulmonary-veins-procedural-findings-and-clinical-outcome
#19
Erwin Ströker, Ken Takarada, Carlo de Asmundis, Juan-Pablo Abugattas, Giacomo Mugnai, Vedran Velagić, Valentina de Regibus, Hugo-Enrique Coutiño, Rajin Choudhury, Saverio Iacopino, Yves De Greef, Kaoru Tanaka, Pedro Brugada, Gian-Battista Chierchia
BACKGROUND: A left common pulmonary vein (LCPV) accounts for the most frequent PV variation. Use of the cryoballoon (CB) for isolating these veins is still debatable. To date, no data are available regarding the feasibility, acute PV isolation, and clinical outcome of the second-generation CB (CB-A) in this setting. OBJECTIVE: The purpose of this study was to investigate the performance of the CB-A in patients with LCPVs. METHODS: In a total cohort of 433 patients having undergone CB-A ablation for drug-refractory atrial fibrillation together with preprocedural computed tomographic scanning, 146 patients presented an LCPV...
June 16, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28625200/the-value-of-performing-invasive-risk-stratification-in-young-patients-with-the-brugada-syndrome
#20
M Cecilia Gonzalez Corcia, Pedro Brugada
No abstract text is available yet for this article.
June 19, 2017: Cardiology in the Young
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