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Brugada syndrome

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https://www.readbyqxmd.com/read/28227777/analysis-of-a-cardiovascular-model-for-the-study-of-the-autonomic-response-of-brugada-syndrome-patients
#1
Mireia Calvo, Virginie Le Rolle, Daniel Romero Perez, Nathalie Behar, Pedro Gomis, Philippe Mabo, Alfredo I Hernandez, Mireia Calvo, Virginie Le Rolle, Daniel Romero Perez, Nathalie Behar, Pedro Gomis, Philippe Mabo, Alfredo I Hernandez, Mireia Calvo, Daniel Romero Perez, Pedro Gomis, Philippe Mabo, Nathalie Behar, Alfredo I Hernandez, Virginie Le Rolle
This paper describes the integration of mathematical models of the cardiac electrical activity, the cardiovascular system and the baroreceptor reflex control of the autonomic nervous system, with a model representing a head-up tilt test. Sensitivity analyses are performed in order to determine those model parameters producing the greatest significant effects on heart rate and blood pressure. An optimization step is then applied to the most influential parameters in order to find the best model fit to real cardiac data obtained from a patient suffering from Brugada syndrome and a healthy subject, in supine and upright postures during a tilt test...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28227258/electrocardiographic-waveforms-fitness-check-device-technique-for-sudden-cardiac-death-risk-screening
#2
O J Escalona, M Mendoza, O J Escalona, M Mendoza, O J Escalona, M Mendoza
A novel cardiac health device technique development for reliable, non-invasive and cost-effective heart screening in preventive cardiovascular healthcare is presented. In particular, identification of apparently healthy individuals involved in sports activities (particularly in the young, age <; 35 years) who may be at-risk of sudden-cardiac-death (SCD) is mainly focused here. Nevertheless, the same device technique may be prospectively extended for detecting cardiovascular abnormalities in children and adolescents with type1-diabetes, and also in detecting patients with Brugada syndrome...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28218629/fever-induced-brugada-pattern-misdiagnosed-as-an-acute-myocardial-infarction
#3
David Tadin, Roberto Quintal
The Brugada syndrome is a rare condition associated with increased risk of ventricular tachyarrhythmias and sudden cardiac death (SCD). The Brugada pattern on electrocardiogram (EKG) is known to be revealed by several precipitants including febrile illnesses. The appearance of a Brugada pattern on EKG with fever may indicate an elevated risk of arrhythmia or sudden cardiac death. We report a case in which the electrocardiographic abnormality of Brugada pattern induced by sepsis was initially misinterpreted as a ST-segment elevation myocardial infarction (STEMI)...
January 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28217615/type-1-brugada-pattern-electrocardiogram-induced-by-hypokalemia
#4
Thein Swe, Muhammad Hassan Dogar
Coved-type ST-segment elevation in the right precordial leads are the characteristics of Brugada syndrome, an inherited arrhythmogenic ion channel disease, which could lead to ventricular arrhythmia and sudden death. Hypokalemia alone may induce Type 1 Brugada pattern electrocardiogram (EKG), and the association has rarely been reported. We describe a patient with hypokalemia 2.9 mmol/L and the appearance of new right bundle branch block pattern with coved ST-segment elevations with inverted T wave in leads V1-V2...
July 2016: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/28217227/cardiac-conduction-defects-and-brugada-syndrome-a-family-with-overlap-syndrome-carrying-a-nonsense-scn5a-mutation
#5
Hisaaki Aoki, Yoshihide Nakamura, Seiko Ohno, Takeru Makiyama, Minoru Horie
BACKGROUND: Phenotypes often differ even within family members carrying the same SCN5A mutation. We aimed to evaluate the genetic modifiers in a family with Brugada syndrome (BrS) and sick sinus syndrome (SSS) with an SCN5A mutation that causes the truncated alpha-subunit of cardiac Na channel protein. METHODS: To detect the genetic modifiers, we performed targeted panel sequencing of the coding region of 46 genes that are related to primary arrhythmia syndrome, by using a bench-top, next generation sequencer...
February 2017: Journal of Arrhythmia
https://www.readbyqxmd.com/read/28192206/fever-vs-drug-battling-with-the-brugada-syndrome-substrate
#6
EDITORIAL
Elijah R Behr, Bode Ensam
No abstract text is available yet for this article.
February 9, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28184198/local-left-ventricular-epicardial-j-waves-and-late-potentials-in-brugada-syndrome-patients-with-inferolateral-early-repolarization-pattern
#7
Satoshi Nagase, Masamichi Tanaka, Hiroshi Morita, Koji Nakagawa, Tadashi Wada, Masato Murakami, Nobuhiro Nishii, Kazufumi Nakamura, Hiroshi Ito, Tohru Ohe, Kengo F Kusano
Background: Brugada syndrome (BrS) is characterized by J-point or ST-segment elevation on electrocardiograms (ECGs) and increased risk of ventricular fibrillation (VF). In BrS, epicardial depolarization abnormality with delayed potential on the right ventricular outflow tract is reportedly the predominant mechanism underlying VF. Yet VF occurrence is also associated with early repolarization (ER) pattern in the inferolateral ECG leads, which may represent the inferior and/or left lateral ventricular myocardium...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28183560/anesthesia-and-brugada-syndrome-a-12-year-case-series
#8
Mélanie Duque, Luís Santos, Sandy Ribeiro, Dora Catré
STUDY OBJECTIVE: The aim of this 12-year case series was to review the drugs used during anesthetic management of patients with diagnosis of or risk criteria for Brugada syndrome (BrS), and to document any possible association between these drugs and arrhythmogenic activity or unexplained hemodynamic instability. DESIGN: A retrospective clinical observational study. SETTING: Tertiary hospital. PATIENTS: Thirty-one patients met our inclusion criteria: 20 belonging to group D (diagnosed BrS) and 11 to group R (risk of BrS)...
February 2017: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/28146213/compound-heterozygous-scn5a-mutations-in-a-toddler-are-they-associated-with-a-more-severe-phenotype
#9
Luciana Sacilotto, Hindalis Ballesteros Epifanio, Francisco Carlos da Costa Darrieux, Fanny Wulkan, Theo Gremen Mimary Oliveira, Denise Tessariol Hachul, Alexandre da Costa Pereira, Mauricio Ibrahim Scanavacca
Compound heterozygosity has been described in inherited arrhythmias, and usually associated with a more severe phenotype. Reports of this occurrence in Brugada syndrome patients are still rare. We report a study of genotype-phenotype correlation after the identification of new variants by genetic testing. We describe the case of an affected child with a combination of two different likely pathogenic SCN5A variants, presenting sinus node dysfunction, flutter and atrial fibrillation, prolonged HV interval, spontaneous type 1 Brugada pattern in the prepubescent age and familiar history of sudden death...
January 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28146053/cardiac-channelopathies-and-sudden-death-recent-clinical-and-genetic-advances
#10
REVIEW
Anna Fernández-Falgueras, Georgia Sarquella-Brugada, Josep Brugada, Ramon Brugada, Oscar Campuzano
Sudden cardiac death poses a unique challenge to clinicians because it may be the only symptom of an inherited heart condition. Indeed, inherited heart diseases can cause sudden cardiac death in older and younger individuals. Two groups of familial diseases are responsible for sudden cardiac death: cardiomyopathies (mainly hypertrophic cardiomyopathy, dilated cardiomyopathy, and arrhythmogenic cardiomyopathy) and channelopathies (mainly long QT syndrome, Brugada syndrome, short QT syndrome, and catecholaminergic polymorphic ventricular tachycardia)...
January 29, 2017: Biology
https://www.readbyqxmd.com/read/28139454/brugada-syndrome-diagnosis-risk-stratification-and-management
#11
REVIEW
Jean-Baptiste Gourraud, Julien Barc, Aurélie Thollet, Hervé Le Marec, Vincent Probst
Brugada syndrome is a rare inherited arrhythmia syndrome leading to an increased risk of sudden cardiac death, despite a structurally normal heart. Diagnosis is based on a specific electrocardiogram pattern, observed either spontaneously or during a sodium channel blocker test. Among affected patients, risk stratification remains a challenge, despite recent insights from large population cohorts. As implantable cardiac defibrillators - the main therapy in Brugada syndrome - are associated with a high rate of complications in this population, the main challenge is risk stratification of patients with Brugada syndrome...
January 27, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28139199/wide-qrs-in-a-2-month-infant-associated-with-recurrent-ventricular-tachycardia-a-case-of-brugada-like-syndrome
#12
W Jomaa, W Selmi, S Hamdi, M A Azaiez, A El Hraiech, K Ben Hamda, F Maatouk
We report the case of a 2-month old infant who experienced recurrent sustained ventricular tachycardia (VT) in a structurally normal heart. Resting electrocardiogram (ECG) showed wide QRS with a complete right bundle branch bloc (RBBB) morphology. There was no family history of syncope or sudden death, but the ECGs of the father and the brother showed incomplete RBBB with negative T waves on V1 lead. This case seems to fit well with the newly defined entity of Brugada-like syndrome with a highly suspected genetic underlying disposition...
January 27, 2017: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/28134132/heart-rate-complexity-analysis-in-brugada-syndrome-during-physical-stress-testing
#13
M Calvo, P Gomis, D Romero, V Le Rolle, N Béhar, P Mabo, A Hernández
Symptoms such as ventricular arrhythmias in Brugada syndrome (BS) typically occur at rest, especially during sleep, suggesting that the autonomic nervous system (ANS) function may be relevant in the arrhythmogenesis of the disease. The aim of this work was to assess the ANS response captured by nonlinear heart rate variability (HRV) measures in 69 patients diagnosed with BS, who underwent a standardized physical stress test. Heart rate complexity (HRC) was evaluated by the power-law scaling analysis (β slope) during rest, exercise, recovery and rest post-recovery, in order to discriminate between symptomatic and asymptomatic BS patients...
February 2017: Physiological Measurement
https://www.readbyqxmd.com/read/28133471/variability-in-local-action-potential-durations-dispersion-of-repolarization-and-wavelength-restitution-in-aged-wild-type-and-scn5a-mouse-hearts-modeling-human-brugada-syndrome
#14
Gary Tse, Sheung Ting Wong, Vivian Tse, Jie Ming Yeo
No abstract text is available yet for this article.
November 2016: Journal of Geriatric Cardiology: JGC
https://www.readbyqxmd.com/read/28130006/interatrial-block-in-brugada-syndrome
#15
Raimundo Barbosa-Barros, Bryce Alexander, Adrian Baranchuk
No abstract text is available yet for this article.
January 24, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28127136/a-variant-of-brugada-syndrome
#16
Maryna Popp Switzer, Mohamed Teleb, Enoch Agunanne, Aamer Abbas
Brugada syndrome is an inherited disorder that can present with syncope, cardiac arrest, or sudden cardiac death. Multiple genetic mutations have been described that cause this disease. We present a 56-year-old man who sustained an out-of-hospital cardiac arrest, was resuscitated, and was found to have typical features of the Brugada criteria on the electrocardiogram. Genetic testing was positive for a heterozygous mutation in the sodium voltage-gated channel alpha subunit 5 (SCN5A) gene with a p. Leu227Pro (L227P) variant located on exon 6...
January 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28110572/-cardiomyopathy-and-ion-channel-diseases-registry-the-szeged-cardiogen-registry
#17
Péter Blazsó, Kornél Kákonyi, Tamás Forster, Róbert Sepp
The Szeged cardiomyopathy and ion channel diseases registry aims to establish a representative disease-specific registry based on the recruitment of patients with different cardiomyopathies and ion channel diseases followed at the Cardiology Center, University of Szeged. The registry collects patient data on the main forms of primary cardiomyopathies (hypertrophic, dilated, restrictive, arrhythmogenic right ventricular, left ventricular non-compact, tako-tsubo cardiomyopathy) and ion channel diseases (long QT syndrome, short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia)...
January 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28090040/outcomes-of-brugada-syndrome-patients-with-coronary-artery-vasospasm
#18
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
#19
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
#20
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...
February 7, 2017: International Heart Journal
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