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Brugada syndrome

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https://www.readbyqxmd.com/read/29784545/spontaneously-aborted-sudden-cardiac-death-in-brugada-syndrome
#1
João Português, Lucy Calvo, Filipa Canário-Almeida, Sílvia Ribeiro, Victor Sanfins, António Lourenço
No abstract text is available yet for this article.
May 18, 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29784488/j-wave-syndromes-electrocardiographic-and-clinical-aspects
#2
REVIEW
Silvia G Priori, Carlo Napolitano
Early repolarization, Brugada syndrome, and pathologic J waves have been described for decades, but only recently experimental and clinical data have allowed reconciliation of Brugada and Early Repolarization under the common definition of J-wave syndromes. The concept was derived from studies showing, in both conditions, the presence of transmural dispersion of repolarization, localized conduction abnormalities, and abnormal transition between QRS and ST segment on electrocardiogram. Although several clinical studies have addressed the clinical presentation and epidemiology of J-wave syndromes, relevant knowledge gaps exist...
June 2018: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/29767628/heart-rate-differences-between-symptomatic-and-asymptomatic-brugada-syndrome-patients-at-night
#3
Mireia Calvo, Virginie Le Rolle, Daniel Romero, Nathalie Behar, Pedro Gomis, Philippe Mabo, Alfredo Hernandez
Ventricular arrhythmias in Brugada syndrome (BS) mainly occur at rest, especially during nighttime, suggesting that parasympathetic activity at night may play an important role in the arrhythmogenesis of the disease. This study examined and compared the autonomic function of symptomatic and asymptomatic BS patients overnight.
 Approach: We analyzed different heart rate variability (HRV) and heart rate complexity (HRC) markers in a clinical series including 87 BS patients, where 23 were symptomatic.
 Main results: Statistically significant differences were found in MIRR, SDNN, SDANN, δSDANN and SampEn, suggesting that symptomatic patients may be related to lower heart rate variability and complexity values, as well as to greater circadian fluctuations overnight...
May 16, 2018: Physiological Measurement
https://www.readbyqxmd.com/read/29766267/-syncopes-and-channelopathies
#4
REVIEW
Johanna Müller-Leisse, Christos Zormpas, Thorben König, David Duncker, Christian Veltmann
Syncope can be the first manifestation of cardiac channelopathies, namely Brugada syndrome, long QT syndrome, short QT syndrome and catecholaminergic polymorphic ventricular tachycardia (CPVT). Patients affected by these rare diseases are at increased risk for sudden cardiac death due to ventricular tachyarrhythmias and require specific therapy and follow-up. As syncope is common in the general population, only few cases are caused by an underlying channelopathy. Nevertheless, the diagnosis should be considered in young patients with structurally normal hearts, especially if the history of syncope is typical for an arrhythmogenic cause, in the presence of characteristic echocardiogram (ECG) patterns, and if there is a family history of channelopathies or sudden cardiac death...
May 15, 2018: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/29764897/diagnostic-yield-of-genetic-testing-in-young-athletes-with-t-wave-inversion
#5
Nabeel Sheikh, Michael Papadakis, Mathew Wilson, Aneil Malhotra, Carmen Adamuz, Tessa Homfray, Lorenzo Monserrat, Elijah R Behr, Sanjay Sharma
Background -T-wave inversion (TWI) is common in patients with cardiomyopathy. However, up to 25% of athletes of African/Afro-Caribbean descent (black athletes) and 5% of white athletes also have TWI of unclear clinical significance despite comprehensive clinical evaluation and long-term follow-up. The aim of this study was to determine the diagnostic yield from genetic testing, beyond clinical evaluation, when investigating athletes with TWI. Methods -We investigated 50 consecutive asymptomatic black and 50 white athletes aged 14-35-years-old with TWI and a normal echocardiogram who were referred to a UK tertiary center for cardiomyopathy and sports cardiology...
May 15, 2018: Circulation
https://www.readbyqxmd.com/read/29763454/multivariate-classification-of-brugada-syndrome-patients-based-on-autonomic-response-to-exercise-testing
#6
Mireia Calvo, Daniel Romero, Virginie Le Rolle, Nathalie Béhar, Pedro Gomis, Philippe Mabo, Alfredo I Hernández
Ventricular arrhythmias in Brugada syndrome (BS) typically occur at rest and especially during sleep, suggesting that changes in the autonomic modulation may play an important role in arrhythmogenesis. The autonomic response to exercise and subsequent recovery was evaluated on 105 patients diagnosed with BS (twenty-four were symptomatic), by means of a time-frequency heart rate variability (HRV) analysis, so as to propose a novel predictive model capable of distinguishing symptomatic and asymptomatic BS populations...
2018: PloS One
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
#7
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/29759672/read-my-lips-a-positive-ajmaline-test-does-not-always-mean-you-have-brugada-syndrome
#8
EDITORIAL
Sami Viskin, Raphael Rosso
No abstract text is available yet for this article.
December 11, 2017: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/29759671/yield-and-pitfalls-of-ajmaline-testing-in-the-evaluation-of-unexplained-cardiac-arrest-and-sudden-unexplained-death-single-center-experience-with-482-families
#9
Rafik Tadros, Eline A Nannenberg, Krystien V Lieve, Doris Škorić-Milosavljević, Najim Lahrouchi, Ronald H Lekanne Deprez, Jeroen Vendrik, Yolan J Reckman, Pieter G Postema, Ahmad S Amin, Connie R Bezzina, Arthur A M Wilde, Hanno L Tan
OBJECTIVES: This study evaluated the yield of ajmaline testing and assessed the occurrence of confounding responses in a large cohort of families with unexplained cardiac arrest (UCA) or sudden unexplained death (SUD). BACKGROUND: Ajmaline testing to diagnose Brugada syndrome (BrS) is routinely used in the evaluation of SUD and UCA, but its yield, limitations, and appropriate dosing have not been studied in a large cohort. METHODS: We assessed ajmaline test response and genetic testing results in 637 individuals from 482 families who underwent ajmaline testing for SUD or UCA...
December 11, 2017: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/29759632/catheter-ablation-of-brugada-syndrome-importance-of-repeated-administration-of-ajmaline-to-unmask-the-entire-epicardial-substrate
#10
Konstantin Krieger, Johannes Steinfurt, Corinna Lenz, Boris Keweloh
No abstract text is available yet for this article.
November 2017: JACC. Clinical Electrophysiology
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
#11
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/29757020/recent-advances-in-the-treatment-of-brugada-syndrome
#12
Mariana Argenziano, Charles Antzelevitch
Brugada syndrome (BrS) is an inherited cardiac arrhythmia syndrome characterized by ST-segment elevation in right precordial ECG leads and associated with sudden cardiac death in young adults. The ECG manifestations of BrS are often concealed but can be unmasked by sodium channel blockers and fever. Areas covered: Implantation of a cardioverter defibrillator (ICD) is first-line therapy for BrS patients presenting with prior cardiac arrest or documented VT. A pharmacological approach to therapy is recommended in cases of electrical storm, as an adjunct to ICD and as preventative therapy...
May 14, 2018: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/29748179/type-i-paradox-of-brugada-syndrome
#13
EDITORIAL
Sami Viskin, Aviram Hochstadt, Raphael Rosso
No abstract text is available yet for this article.
May 10, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29748178/prognostic-significance-of-the-sodium-channel-blocker-test-in-patients-with-brugada-syndrome
#14
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/29744527/inherited-primary-arrhythmia-disorders-cardiac-channelopathies-and-sports-activity
#15
REVIEW
S Marrakchi, I Kammoun, E Bennour, L Laroussi, M Ben Miled, S Kachboura
Sudden cardiac death (SCD) in an apparently healthy individual is a tragedy. It is important to identify the cause of death and to prevent SCD in potentially at-risk family members. Inherited primary arrhythmia disorders are associated with exercise-related SCD. Despite the well-known benefits of exercise, exercise restriction has been a historical mainstay of therapy for these conditions. However, since familiarity with inherited arrhythmia conditions has increased and patients are often children and young adults, it is necessary to reassess the treatment guidelines regarding exercise constraints...
May 9, 2018: Herz
https://www.readbyqxmd.com/read/29740331/voltage-gated-sodium-channel-%C3%AE-1-%C3%AE-1b-subunits-regulate-cardiac-physiology-and-pathophysiology
#16
REVIEW
Nnamdi Edokobi, Lori L Isom
Cardiac myocyte contraction is initiated by a set of intricately orchestrated electrical impulses, collectively known as action potentials (APs). Voltage-gated sodium channels (NaV s) are responsible for the upstroke and propagation of APs in excitable cells, including cardiomyocytes. NaV s consist of a single, pore-forming α subunit and two different β subunits. The β subunits are multifunctional cell adhesion molecules and channel modulators that have cell type and subcellular domain specific functional effects...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29728395/-scn5a-na-v-1-5-variant-functional-perturbation-and-clinical-presentation-variants-of-a-certain-significance
#17
Brett M Kroncke, Andrew M Glazer, Derek K Smith, Jeffrey D Blume, Dan M Roden
BACKGROUND: Accurately predicting the impact of rare nonsynonymous variants on disease risk is an important goal in precision medicine. Variants in the cardiac sodium channel SCN5A (protein NaV 1.5; voltage-dependent cardiac Na+ channel) are associated with multiple arrhythmia disorders, including Brugada syndrome and long QT syndrome. Rare SCN5A variants also occur in ≈1% of unaffected individuals. We hypothesized that in vitro electrophysiological functional parameters explain a statistically significant portion of the variability in disease penetrance...
May 2018: Circulation. Genomic and precision medicine
https://www.readbyqxmd.com/read/29716710/catheter-ablation-for-electrical-storm-in-brugada-syndrome-results-of-substrate-based-ablation
#18
Abhijeet Shelke, Ajit Tachil, Daljeet Saggu, Masilamani Lawrance Jesuraj, Sachin Yalagudri, Calambur Narasimhan
BACKGROUND: Brugada syndrome (BrS) is known to cause malignant ventricular arrhythmia (VA) and sudden cardiac death (SCD). Patients with implantable cardioverter defibrillator (ICD) may experience recurrent shocks from ICD. Recent reports indicate that radiofrequency ablation (RFA) in BrS is feasible, and effective. Catheter ablation of premature ventricular complexes (PVCs) triggering VA and substrate modification of right ventricular outflow tract (RVOT) has been described. METHODS AND RESULTS: Five patients (4 males, age-23 to 32 years) with BrS and electrical storm (ES) despite being on isoprenaline infusion and cilostazol (phosphodiestrase-3 inhibitor) underwent 3 dimensional electroanatomic mapping and RFA...
March 2018: Indian Heart Journal
https://www.readbyqxmd.com/read/29709244/scn5a-mutation-type-and-topology-are-associated-with-the-risk-of-ventricular-arrhythmia-by-sodium-channel-blockers
#19
Ahmad S Amin, Yolan J Reckman, Elena Arbelo, Anne M Spanjaart, Pieter G Postema, Rafik Tadros, Michael W Tanck, Maarten P Van den Berg, Arthur A M Wilde, Hanno L Tan
BACKGROUND: Ventricular fibrillation in patients with Brugada syndrome (BrS) is often initiated by premature ventricular contractions (PVCs). Presence of SCN5A mutation increases the risk of PVCs upon exposure to sodium channel blockers (SCB) in patients with baseline type-1 ECG. In patients without baseline type-1 ECG, however, the effect of SCN5A mutation on the risk of SCB-induced arrhythmia is unknown. We aimed to establish whether presence/absence, type, and topology of SCN5A mutation correlates with PVC occurrence during ajmaline infusion...
April 27, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29709101/genotype-phenotype-relationship-and-risk-stratification-in-loss-of-function-scn5a-mutation-carriers
#20
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
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