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Brugada syndrome

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https://www.readbyqxmd.com/read/29351608/feasibility-of-drugs-in-brugada-syndrome-authors-reply
#1
Tsukasa Kamakura, Mitsuru Wada, Kohei Ishibashi, Yuko Y Inoue, Koji Miyamoto, Hideo Okamura, Satoshi Nagase, Takashi Noda, Takeshi Aiba, Satoshi Yasuda, Wataru Shimizu, Shiro Kamakura, Kengo Kusano
No abstract text is available yet for this article.
January 16, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29332241/atrial-fibrillation-is-associated-with-sudden-cardiac-death-a-systematic-review-and-meta-analysis
#2
Pattara Rattanawong, Sikarin Upala, Tanawan Riangwiwat, Veeravich Jaruvongvanich, Anawin Sanguankeo, Wasawat Vutthikraivit, Eugene H Chung
PURPOSE: Recent studies suggest that atrial fibrillation (AF) is associated with increased cardiovascular risk and mortality including sudden cardiac death (SCD). According to the Cardiovascular Heath Study cohort, the incident rate of SCD was higher in the AF population (2.9 per 1000 per year) compared with non-AF controls (1.3 per 1000 per year). In this study, we performed a systematic review and meta-analysis to explore the association between AF and SCD. METHODS: We comprehensively searched the databases of MEDLINE and EMBASE from inception to January 2017...
January 13, 2018: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/29326874/only-some-patients-with-bulbar-and-spinal-muscular-atrophy-may-develop-cardiac-disease
#3
Josef Finsterer, Claudia Stöllberger
Objectives: According to recent publications, some patients with spinal and bulbar muscular atrophy (BSMA) develop cardiac disease, manifesting as ST-segment abnormalities, Brugada-syndrome, dilative cardiomyopathy, or sudden cardiac death. Here we present neurological and cardiac data of a BSMA patient who was followed up for 10 y. Case report: In a male patient aged 47 y, BSMA was diagnosed at age 37 y upon the typical clinical presentation (postural tremor since age 12 y, dysarthria since age 15 y, muscle cramps since age 29 y, general myalgias since age 32 y, general fasciculations since age 34 y, myoclonic jerks, easy fatigability, dyspnea upon exercise since age 36 y) and a CAG-repeat expansion of 47 ± 1 repeats in the androgen-receptor gene detected at age 37 y...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29325976/profile-of-brugada-syndrome-patients-presenting-with-their-first-documented-arrhythmic-event-data-from-the-survey-on-arrhythmic-events-in-brugada-syndrome-sabrus
#4
Anat Milman, Antoine Andorin, Jean-Baptiste Gourraud, Pieter G Postema, Frederic Sacher, Philippe Mabo, Sung-Hwan Kim, Jimmy Jm Juang, Shingo Maeda, Yoshihide Takahashi, Tsukasa Kamakura, Takeshi Aiba, Giulio Conte, Georgia Sarquella-Brugada, Eran Leshem, Michael Rahkovich, Aviram Hochstadt, Yuka Mizusawa, Elena Arbelo, Zhengrong Huang, Isabelle Denjoy, Carla Giustetto, Yanushi D Wijeyeratne, Carlo Napolitano, Yoav Michowitz, Ramon Brugada, Ruben Casado-Arroyo, Jean Champagne, Leonardo Calo, Jacob Tfelt-Hansen, Silvia G Priori, Masahiko Takagi, Christian Veltmann, Pietro Delise, Domenico Corrado, Elijah R Behr, Fiorenzo Gaita, Gan-Xin Yan, Josep Brugada, Antoine Leenhardt, Arthur A M Wilde, Pedro Brugada, Kengo F Kusano, Kenzo Hirao, Gi-Byoung Nam, Vincent Probst, Bernard Belhassen
BACKGROUND: Detailed information on the profile of Brugada syndrome (BrS) patients presenting their first arrhythmic event (AE) after prophylactic implantation of a cardioverter defibrillator (ICD) is limited. OBJECTIVES: 1) To compare clinical, electrocardiographic, electrophysiologic and genetic profiles of patients who exhibited their first documented AE as aborted cardiac arrest (CA) (group A) with those in whom the AE was documented after prophylactic ICD implantation (group B); 2) To characterize group B patients' profile using the Class II indications for ICD implantation established by HRS/EHRA/APHRS Expert Consensus Statement in 2013...
January 8, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29325639/pediatric-brugada-syndrome-avoiding-the-inappropriate-icd
#5
EDITORIAL
Peter F Aziz
No abstract text is available yet for this article.
January 16, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29325638/implantable-cardioverter-defibrillators-in%C3%A2-children-and-adolescents-with-brugada%C3%A2-syndrome
#6
M Cecilia Gonzalez Corcia, Juan Sieira, Gudrun Pappaert, Carlo de Asmundis, Gian Battista Chierchia, Mark La Meir, Andrea Sarkozy, Pedro Brugada
BACKGROUND: Young patients presenting with symptomatic Brugada syndrome have very high risks for ventricular arrhythmias and should be carefully considered for implantable cardioverter-defibrillator (ICD) placement. However, this therapy is associated with high rates of inappropriate shocks and device-related complications. OBJECTIVES: This study investigated clinical features, management, and long-term follow-up of young patients with Brugada syndrome and ICD. METHODS: Patients diagnosed with Brugada syndrome, who underwent implantation of an ICD at an age of ≤20 years, were studied...
January 16, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29309564/role-of-syncope-in-predicting-adverse-outcomes-in-patients-with-suspected-brugada-syndrome-undergoing-standardized-flecainide-testing
#7
Juan Pablo Flórez, Daniel García, Irene Valverde, José Rubín, Diego Pérez, Mar González-Vasserot, Julián Reguero, Jesús María de la Hera, Pablo Avanzas, Juan Gómez, Eliecer Coto, César Morís, David Calvo
Aims: Sensitivity to flecainide testing results in suboptimal findings in patients with Brugada syndrome (BrS), leading to safety concerns. Because cardiac syncope effectively predicts outcomes in BrS, we aimed to explore its predictive value in a large cohort of negative and positive responders (NR and PR) to standard flecainide testing. Methods and results : We analysed the data of 251 consecutive patients, 177 NR vs. 74 PR, to flecainide testing, performed according to standard recommendations...
December 22, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29306897/critical-roles-of-xirp-proteins-in-cardiac-conduction-and-their-rare-variants-identified-in-sudden-unexplained-nocturnal-death-syndrome-and-brugada-syndrome-in-chinese-han-population
#8
Lei Huang, Kuo-Ho Wu, Liyong Zhang, Qinchuan Wang, Shuangbo Tang, Qiuping Wu, Pei-Hsiu Jiang, Jim Jung-Ching Lin, Jian Guo, Lin Wang, Shih-Hurng Loh, Jianding Cheng
BACKGROUND: Sudden unexplained nocturnal death syndrome (SUNDS) remains an autopsy negative entity with unclear etiology. Arrhythmia has been implicated in SUNDS. Mutations/deficiencies in intercalated disc components have been shown to cause arrhythmias. Human cardiomyopathy-associated 1 (XIRP1) and 3 (XIRP2) are intercalated disc-associated, Xin repeats-containing proteins. Mouse Xirp1 is necessary for the integrity of intercalated disc and for the surface expression of transient outward and delayed rectifier K+ channels, whereas mouse Xirp2 is required for Xirp1 intercalated disc localization...
January 6, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29306474/concealed-abnormal-atrial-phenotype-in-patients-with-brugada-syndrome-and-no-history-of-atrial-fibrillation
#9
Giulio Conte, Maria Luce Caputo, Paul G A Volders, Adrian Luca, Luca Mainardi, Ulrich Schotten, Valentina D A Corino, François Regoli, Stef Zeemering, Matthias Zink, Sasan Yazdani, Lukas Kappenberger, Tiziano Moccetti, Jean-Marc Vesin, Angelo Auricchio
OBJECTIVES: The electrocardiogram (ECG) of patients with BrS in sinus rhythm might reflect intrinsic atrial electrical abnormalities independent from any previous atrial fibrillation (AF). Aim of this study is to investigate the presence of P-wave abnormalities in patients with BrS and no history of AF, and to compare them with those displayed by patients with documented paroxysmal AF and by healthy subjects. METHODS: Continuous 5-min 16-lead ECG recordings in sinus rhythm were obtained from 72 participants: 32 patients with a type 1 Brugada ECG, 20 patients with a history of paroxysmal AF and 20 age-matched healthy subjects...
February 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29302124/extracorporeal-life-support-as-a-rescue-measure-for-managing-life-threatening-arrythmia-and-brugada-syndrome
#10
Asaad G Beshish, Allison Weinberg, Waseem Ostwani, Gabe E Owens
We describe the use of extracorporeal cardiopulmonary resuscitation (E-CPR) to transiently stabilize a 3-month-old patient who presented with ventricular tachyarrhythmias leading to spontaneous cardiac arrest. The patient required 4 days of extracorporeal life support (ECLS) where he was diagnosed with probable Brugada syndrome (BS). The patient was discharged home in stable condition after implantable cardioverter defibrillator placement. This case highlights the importance of early transfer to extracorporeal membrane oxygenation (ECMO) center in the setting of unexplained cardiac arrhythmia in a pediatric patient...
December 2017: Journal of Extra-corporeal Technology
https://www.readbyqxmd.com/read/29302023/a-case-of-type-i-and-ii-brugada-phenocopy-unmasked-in-a-patient-with-normal-baseline-electrocardiogram-ecg
#11
Marie H Bernardo, Satish R Tiyyagura
BACKGROUND Brugada pattern on electrocardiogram (ECG) is seen when there are at least 2 mm J-point elevation and 1 mm ST-segment elevation in two or more of the right precordial leads, with right bundle-branch block (RBBB)-like morphology. Elevation of a coved-type shape in leads V1 and V2 is consistent with type I Brugada pattern, whereas elevation of a saddle-back configuration distinguishes type II Brugada. If accompanied by life-threatening arrhythmias or sudden cardiac death, Brugada syndrome (BrS) is diagnosed...
January 5, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29301723/brugada-syndrome-or-not-that-is-the-question
#12
EDITORIAL
Guillaume Bassil, Sami F Noujaim
No abstract text is available yet for this article.
December 21, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29296584/local-activation-delay-exacerbates-local-j-st-elevation-in-the-epicardium-electrophysiological-substrate-in-brugada-syndrome
#13
Naoya Kataoka, Satoshi Nagase, Tsukasa Kamakura, Takashi Noda, Takeshi Aiba, Kengo Kusano
No abstract text is available yet for this article.
December 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/29280897/brugada-syndrome-induced-by-an-interscalene-block-a-case-report
#14
Sally A Corey, Santosh Varkey, Douglas R Phillips, Gregory L Gay
CASE: A 57-year-old woman with no noteworthy medical or surgical history underwent an interscalene block with bupivacaine in preparation for an arthroscopic rotator cuff repair. Following administration of the bupivacaine, the patient sustained a ventricular fibrillation arrest. After successful cardiopulmonary resuscitation, she was diagnosed with Brugada syndrome. An implantable cardioverter-defibrillator was placed, and the rotator cuff repair was performed 1 month later. CONCLUSION: Brugada syndrome is an abnormality of the cardiac conduction system that leads to cardiac arrhythmias...
October 2017: JBJS Case Connector
https://www.readbyqxmd.com/read/29278359/incomplete-penetrance-and-variable-expressivity-hallmarks-in-channelopathies-associated-with-sudden-cardiac-death
#15
REVIEW
Monica Coll, Alexandra Pérez-Serra, Jesus Mates, Bernat Del Olmo, Marta Puigmulé, Anna Fernandez-Falgueras, Anna Iglesias, Ferran Picó, Laura Lopez, Ramon Brugada, Oscar Campuzano
Sudden cardiac death is defined as an unexpected decease of cardiac origin. In individuals under 35 years old, most of these deaths are due to familial arrhythmogenic syndromes of genetic origin, also known as channelopathies. These familial cardiac syndromes commonly follow an autosomal dominant pattern of inheritance. Diagnosis, however, can be difficult, mainly due to incomplete penetrance and variable expressivity, which are hallmarks in these syndromes. The clinical manifestation of these diseases can range from asymptomatic to syncope but sudden death can sometimes be the first symptom of disease...
December 26, 2017: Biology
https://www.readbyqxmd.com/read/29250293/hypothetical-anatomy-of-brugada-phenomenon-long-qt-sine-long-qt-syndrome-implicating-morphologically-undefined-specific-brugada-s-myocells
#16
REVIEW
Petras Stirbys
The Brugada syndrome (BrS) is associated with increased risk of ventricular arrhythmias and sudden cardiac death. It generates genetically mediated arrhythmias posing a true pathophysiological challenge. In search of the similarities between BrS and long QT syndrome some novel insights are suggested. In patients with BrS the duration of QT interval is usually normal. Some investigators have found prolonged QT interval in the syndrome's natural course or the duration of QT segment have been extended by provocative tests unmasking BrS...
April 2017: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/29242054/brugada-phenocopy-induced-by-severe-pneumothorax
#17
Javier C Barcos, Iván A Tello Santacruz, César Cáceres Monié, María L Fernández Recalde, Juan D Humphreys
A Brugada phenocopy has been defined as a clinical situation that presents with an abnormal electrocardiogram identical to any of the electrocardiographic patterns found in Brugada syndrome in the absence of the characteristic congenital genetic abnormalities. The first confirmed case of type 1 Brugada phenocopy associated with severe left pneumothorax is presented. A provocative test with ajmaline, which proved to be negative, was performed to confirm the diagnosis. The presence of ST-segment elevation in the context of pneumothorax is most infrequent...
October 28, 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/29241490/short-qt-syndrome-and-hydroquinidine-rare-diseases-and-unavailable-drugs
#18
EDITORIAL
Pedro Brugada
No abstract text is available yet for this article.
December 19, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29238365/spontaneous-type-1-pattern-ventricular-arrhythmias-and-sudden-cardiac-death-in-brugada-syndrome-an-updated-systematic-review-and-meta-analysis
#19
Ahmed Bayoumy, Meng-Qi Gong, Ka Hou Christien Li, Sunny Hei Wong, William Kk Wu, Guang-Ping Li, George Bazoukis, Konstantinos P Letsas, Wing Tak Wong, Yun-Long Xia, Tong Liu, Gary Tse
No abstract text is available yet for this article.
October 2017: Journal of Geriatric Cardiology: JGC
https://www.readbyqxmd.com/read/29233646/induced-brugada-syndrome-possible-sources-of-arrhythmogenesis
#20
REVIEW
Gonçalo Tomé, João Freitas
Brugada syndrome is an inherited cardiac condition with the potential for development of life-threatening arrhythmias in relatively young individuals without significant structural cardiac abnormalities. The condition is characterized by a distinct coved-type ST segment elevation in the right precordial leads (V1-V3). This hallmark pattern (type 1) is often dynamic and sometimes concealed, and may be unmasked in certain conditions or under the effect of certain agents, which include variation of sympathovagal balance, hormones, metabolic factors and drugs...
December 9, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
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