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Brugada syndrome

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https://www.readbyqxmd.com/read/29163198/ankyrins-and-spectrins-in-cardiovascular-biology-and-disease
#1
REVIEW
Mona M El Refaey, Peter J Mohler
Ankyrins are adaptor proteins critical for the expression and targeting of cardiac membrane proteins, signaling molecules, and cytoskeletal elements. Findings in humans and animal models have highlighted the in vivo roles for ankyrins in normal physiology and in cardiovascular disease, most notably in cardiac arrhythmia. For example, human ANK2 loss-of-function variants are associated with a complex array of electrical and structural phenotypes now termed "ankyrin-B syndrome," whereas alterations in the ankyrin-G pathway for Nav channel targeting are associated with human Brugada syndrome...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/29159074/only-some-patients-with-bulbar-and-spinal-muscular-atrophy-may-develop-cardiac-disease
#2
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/29148067/should-theophylline-be-added-to-the-j-wave-syndrome-therapeutic-armamentarium
#3
EDITORIAL
Charles Antzelevitch, Sami Viskin
The substrate for both early repolarization syndrome (ERS) and Brugada syndrome (BrS), the so-called J wave syndromes, has been shown to develop as a consequence of genetic variants causing a loss of function of inward currents such sodium (INa ) and calcium (ICa ) channel currents or a gain of function in outward currents such as the transient outward current (Ito ). These changes in ion channel function result in an outward shift of the balance of currents active during the early phases of the epicardial action potential in the right ventricular outflow tract (RVOT) of the heart in the case BrS and in the inferior left ventricle in the case of ERS...
November 16, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29145303/epicardial-radiofrequency-catheter-ablation-of-brugada-syndrome-with-electrical-storm-during-ventricular-fibrillation-a-case-report
#4
Shufen Jiang, Xiaomeng Yin, Chang Dong, Yunlong Xia, Jinqiu Liu
RATIONALE: Brugada syndrome (BrS) is characterized by ST segment elevation at the J point ≥2 mm in the right precordial electrocardiogram (ECG) leads, in the absence of structural heart disease, electrolyte disturbances, or ischemia. It is a well-described cause of sudden death in young patients, especially in the age of between 30 and 40 years old. Here, we reported an unusual case of electrical storm (ES) of ventricular fibrillation (VF) caused by BrS with complete right bundle-branch block (CRBBB) in a 75-year-old male patient...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29133531/unusual-st-segment-elevation-in-the-anterolateral-precordial-leads-ischemia-brugada-phenocopy-brugada-syndrome-all-or-none
#5
Andrés Ricardo Pérez-Riera, Raimundo Barbosa-Barros, Rodrigo Daminello-Raimundo, Luiz Carlos de Abreu, Adrian Baranchuk
No abstract text is available yet for this article.
November 14, 2017: Circulation
https://www.readbyqxmd.com/read/29105669/-ten-questions-on-brugada-syndrome
#6
Pietro Delise
Brugada syndrome is a possible cause of sudden cardiac death in subjects with characteristic ECG pattern (type 1 Brugada ECG pattern). Brugada ECG pattern is not infrequent in the general population and the challenge is to identify within a large population those patients who are really at risk. Unfortunately, this is a partially unresolved problem and this uncertainty determines an inhomogeneous behavior among doctors. Some of them overestimate the risk extensively indicating cardioverter-defibrillator implantation, others apply restrictive indications of guidelines, many are not able to decide without doubts...
November 2017: Giornale Italiano di Cardiologia
https://www.readbyqxmd.com/read/29097318/systematic-review-for-the-2017-aha-acc-hrs-guideline-for-management-of-patients-with-ventricular-arrhythmias-and-the-prevention-of-sudden-cardiac-death-a-report-of-the-american-college-of-cardiology-american-heart-association-task-force-on-clinical-practice
#7
Fred M Kusumoto, Kent R Bailey, Ahmad Sami Chaouki, Abhishek J Deshmukh, Sandeep Gautam, Robert J Kim, Daniel B Kramer, Litsa K Lambrakos, Naseer H Nasser, Dan Sorajja
BACKGROUND: Although large randomized clinical trials have found that primary prevention use of an implantable cardioverter-defibrillator (ICD) improves survival in patients with cardiomyopathy and heart failure symptoms, patients who receive ICDs in practice are often older and have more comorbidities than patients who were enrolled in the clinical trials. In addition, there is a debate among clinicians on the usefulness of electrophysiological study for risk stratification of asymptomatic patients with Brugada syndrome...
October 25, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29097297/systematic-review-for-the-2017-aha-acc-hrs-guideline-for-management-of-patients-with-ventricular-arrhythmias-and-the-prevention-of-sudden-cardiac-death-a-report-of-the-american-college-of-cardiology-american-heart-association-task-force-on-clinical-practice
#8
Fred M Kusumoto, Kent R Bailey, Ahmad Sami Chaouki, Abhishek J Deshmukh, Sandeep Gautam, Robert J Kim, Daniel B Kramer, Litsa K Lambrakos, Naseer H Nasser, Dan Sorajja
BACKGROUND: Although large randomized clinical trials have found that primary prevention use of an implantable cardioverter-defibrillator (ICD) improves survival in patients with cardiomyopathy and heart failure symptoms, patients who receive ICDs in practice are often older and have more comorbidities than patients who were enrolled in the clinical trials. In addition, there is a debate among clinicians on the usefulness of electrophysiological study for risk stratification of asymptomatic patients with Brugada syndrome...
October 24, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29091329/repolarization-abnormalities-unmasked-with-exercise-in-sudden-cardiac-death-survivors-with-structurally-normal-hearts
#9
Kevin Mw Leong, Fu Siong Ng, Caroline Roney, Christopher Cantwell, Matthew J Shun-Shin, Nicholas Wf Linton, Zachary I Whinnett, David C Lefroy, D Wyn Davies, Sian E Harding, Phang Boon Lim, Darrel Francis, Nicholas S Peters, Amanda M Varnava, Prapa Kanagaratnam
BACKGROUND: Models of cardiac arrhythmogenesis predict that non-uniformity in repolarization and/or depolarization promotes ventricular fibrillation and is modulated by autonomic tone, but this is difficult to evaluate in patients. We hypothesize that such spatial heterogeneities would be detected by non-invasive ECG imaging (ECGi) in sudden cardiac death (SCD) survivors with structurally normal hearts under physiological stress. METHODS: ECGi was applied to 11 SCD survivors, 10 low-risk Brugada Syndrome patients (BrS) and 10 controls undergoing exercise treadmill testing...
November 1, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/29081906/brugada-type-1-electrocardiogram-should-we-treat-the-electrocardiogram-or-the-patient
#10
REVIEW
Pietro Delise, Giuseppe Allocca, Nadir Sitta
Patients with a Brugada type 1 electrocardiogram (ECG) pattern may suffer sudden cardiac death (SCD). Recognized risk factors are spontaneous type 1 ECG and syncope of presumed arrhythmic origin. Familial sudden cardiac death (f-SCD) is not a recognized independent risk factor. Finally, positive electrophysiologic study (+EPS) has a controversial prognostic value. Current ESC guidelines recommend implantable cardioverter defibrillator (ICD) implantation in patients with a Brugada type 1 ECG pattern if they have suffered a previous resuscitated cardiac arrest (class I recommendation) or if they have syncope of presumed cardiac origin (class IIa recommendation)...
September 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/29079570/thoracic-epidural-anesthesia-can-be-effective-for-the-short-term-management-of-ventricular-tachycardia-storm
#11
Duc H Do, Jason Bradfield, Olujimi A Ajijola, Marmar Vaseghi, John Le, Siamak Rahman, Aman Mahajan, Akihiko Nogami, Noel G Boyle, Kalyanam Shivkumar
BACKGROUND: Novel therapies aimed at modulating the autonomic nervous system, including thoracic epidural anesthesia (TEA), have been shown in small case series to be beneficial in treating medically refractory ventricular tachycardia (VT) storm. However, it is not clear when these options should be considered. We reviewed a multicenter experience with TEA in the management of VT storm to determine its optimal therapeutic use. METHODS AND RESULTS: Data for 11 patients in whom TEA was instituted for VT storm between July 2005 and March 2016 were reviewed to determine the clinical characteristics, outcomes, and role in management...
October 27, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29077258/whole-exome-sequencing-identifies-a-novel-mutation-of-gpd1l-r189x-associated-with-familial-conduction-disease-and-sudden-death
#12
Hao Huang, Ya-Qin Chen, Liang-Liang Fan, Shuai Guo, Jing-Jing Li, Jie-Yuan Jin, Rong Xiang
Cardiac conduction disease (CCD) is a serious disorder and the leading cause of mortality worldwide. It is characterized by arrhythmia, syncope or even sudden cardiac death caused by the dysfunction of cardiac voltage-gated channel. Previous study has demonstrated that mutations in genes encoding voltage-gated channel and related proteins were the crucial genetic lesion of CCD. In this study, we employed whole-exome sequencing to explore the potential causative genes in a Chinese family with ventricular tachycardia and syncope...
October 27, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/29067908/evaluation-of-baseline-ecg-in-patients-undergoing-oral-flecainide-challenge-test-for-suspected-brugada-syndrome-an-analysis-of-lead-ii
#13
Maneesh K Rai, Mukund A Prabhu, Jayaprakash Shenthar, Natarajan Kumaraswamy U, Ritesh Vekariya, Padmanabh Kamath, Narasimha Pai, Ramanath L Kamath, Vivek Pillai
BACKGROUND AND OBJECTIVES: We analyzed Lead II in patients undergoing an Oral Flecainide Challenge test (FCT), to identify any pointers that could predict a positive FCT and thereby help in recognition of latent BS. METHODS: The following parameters in lead II were retrospectively analyzed from the pre-test ECG in 62 patients undergoing FCT for suspected BS: The presence or absence of S waves, S wave amplitude, duration and upslope duration; J point parameters- Early repolarization, QRS notch, and QRS Slur; ST segment parameters-lack of isoelectric ST segment, ST duration and QT interval...
July 2017: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/29067907/how-much-do-we-need-to-provoke-challenges-and-opportunities-in-refining-the-pharmacological-tests-to-unmask-brugada-syndrome
#14
EDITORIAL
Syamkumar Divakara Menon
No abstract text is available yet for this article.
July 2017: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/29062700/oral-quinine-sulfate-for-the-treatment-of-electrical-storm-and-prevention-of-recurrent-shocks-in-brugada-syndrome-after-failed-cilostazol-therapy
#15
Jayaprakash Shenthar, Siva Sankara Chakali, Debashish Acharya, Javed Parvez, Bharatraj Banavalikar
No abstract text is available yet for this article.
October 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/29062695/inaccurate-diagnosis-of-brugada-syndrome-in-a-healthy-woman-based-on-scn5a-mutation-classification
#16
Simrit K Warring, Heather N Anderson, J Martijn Bos, Michael J Ackerman
No abstract text is available yet for this article.
October 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/29056961/determination-of-action-potential-wavelength-restitution-in-scn5a-mouse-hearts-modelling-human-brugada-syndrome
#17
Gary Tse, Sheung Ting Wong, Vivian Tse, Jie Ming Yeo
No abstract text is available yet for this article.
September 2017: Journal of Geriatric Cardiology: JGC
https://www.readbyqxmd.com/read/29050870/brugada-syndrome-is-associated-with-scar-and-endocardial-involvement-insights-from-high-density-mapping-with-the-rhythmia%C3%A2-mapping-system
#18
Rui Providência, Pedro Carmo, Francisco Moscoso Costa, Diogo Cavaco, Francisco Morgado, Mauricio Scanavacca, Pedro Adragão
The authors report the first catheter ablation of Brugada syndrome in the literature using the Rhythmia™ mapping system. Learning points include: (1) low voltage areas can be documented while mapping in some individuals, suggesting that Brugada syndrome may not be a pure ion channel disorder; (2) typical long fractionated potentials can also be identified in the endocardium, supporting the need to map the endocardium in all Brugada patients requiring ablation; (3) disappearance of the typical coved pattern following ablation does not necessarily predict cure, as the patient we present experienced ventricular fibrillation recurrence a few months later...
October 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29050740/-asymptomatic-brugada-syndrome-from-diagnosis-to-treatment
#19
W Amara, A Feki, F Monsel
Brugada syndrome management may be a difficult question. This article reviews diagnosis, prognosis evaluation, current and investigated treatments.
November 2017: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/29050732/-epidemiology-of-early-repolarization-pattern-in-maghreb
#20
W Amara, S A Bouallouche, A Rezoug, A El Hraiech, A Iusuf, N Hammoudi
An early repolarization variant (ERV) in inferolateral leads has recently been associated with vulnerability to ventricular fibrillation. These studies have been conducted in the occidental countries. The prevalence of ERV in the population of the Maghreb is unknown. The aim of this study was to evaluate the prevalence and risk factors of ERV in a young population from Algeria. METHODS: We assessed the prevalence of ERV within a population of 441 healthy subjects (mean age 25 years) using 12-lead electrocardiography...
November 2017: Annales de Cardiologie et D'angéiologie
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