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Syndrom Brugada

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https://www.readbyqxmd.com/read/28090040/outcomes-of-brugada-syndrome-patients-with-coronary-artery-vasospasm
#1
Shingo Kujime, Harumizu Sakurada, Naoki Saito, Yoshinari Enomoto, Naoshi Ito, Keijiro Nakamura, Seiji Fukamizu, Tamotsu Tejima, Yuzuru Yambe, Mitsuhiro Nishizaki, Mahito Noro, Masayasu Hiraoka, Kaoru Sugi
Objective To evaluate the outcomes of patients with concomitant Brugada syndrome and coronary artery vasospasm. Methods Patients diagnosed with Brugada syndrome with an implantable cardiac defibrillator were retrospectively investigated, and the coexistence of vasospasm was evaluated. The clinical features and outcomes were evaluated, especially in patients with coexistent vasospasm. A provocation test using acetylcholine was performed in patients confirmed to have no organic stenosis on percutaneous coronary angiography to confirm the presence of vasospasm...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28087596/a-meta-analysis-on-the-prognostic-significance-of-inferolateral-early-repolarization-pattern-in-brugada-syndrome
#2
Stamatis Georgopoulos, Konstantinos P Letsas, Tong Liu, Maria Kalafateli, Panagiotis Korantzopoulos, Gerd Bürkle, Konstantinos Vlachos, Georgios Giannopoulos, Michael Efremidis, Spyridon Deftereos, Antonios Sideris, Masahiko Takagi, Gan-Xin Yan, Joachim R Ehrlich
AIMS: The early repolarization (ER) pattern has been linked to an increased risk for arrhythmic death in various clinical settings. There are limited and conflicting data regarding the prognostic significance of ER pattern in Brugada syndrome (BS). The aim of this meta-analysis was to provide a detailed analysis of the currently available studies regarding the arrhythmic risk in patients with BS and ER pattern. METHODS AND RESULTS: Current databases were searched until May 2015...
January 13, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28077816/incidence-of-atrial-tachyarrhythmias-in-patients-with-early-repolarization-syndrome
#3
Ki Won Hwang, Gi-Byoung Nam, Junhee Han, Yong Giun Kim, Hyung Oh Choi, Jun Kim, Kee-Joon Choi, You-Ho Kim
Atrial tachyarrhythmias (ATAs) occur in a significant proportion of Brugada syndrome (BrS) patients and are often an important cause of inappropriate shocks. The aim of this retrospective study was to evaluate the incidence of ATAs and ATA-induced inappropriate shocks in early repolarization syndrome (ERS) patients as compared to BrS patients.We analyzed data from 20 consecutive patients who were diagnosed with ERS and compared them with patients diagnosed with BrS (n = 31). Clinical and ICD interrogation data were collected and analyzed for all events with ICD shocks...
January 11, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28072945/uneventful-prolonged-propofol-infusion-in-a-patient-with-brugada-syndrome
#4
P Flamee, W Dewals, J Poelaert
No abstract text is available yet for this article.
January 2017: Anaesthesia and Intensive Care
https://www.readbyqxmd.com/read/28070060/the-brugada-syndrome%C3%A3-from-gene-to-therapy
#5
Antonio Curcio, Giuseppe Santarpia, Ciro Indolfi
It is almost a quarter of century that a pioneering work of 2 researchers named Brugada brought the entire scientific community to understanding the molecular, clinical, and electrophysiological aspects of a distinctive syndrome. It affects mainly young adults with syncope and/or sudden cardiac death caused by polymorphic ventricular tachycardia or ventricular fibrillation in the absence of any sign of cardiac degeneration or alteration. Although the involvement of the epicardial layer of the right ventricular outflow tract, and the requirement of pharmacologic challenge for unveiling concealed forms, have been fully characterized, many areas of uncertainties remain to be elucidated, such as the unpredictable usefulness of programmed ventricular stimulation, the role of radiofrequency catheter ablation for reducing ST-segment elevation, and the value of risk stratification in patients diagnosed with upper displacement of right precordial leads...
January 7, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28069669/refining-the-molecular-organization-of-the-cardiac-intercalated-disc
#6
REVIEW
Sarah H Vermij, Hugues Abriel, Toon A B van Veen
This review presents an extensively integrated model of the cardiac intercalated disc (ID), a highly orchestrated structure that connects adjacent cardiomyocytes. Classically, three main structures are distinguished: gap junctions (GJs) metabolically and electrically connect cytoplasm of adjacent cardiomyocytes; adherens junctions (AJs) connect the actin cytoskeleton of adjacent cells; and desmosomes function as cell anchors and connect intermediate filaments. Furthermore, ion channels reside in the ID. Mutations in ID proteins have been associated with cardiac arrhythmias such as Brugada syndrome and arrhythmogenic cardiomyopathy...
January 8, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28065832/a-novel-method-to-enhance-phenotype-epicardial-functional-substrates-and-ventricular-tachyarrhythmias-in-brugada-syndrome
#7
Fa-Po Chung, Sunu Budhi Raharjo, Yenn-Jiang Lin, Shih-Lin Chang, Li-Wei Lo, Yu-Feng Hu, Ta-Chuan Tuan, Tze-Fan Chao, Jo-Nan Liao, Chin-Yu Lin, Yao-Ting Chang, Yuan Hung, Abigail Te, Shinya Yamada, Hiroshi Tasaka, Shih-Ann Chen, Chin-Tien Wang
BACKGROUND: Fever is associated with the manifestation of Brugada phenotype and ventricular tachycardia/fibrillation (VT/VF) in patients with Brugada syndrome (BrS). The thermal effect on the pathogenesis of functional substrates in BrS remains unknown. OBJECTIVE: The study aimed to elucidate the thermal effect on BrS phenotype, VT/VF, and electrophysiological characteristics of epicardial functional substrates in BrS. METHODS: We consecutively studied 15 patients with BrS receiving radiofrequency catheter ablation (RFCA) for drug-refractory ventricular tachyarrhythmias...
January 5, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28055993/-fever-and-sudden-death-a-reality-illustrative-case-report
#8
Carlos Rodríguez-Artuza, Juan Osorio, Freddy Madueño, Agustín Payares
The connection between fever and sudden death due to ventricular tachycardia has been usually reported in Brugada Syndrome. However the thermosensitive effects caused by fever have been recently described as a possible cause of sudden death in congenital long QT type II syndrome and in idiopathic ventricular fibrillation syndrome. We present a case where fever unmasked a congenital long QT type II syndrome.
November 21, 2016: Medwave
https://www.readbyqxmd.com/read/28038951/-clues-of-an-underlying-organic-substrate-in-the-brugada-syndrome
#9
Bortolo Martini, Nicolò Martini, Margarita Dorantes Sánchez, Manlio F Márquez, Li Zhang, Guy Fontaine, Andrea Nava
No abstract text is available yet for this article.
December 27, 2016: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/28032242/molecular-pathological-study-on-lrrc10-in-sudden-unexplained-nocturnal-death-syndrome-in-the-chinese-han-population
#10
Lei Huang, Shuangbo Tang, Yili Chen, Liyong Zhang, Kun Yin, Yeda Wu, Jinxiang Zheng, Qiuping Wu, Jonathan C Makielski, Jianding Cheng
Sudden unexplained nocturnal death syndrome (SUNDS) is a perplexing disorder to both forensic pathologists and clinic physicians. Clinical features of SUNDS survivors suggested that SUNDS is similar to Brugada syndrome (BrS). Leucine-rich repeat containing 10 (LRRC10) gene was a newly identified gene linked to dilated cardiomyopathy, a disease associated with sudden cardiac death. To investigate the prevalence and spectrum of genetic variants of LRRC10 gene in SUNDS and BrS, the coding regions of LRRC10 were genetically screened in 113 sporadic SUNDS victims (from January 2005 to December 2015, 30...
December 28, 2016: International Journal of Legal Medicine
https://www.readbyqxmd.com/read/28000227/variants-of-brugada-syndrome-and-early-repolarization-syndrome-an-expanded-concept-of-j-wave-syndrome
#11
Sung-Hwan Kim, Gi-Byoung Nam, Sung-Cheol Yun, Hyung Oh Choi, Kee-Joon Choi, Boyoung Joung, Hui-Nam Pak, Moon-Hyoung Lee, Sung Soon Kim, Seung-Jung Park, Young Keun On, June Soo Kim, Il-Young Oh, Eue-Keun Choi, Seil Oh, Yun-Shik Choi, Jong Il Choi, Sang Weon Park, Young-Hoon Kim, Yong-Seog Oh, Man Young Lee, Hong Euy Lim, Young-Soo Lee, Yongkeun Cho, Jun Kim, Kyoung-Suk Rhee, Dong-Il Lee, Dae Kyoung Cho, You-Ho Kim
BACKGROUND: The role of J-waves in the pathogenesis of ventricular fibrillation (VF) occurring in structurally normal hearts is important. METHODS: We evaluated 127 patients who received an implantable cardioverter-defibrillator (ICD) for Brugada syndrome (BS, n = 53), early repolarization syndrome (ERS, n = 24), and patients with unknown or deferred diagnosis (n = 50). ECG, clinical characteristics, and ICD data were analyzed. RESULTS: J-waves were found in 27/50 patients with VF of unknown/deferred diagnosis...
December 20, 2016: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/27979714/epicardial-substrate-ablation-for-brugada-syndrome
#12
Koonlawee Nademanee, Meleze Hocini, Michel Haïssaguerre
No abstract text is available yet for this article.
December 12, 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27932425/scn2b-deletion-in-mice-results-in-ventricular-and-atrial-arrhythmias
#13
Yangyang Bao, B Cicero Willis, Chad R Frasier, Luis F Lopez-Santiago, Xianming Lin, Roberto Ramos-Mondragón, David S Auerbach, Chunling Chen, Zhenxun Wang, Justus Anumonwo, Héctor H Valdivia, Mario Delmar, José Jalife, Lori L Isom
BACKGROUND: Mutations in SCN2B, encoding voltage-gated sodium channel β2-subunits, are associated with human cardiac arrhythmias, including atrial fibrillation and Brugada syndrome. Because of this, we propose that β2-subunits play critical roles in the establishment or maintenance of normal cardiac electric activity in vivo. METHODS AND RESULTS: To understand the pathophysiological roles of β2 in the heart, we investigated the cardiac phenotype of Scn2b null mice...
December 2016: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/27930354/simultaneous-non-invasive-epicardial-and-endocardial-mapping-in-patients-with-brugada-syndrome-new-insights-into-arrhythmia-mechanisms
#14
Boris Rudic, Maria Chaykovskaya, Alexey Tsyganov, Vitaly Kalinin, Erol Tülümen, Theano Papavassiliu, Christina Dösch, Volker Liebe, Jürgen Kuschyk, Susanne Röger, Ibrahim El-Battrawy, Ibrahim Akin, Marina Yakovleva, Elena Zaklyazminskaya, Anna Shestak, Stanislav Kim, Mikhail Chmelevsky, Martin Borggrefe
BACKGROUND: The underlying mechanisms of Brugada syndrome (BrS) are not completely understood. Recent studies provided evidence that the electrophysiological substrate, leading to electrocardiogram abnormalities and/or ventricular arrhythmias, is located in the right ventricular outflow tract (RVOT). The purpose of this study was to examine abnormalities of epicardial and endocardial local unipolar electrograms by simultaneous noninvasive mapping in patients with BrS. METHODS AND RESULTS: Local epicardial and endocardial unipolar electrograms were analyzed using a novel noninvasive epi- and endocardial electrophysiology system (NEEES) in 12 patients with BrS and 6 with right bundle branch block for comparison...
November 14, 2016: Journal of the American Heart Association
https://www.readbyqxmd.com/read/27925329/fragmented-qrs-is-a-novel-risk-factor-for-ventricular-arrhythmic-events-after-receiving-cardiac-resynchronization-therapy-in-nonischemic-cardiomyopathy
#15
Miyako Igarashi, Hiroshi Tada, Hiro Yamasaki, Kenji Kuroki, Tomoko Ishizu, Yoshihiro Seo, Takeshi Machino, Nobuyuki Murakoshi, Yukio Sekiguchi, Yuichi Noguchi, Akihiko Nogami, Kazutaka Aonuma
INTRODUCTION: A fragmented QRS (fQRS) is reported to be associated with a poor prognosis or sudden cardiac death (SCD) in patients with Brugada syndrome or ischemic heart disease. However, no studies have clarified the impact of the presence of an fQRS on SCD or ventricular arrhythmic events in patients receiving cardiac resynchronization therapy (CRT). This study aimed to clarify this point in patients with nonischemic cardiomyopathy. METHODS AND RESULTS: This study included 137 heart failure patients with nonischemic cardiomyopathy who received CRT (NYHA functional class: II/III/IV = 25/84/28)...
December 7, 2016: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/27919766/brugada-syndrome-merely-a-ion-channelopathy-a-structural-heart-disease-or-mixed
#16
EDITORIAL
Jyh-Ming Jimmy Juang, Jiunn-Lee Lin
No abstract text is available yet for this article.
December 3, 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27919765/late-gadolinium-enhancement-in-brugada-syndrome-a-marker-for-subtle-underlying-cardiomyopathy
#17
Rachel Bastiaenen, Andrew T Cox, Silvia Castelletti, Yanushi D Wijeyeratne, Nicholas Colbeck, Nadia Pakroo, Hammad Ahmed, Nick Bunce, Lisa Anderson, James C Moon, Sanjay Prasad, Sanjay Sharma, Elijah R Behr
BACKGROUND: There is increasing evidence that the Brugada ECG pattern is a marker of subtle structural heart disease. OBJECTIVE: We characterised Brugada syndrome (BrS) patients using cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). METHODS: BrS was diagnosed according to international guidelines. 26% BrS patients carried SCN5A mutations. CMR data from 78 BrS patients were compared with 78 healthy controls (44±15 vs 42±14 years; p=0...
December 2, 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/27915266/systematic-ajmaline-challenge-in-patients-with-long-qt-3-syndrome-caused-by-the-most-common-mutation-a-multicentre-study
#18
Stephan Hohmann, Boris Rudic, Torsten Konrad, David Duncker, Thorben König, Erol Tülümen, Thomas Rostock, Martin Borggrefe, Christian Veltmann
AIMS: Overlap syndromes of long QT 3 syndrome (LQT3) and the Brugada syndrome (BrS) have been reported. Identification of patients with an overlapping phenotype is crucial before initiation of Class I antiarrhythmic drugs for LQT3. Aim of the present study was to elucidate the yield of ajmaline challenge in unmasking the Brugada phenotype in patients with LQT3 caused by the most common mutation, SCN5A-E1784K. METHODS AND RESULTS: Consecutive families in tertiary referral centres diagnosed with LQT3 caused by SCN5A-E1784K were included in the study...
December 2, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/27909531/brugada-syndrome-risk-stratification-and-management
#19
REVIEW
Konstantinos P Letsas Md Fesc, Stamatis Georgopoulos Md, Konstantinos Vlachos Md, Nikolaos Karamichalakis Md, Ioannis Liatakis Md, Panagiotis Korantzopoulos Md PhD, Tong Liu Md PhD, Michael Efremidis Md, Antonios Sideris Md
The Brugada syndrome (BrS) is an arrhythmogenic disease associated with an increased risk of ventricular fibrillation and sudden cardiac death. The risk stratification and management of BrS patients, particularly of asymptomatic ones, still remains challenging. A previous history of aborted sudden cardiac death or arrhythmic syncope in the presence of spontaneous type 1 ECG pattern of BrS phenotype appear to be the most reliable predictors of future arrhythmic events. Several other ECG parameters have been proposed for risk stratification...
August 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27909514/influence-of-novel-electrocardiographic-features-of-provocable-brugada-ecg-in-arrhythmogenic-cardiomyopathy-and-its-exclusion-by-lead-avr
#20
REVIEW
Stefan Peters
In 19 patients (14 females, mean age 49.1 ± 11.3 years) with typical arrhythmogenic cardiomyopathy and provocable type I Brugada ECG pattern by ajmaline administration were analysed by novel electrocardiographic features as having "true" or "false" Brugada syndrome. Three patients turned out as having false Brugada syndrome, the diagnosis is pure arrhythmogenic cardiomyopathy. In 16 patients, however, true Brugada syndrome could be provoked. In these patients the diagnosis was arrhythmogenic cardiomyopathy associated by provocable Brugada syndrome...
June 2016: Journal of Atrial Fibrillation
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