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Syndrom Brugada

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https://www.readbyqxmd.com/read/28709698/revisiting-the-sensitivity-of-sodium-channel-blocker-testing-in-brugada-syndrome-using-obligate-transmittance
#1
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
#2
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
#3
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/28690312/wolff-parkinson-white-syndrome-with-ventricular-hypertrophy-in-a-brazilian-family
#4
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
#5
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/28666944/sodium-channel-blocker-challenge-in-the-familial-screening-of-brugada-syndrome-safety-and-predictors-of-positivity
#6
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
#7
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/28660543/therapeutic-strategies-targeting-inherited-cardiomyopathies
#8
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/28652678/pectus-excavatum-right-ventricular-compromise-with-orthostatic-syndrome-and-brugada-phenocopy
#9
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
#10
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
#11
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/28625200/the-value-of-performing-invasive-risk-stratification-in-young-patients-with-the-brugada-syndrome
#12
M Cecilia Gonzalez Corcia, Pedro Brugada
No abstract text is available yet for this article.
June 19, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28625016/brugada-syndrome-and-exercise-practice-current-knowledge-shortcomings-and-open-questions
#13
Giuseppe Mascia, Elena Arbelo, Jaime Hernández Ojeda, Francesco Solimene, Ramon Brugada, Josep Brugada
Since its recognition as a clinical entity in 1992, the Brugada Syndrome (BrS), a hereditary disease characterized by a typical electrocardiogram (ECG) pattern potentially predisposing to sudden cardiac death (SCD), has attracted the attention of many physicians for its circadian pattern of ventricular arrhythmias (VA), mostly occurring at rest. Exercise may potentially worsen the ECG abnormalities in BrS patients, resulting in higher peak J-point amplitudes during the vasovagal reaction of the recovery period, possibly leading to an increased risk of cardiac events...
July 2017: International Journal of Sports Medicine
https://www.readbyqxmd.com/read/28616803/amitriptyline-may-have-possibility-to-induce-brugada-syndrome-rather-than-long-qt-syndrome
#14
Nur Jaharat Lubna, Takeshi Wada, Yuji Nakamura, Koki Chiba, Xin Cao, Hiroko Izumi-Nakaseko, Kentaro Ando, Atsuhiko T Naito, Yoshioki Satoh, Atsushi Sugiyama
Amitriptyline has been reported to induce long QT syndrome in addition to Brugada syndrome. We qualitatively and quantitatively analyzed the potential of amitriptyline to induce these lethal syndromes by using the halothane-anesthetized dogs (n = 6). Amitriptyline was intravenously administered in doses of 0.1, 1 and 10 mg/kg over 10 min every 20 min, which would provide approximately 1, 10 and 100 times higher plasma concentrations than a therapeutic one, respectively. The low dose hardly altered any of the cardiovascular variables...
June 14, 2017: Cardiovascular Toxicology
https://www.readbyqxmd.com/read/28608483/brugada-syndrome-brugada-phenocopy-or-none
#15
Umut Kocabas, Can Hasdemir, Esra Kaya, Cuneyt Turkoglu, Adrian Baranchuk
Brugada syndrome is a form of inherited arrhythmia syndrome characterized by a distinct ST-segment elevation in the right precordial leads. Brugada phenocopies are clinical entities that present with an electrocardiographic pattern identical to Brugada syndrome and may obey to various clinical conditions. We present a case of a suicidal attempt using a high dose of propafenone causing a Brugada-type electrocardiographic pattern. Is this a Brugada syndrome case, a Brugada phenocopy or something else?
June 13, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28606694/syncope-in-brugada-syndrome-a-plea-for-thorough-anamnesis-and-eps-the-lessons-from-our-journey-in-the-region-of-piedmont-italy
#16
EDITORIAL
Bernard Belhassen, Anat Milman
No abstract text is available yet for this article.
August 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28597987/trafficking-and-localization-to-the-plasma-membrane-of-nav-1-5-promoted-by-the-%C3%AE-2-subunit-is-defective-due-to-a-%C3%AE-2-mutation-associated-with-brugada-syndrome
#17
Gemma Dulsat, Sonia Palomeras, Eric Cortada, Helena Riuró, Ramon Brugada, Marcel Vergés
BACKGROUND INFORMATION: Cardiac channelopathies arise by mutations in genes encoding ion channel subunits. One example is Brugada Syndrome (BrS), which causes arrhythmias and sudden death. BrS is often associated with mutations in SCN5A, encoding Nav 1.5, the α subunit of the major cardiac voltage-gated sodium channel. This channel forms a protein complex including one or two associated β subunits as well as other proteins. RESULTS: We analyzed regulation of Nav 1...
June 9, 2017: Biology of the Cell
https://www.readbyqxmd.com/read/28584071/scn5a-genetic-polymorphisms-associated-with-increased-defibrillator-shocks-in-brugada-syndrome
#18
Pattarapong Makarawate, Narumol Chaosuwannakit, Suda Vannaprasaht, Dujdao Sahasthas, Seok Hwee Koo, Edmund Jon Deoon Lee, Wichittra Tassaneeyakul, Hector Barajas-Martinez, Dan Hu, Kittisak Sawanyawisuth
BACKGROUND: Brugada syndrome (BrS) is an inherited primary arrhythmia disorder leading to sudden cardiac arrest. SCN5A, encoding the α-subunit of the cardiac sodium channel (Nav1.5), is the most common pathogenic gene of BrS. An implantable cardioverter defibrillator (ICD) is the standard treatment for secondary prevention. This study aimed to evaluate association of the SCN5A variant with this cardiac conduction disturbance and appropriate ICD shock therapy in Thai symptomatic BrS patients with ICD implants...
June 5, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28583685/usefulness-of-the-r-wave-sign-as-a-predictor-for-ventricular-tachyarrhythmia-in-patients-with-brugada-syndrome
#19
Ahmed A Y Ragab, Charlotte A Houck, Lisette J M E van der Does, Eva A H Lanters, Danielle E Burghouwt, Agnes J Q M Muskens, Natasja M S de Groot
Brugada syndrome (BrS) is an autosomal dominant channelopathy which is responsible for a large number of sudden cardiac deaths in young subjects without structural abnormalities. The most challenging step in management of patients with BrS is identifying who is at risk for developing malignant ventricular tachyarrhythmia (VTA). In patients with BrS, conduction delay in the right ventricular outflow tract (RVOT) causes a prominent R wave in lead aVR. This electrocardiographic parameter can be useful to detect these high-risk patients...
August 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28580152/brugada-phenocopy-in-a-patient-with-acute-pulmonary-embolism-presenting-with-recurrent-syncope
#20
Nixiao Zhang, Tong Liu, Gary Tse, Shuyu Yu, Huaying Fu, Gang Xu, Changyu Zhou, Chengzong Zhang, Guangping Li
Brugada phenocopy (BrP) refers to a group of clinical conditions that have etiologies distinct from Brugada syndrome (BrS). Although both demonstrate features of ST-segment elevation in the right precordial leads on the electrocardiogram (ECG), one must be distinguished from the other as their treatment options are different. We report a male patient who presented with recurrent syncope with a Brugada and a S1Q3T3 pattern on the ECG. Acute pulmonary embolism (APE) complicated by BrS was suspected. Twenty-four hours Holter monitoring did not demonstrate any evidence of ventricular arrhythmias...
May 2017: Oxford Medical Case Reports
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