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Brugada syndrome

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https://www.readbyqxmd.com/read/28432805/pitfalls-in-electrogram-interpretation-subcutaneous-cardioverter-defibrillator-malfunction-in-brugada-syndrome
#1
Giovanni Morani, Bruna Bolzan, Luca Tomasi, Ruggero Tomei, Corrado Vassanelli
A patient with Brugada Syndrome implanted with subcutaneous cardioverter defibrillator (S-ICD) had oversensing episodes treated with S-ICD shocks. Comparable artefacts weren't evocable with S-ICD pocket manipulation. The fluoroscopy excluded S-ICD macroscopic damage. The device extraction revealed undamaged pulse generator and connector, but the lead was inappropriately tunnelled under the sixth rib. Then the S-ICD malfunction was due to lead microscopic damage caused by lead rubbing the rib surface. This article is protected by copyright...
April 22, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28431069/-tpeak-tend-qrs-and-tpeak-tend-qt-%C3%A3-qrs-novel-markers-for-predicting-arrhythmic-risk-in-the-brugada-syndrome
#2
Gary Tse
No abstract text is available yet for this article.
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28416987/importance-of-exercise-testing-shortly-after-subcutaneous-implantable-cardioverter-defibrillator-implantation-in-patients-with-brugada-syndrome-the-first-case-of-associated-inappropriate-shock-in-japan
#3
Kohei Ishibashi, Takashi Noda, Tsukasa Kamakura, Mitsuru Wada, Yuko Inoue, Hideo Okamura, Kengo Kusano
We report the case of a 51-year-old patient with Brugada syndrome (BrS) who experienced inappropriate shock due to T-wave oversensing (TWOS) during exercise when the optimal sensing vector was selected based on the automatic analysis by a subcutaneous implantable cardioverter-defibrillator (S-ICD). After selecting another vector during exercise testing, TWOS did not re-occur. Selection of appropriate sensing vector based on analyses under various conditions, including during exercise after S-ICD implantation, should be considered for patients with BrS...
April 2017: Journal of Arrhythmia
https://www.readbyqxmd.com/read/28411139/the-quidam-study-hydroquinidine-therapy-in-the-management-of-brugada-syndrome-patients-at-high-arrhythmic-risk
#4
Antoine Andorin, Jean-Baptiste Gourraud, Jacques Mansourati, Swanny Fouchard, Hervé le Marec, Philippe Maury, Philippe Mabo, Jean-Sylvain Hermida, Jean-Claude Deharo, Beatrice Delasalle, Simon Esnault, Nicolas Sadoul, Jean-Marc Davy, Antoine Leenhardt, Didier Klug, Pascal Defaye, Dominique Babuty, Frédéric Sacher, Vincent Probst
BACKGROUND: Although the implantable cardiac defibrillator (ICD) remains the main therapy in Brugada syndrome (BrS), it does not reduce life-threatening ventricular arrhythmia. Based on pathophysiological mechanisms, hydroquinidine (HQ) has been suggested for the effective prevention of arrhythmia. OBJECTIVE: To provide evidence-based data supporting HQ use to prevent life threatening ventricular arrhythmia in high-risk patients with BrS. METHODS: We performed a prospective multicentre randomised (HQ vs...
April 11, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28401855/brugada-syndrome-and-calcium-channel-mutation-in-a-patient-with-congenital-deaf-mutism
#5
Uğur Canpolat, Cem Coteli, Kudret Aytemir
To the best of our knowledge, for the first time in the literature, we described a congenitally deaf-mute patient with Brugada syndrome (BrS) in whom a mutation in L-type Ca(+2) channel [CACNA1C (Cav1.2α1)] was identified.
January 2017: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/28401854/inappropriate-implantable-cardioverter-defibrillator-shocks-in-brugada-syndrome-pattern-in-primary-and-secondary-prevention
#6
Aimé Bonny, Mohammed A Talle, Thibaut Vaugrenard, Jérôme Taieb, Marcus Ngantcha
BACKGROUND: Inappropriate implantable cardioverter-defibrillator (ICD) shocks is a common complication in Brugada syndrome. However, the incidence in recipients of ICD for primary and secondary prevention is unknown. METHOD AND RESULTS: We compared the rate of inappropriate shocks in patients with Brugada syndrome that had an ICD for primary and secondary prevention. We studied 51 patients, 86.5% of whom were males. Their mean age at diagnosis was 47 ± 11 years...
January 2017: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/28398527/-tpeak-tend-qrs-and-tpeak-tend-qt-%C3%A3-qrs-as-risk-markers-in-brugada-syndrome-authors-reply
#7
Sven Zumhagen, Birgit Stallmeyer, Lars Eckardt, Eric Schulze-Bahr
No abstract text is available yet for this article.
April 7, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28392310/the-role-of-the-autonomic-nervous-system-in-arrhythmias-and-sudden-cardiac-death
#8
REVIEW
Sonia Franciosi, Frances K G Perry, Thomas M Roston, Kathryn R Armstrong, Victoria E Claydon, Shubhayan Sanatani
The autonomic nervous system (ANS) is complex and plays an important role in cardiac arrhythmia pathogenesis. A deeper understanding of the anatomy and development of the ANS has shed light on its involvement in cardiac arrhythmias. Alterations in levels of Sema-3a and NGF, both growth factors involved in innervation patterning during development of the ANS, leads to cardiac arrhythmias. Dysregulation of the ANS, including polymorphisms in genes involved in ANS development, have been implicated in sudden infant death syndrome...
March 31, 2017: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/28391114/genotype-phenotype-dilemma-in-a-case-of-sudden-cardiac-death-with-the-e1053k-mutation-and-a-deletion-in-the-scn5a-gene
#9
T Jenewein, B M Beckmann, S Rose, H H Osterhues, U Schmidt, C Wolpert, P Miny, C Marschall, M Alders, C R Bezzina, A A M Wilde, S Kääb, S Kauferstein
Mutations in the cardiac sodium channel gene SCN5A may result in various arrhythmia syndromes such as long QT syndrome type 3 (LQTS), Brugada syndrome (BrS), sick sinus syndrome (SSS), cardiac conduction diseases (CCD) and possibly dilated cardiomyopathy (DCM). In most of these inherited cardiac arrhythmia syndromes the phenotypical expression may range from asymptomatic phenotypes to sudden cardiac death (SCD). A 16-year-old female died during sleep. Autopsy did not reveal any explanation for her death and a genetic analysis was performed...
March 20, 2017: Forensic Science International
https://www.readbyqxmd.com/read/28389016/myotonic-dystrophy-and-brugada-syndrome-a-common-pathophysiologic-pathway
#10
REVIEW
Andrés Ricardo Pérez-Riera, Adrian Baranchuk, Li Zhang, Raimundo Barbosa-Barros, Luiz Carlos de Abreu, Pedro Brugada
Type 1 myotonic dystrophy (DM1) is a hereditary neuromuscular disease affecting multiple organs in human adults. Here we report a 42-year-old man diagnosed with DM1. Having a history of progressive muscular weakness and gradual loss of visual acuity, he was referred to us by his ophthalmologist for risk assessment of undergoing cataract surgery. Cardiology workup revealed type 1 Brugada ECG pattern, positive late potentials and inducible ventricular fibrillation in an electrophysiology study. Literature review revealed that those ECG changes may be observed in DM1, suggesting that DM1 and Brugada syndrome may share a common pathophysiologic pathway...
March 14, 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/28384965/anaesthetic-management-in-brugada-syndrome-a-case-report
#11
Sulochana Dash, Shanmugam Pragathee
Brugada Syndrome (BS) is a rare congenital cardiac disorder involving cardiac sodium channels, sometimes presenting with ventricular arrhythmia and sudden cardiac death. Here, we are reporting a case of BS who presented for laparotomy due to obstructed inguinal hernia which was managed satisfactorily with combined epidural and general anaesthesia without any complications.
February 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28381391/brugada-phenocopy-induced-by-propafenone-overdose-and-successful-treatment-a-case-report
#12
Mehmet Emre Arı, Filiz Ekici
BACKGROUND: Brugada syndrome is an inherited arrhythmogenic disease that may cause sudden cardiac death due to ventricular fibrillation in young adults. Brugada syndrome caused by propafenone intoxication has been noted rarely in the literature. We reported a rare case, Brugada-phenocopy due to propafenone intoxication and its treatment. CASE REPORT: A fifteen-year-old girl having a seizure was brought to the emergency room. She took 1.5 g propafenone (Rythmol, Abbott, Chicago, IL, USA) for suicidal intention...
April 6, 2017: Balkan Medical Journal
https://www.readbyqxmd.com/read/28379344/a-score-model-to-predict-risk-of-events-in-patients-with-brugada-syndrome
#13
Juan Sieira, Giulio Conte, Giuseppe Ciconte, Gian-Battista Chierchia, Ruben Casado-Arroyo, Giannis Baltogiannis, Giacomo Di Giovanni, Yukio Saitoh, Justo Juliá, Giacomo Mugnai, Mark La Meir, Francis Wellens, Jens Czapla, Gudrun Pappaert, Carlo de Asmundis, Pedro Brugada
Aims: Risk stratification in Brugada Syndrome (BS) remains challenging. Arrhythmic events can occur life-long and studies with long follow-ups are sparse. The aim of our study was to investigate long-term prognosis and risk stratification of BS patients. Methods and results: A single centre consecutive cohort of 400 BS patients was included and analysed. Mean age was 41.1 years, 78 patients (19.5%) had a spontaneous type I electrocardiogram (ECG). Clinical presentation was aborted sudden cardiac death (SCD) in 20 patients (5...
March 31, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28372913/antitachycardia-pacing-effectiveness-for-monomorphic-ventricular-tachycardia-in-brugada-syndrome-after-quinidine-administration
#14
Paolo D Dallaglio, Ignasi Anguera, Arcadi García-Alberola, Xavier Sabaté, Ángel Cequier, Josep Brugada
No abstract text is available yet for this article.
March 31, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28370132/infant-sudden-death-mutations-responsible-for-impaired-nav1-5-channel-trafficking-and-function
#15
Ivan Gando, Jace Morganstein, Kundan Jana, Thomas V McDonald, Yingying Tang, William A Coetzee
BACKGROUND: Two genetic variants in SCN5A, encoding the Nav1.5 Na(+) channel α-subunit, were found in a five month-old girl who died suddenly in her sleep. The first variant is a missense mutation, resulting in an amino acid change (Q1832E), which has been described (but not characterized) in a patient with Brugada syndrome. The second is a nonsense mutation that produces a premature stop codon and a C-terminal truncation (R1944Δ). METHODS AND RESULTS: To investigate their functional relevance with patch clamp experiments in transfected HEK293 cells...
March 31, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28369767/multiple-targets-for-flecainide-action-implications-for-cardiac-arrhythmogenesis
#16
REVIEW
Samantha C Salvage, Karthik H Chandrasekharan, Kamalan Jeevaratnam, Angela F Dulhunty, Andrew J Thompson, Antony P Jackson, Christopher L-H Huang
Flecainide suppresses cardiac tachyarrhythmias including paroxysmal atrial fibrillation, supraventricular tachycardia, arrhythmic long QT syndromes (LQTS), as well as the Ca(2+) -mediated, catecholaminergic polymorphic ventricular, tachycardia (CPVT). However, flecainide can also exert pro-arrhythmic effects most notably following myocardial infarction and when used to diagnose Brugada Syndrome (BrS). These divergent actions result from its physiological and pharmacological actions at multiple, interacting, levels of cellular organisation...
April 3, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28367844/surface-electrocardiogram-screening-for-subcutaneous-implantable-cardioverter-defibrillators-in-japanese-patients-with-and-without-brugada-syndrome
#17
Mihoko Kawabata, Masahiko Goya, Takeshi Sasaki, Shingo Maeda, Atsuhiko Yagishita, Yasuhiro Shirai, Masakazu Kaneko, Shinya Shiohira, Mitsuaki Isobe, Kenzo Hirao
BACKGROUND: Subcutaneous implantable cardioverter-defibrillators (S-ICD) could eliminate lead-associated complications. We assessed the prevalence of S-ICD ineligibility in conventional ICD recipients and compared it in patients with and without Brugada syndrome (BrS).Methods and Results:Consecutive patients with a transvenous ICD without an indication for antibradycardia pacing were assessed. A patient was considered eligible for S-ICD if the ECG satisfied the screening template, both supine and standing, in ≥1 lead...
March 28, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28364267/utility-of-12-lead-and-signal-averaged-holter-electrocardiograms-after-pilsicainide-provocation-for-risk-stratification-in-brugada-syndrome
#18
Jun Kakihara, Masahiko Takagi, Yusuke Hayashi, Hiroaki Tatsumi, Atsushi Doi, Minoru Yoshiyama
Non-invasive risk stratification for ventricular fibrillation (VF) in Brugada syndrome (BrS) has not been fully evaluated. The aim of this study was to assess the utility of signal-averaged Holter electrocardiogram (Holter SAECG) and 12-lead Holter electrocardiogram (Holter ECG) after a pilsicainide provocation test for non-invasive risk stratification in BrS. We enrolled 30 consecutive patients with BrS [divided into 2 groups: the VF group, those with a previous history of VF (n = 10); and the non-VF group, those without a history of VF (n = 20)] and 10 control subjects without type 1 ECG...
March 31, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28363711/prevalence-of-electrocardiographic-patterns-associated-with-sudden-cardiac-death-in-the-spanish-population-aged-40-years-or-older-results-of-the-ofrece-study
#19
Paula Awamleh García, Joaquín Jesús Alonso Martín, Catherine Graupner Abad, Rosa María Jiménez Hernández, Alejandro Curcio Ruigómez, Pedro Talavera Calle, Carmen Cristóbal Varela, José Serrano Antolín, Javier Muñiz, Juan José Gómez Doblas, Eulalia Roig
INTRODUCTION AND OBJECTIVES: Some electrocardiographic patterns are associated with an increased risk of sudden cardiac death due to ventricular arrhythmias. There is no information on the prevalence of these patterns in the general population in Spain. The objective of this study was to analyze the prevalence of these patterns and associated clinical and epidemiological factors. METHODS: This subanalysis of the OFRECE study selected a representative sample of the Spanish population aged ≥ 40 years...
March 28, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28344931/proarrhythmogenic-effects-of-lamotrigine-during-ajmaline-testing-for-brugada-syndrome
#20
Kevin M W Leong, Henry Seligman, Amanda M Varnava
No abstract text is available yet for this article.
March 2017: HeartRhythm Case Reports
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