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Syndrom Brugada

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https://www.readbyqxmd.com/read/28329154/brugada-syndrome-induced-by-braf-and-mek-inhibitors-in-a-melanoma-patient
#1
Charlée Nardin, Morgane Colas, Marc Badoz, Blandine Roche-Kubler, Nicolas Meneveau, Eve Puzenat, François Aubin
No abstract text is available yet for this article.
March 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28326201/hyperkalemia-induced-brugada-phenocopy-a-rare-ecg-manifestation
#2
Muhammad Ameen, Ghulam Akbar, Naeem Abbas, Ghazi Mirrani
Brugada syndrome (BrS) is an inherited disorder of cardiac ion channels characterized by peculiar ECG findings predisposing individuals to ventricular arrhythmias, syncope, and sudden cardiac death (SCD). Various electrolyte disturbances and ion channels blocking drugs could also provoke BrS ECG findings without genetic BrS. Clinical differentiation and recognition are essential for guiding the legitimate action. Hyperkalemia is well known to cause a wide variety of ECG manifestations. Severe hyperkalemia can even cause life threatening ventricular arrhythmias and cardiac conduction abnormalities...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28324986/electrocardiographic-waveforms-fitness-check-device-technique-for-sudden-cardiac-death-risk-screening
#3
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/28321340/right-ventricular-compression-mimicking-brugada-like-electrocardiogram-in-a-patient-with-recurrent-pectus-excavatum
#4
Jinhee Ahn, Jong-Il Choi, Jaemin Shim, Sung Ho Lee, Young-Hoon Kim
Pectus excavatum (PE), the most common skeletal anomaly of chest wall, sometimes requires a surgical correction but recurrent PE is not uncommon. PE usually has a benign course; however, this chest deformity may be associated with symptomatic tachyarrhythmias due to mechanical compression. We report a case of a patient with recurrent PE after surgical correction presenting with palpitation and electrocardiogram (ECG) showing ST-segment elevation on the right precordial leads, which could be mistaken for a Brugada syndrome (BrS)...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28320012/acute-myocardial-infarction-masked-by-brugada-syndrome-a-case-report
#5
Alicia Mateo Martinez, Rocio Cortes Sanchez, Javier Lacunza-Ruiz, Jose Maria Lopez Ayala
No abstract text is available yet for this article.
March 21, 2017: Annals of Internal Medicine
https://www.readbyqxmd.com/read/28316113/the-utility-of-exercise-testing-in-risk-stratification-of-asymptomatic-patients-with-type-1-brugada-pattern
#6
Muthiah Subramanian, Mukund A Prabhu, M S Harikrishnan, Saritha S Shekhar, Praveen G Pai, K U Natarajan
INTRODUCTION: Risk stratification of asymptomatic patients with a Brugada type 1 ECG pattern remains an unresolved clinical conundrum. In contrast to provocative pharmacological testing in Brugada syndrome, there is limited data on the role of exercise stress testing as a risk stratification modality. The objective of this study was to evaluate the utility of exercise testing in asymptomatic patients with type 1 Brugada pattern to prognosticate major arrhythmic events(MAE) during follow-up...
March 18, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28314845/distribution-and-prognostic-significance-of-fragmented-qrs-in-patients-with-brugada-syndrome
#7
Hiroshi Morita, Atsuyuki Watanabe, Yoshimasa Morimoto, Satoshi Kawada, Motomi Tachibana, Koji Nakagawa, Nobuhiro Nishii, Hiroshi Ito
BACKGROUND: Fragmented QRS complexes (fQRS) in the right precordial leads are associated with occurrence of ventricular fibrillation (VF) in Brugada syndrome. Recently, epicardial mapping has revealed abnormal electrograms at the right ventricular (RV) outflow tract and inferior region of the right ventricle. fQRS may reflect the extent of the area of abnormal potentials, but whether the distribution of fQRS has prognostic value is not known. METHODS AND RESULTS: We evaluated the existence of fQRS in 456 patients with Brugada syndrome, including 117 patients with syncope and 23 patients with VF...
March 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28303324/short-qt-syndrome-in-pediatrics
#8
REVIEW
Roberta Pereira, Oscar Campuzano, Georgia Sarquella-Brugada, Sergi Cesar, Anna Iglesias, Josep Brugada, Fernando E S Cruz Filho, Ramon Brugada
Short QT syndrome is a malignant cardiac disease characterized by the presence of ventricular tachyarrhythmias leading to syncope and sudden cardiac death. Currently, international guidelines establish diagnostic criteria when QTc is below 340 ms. This entity is one of the main diseases responsible for sudden cardiac death in the pediatric population. In recent years, clinical, genetic and molecular advances in pathophysiological mechanisms related to short QT syndrome have improved diagnosis, risk stratification, and preventive measures...
March 16, 2017: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
https://www.readbyqxmd.com/read/28290897/-the-brugada-syndrome-as-a-cause-of-sudden-death-diagnostics-and-clinical-manifestations-in-children
#9
I M Miklashevich, M A Shkolnikova
The Brugada syndrome is a hereditary potentially arrhythmogenic disease related to the category of channelopathies. It is manifested as syncopal states and sudden death in young people in the absence of structural cardiac disease. The basis of the disease is genetically determined abnormality of function of ionic channels of cardiomyocytes (sodium, potassium, calcium) phenotypically manifesting as sustained or transitory segment-ST elevation and high risk of polymorphic ventricular tachycardia, fibrillation, and sudden death...
October 2016: Kardiologiia
https://www.readbyqxmd.com/read/28290797/-the-brugada-syndrome-in-a-teenager
#10
I M Miklashevich, E V Kuleshova, S A Termosesov, M A Shkolnikova
The Brugada syndrome (BS) belongs to the group of hereditary channelopathies associated with elevated risk of sudden death (SD) in the absence of structural heart diseases. The disorder phenotypically manifests by specific electrocardiographic pattern, associated with ventricular tachycardia (VT). VT can be accompanied by loss of conscience, and after transformation to ventricular fibrillation result in SD. BS is extremely rare among children and adolescents. We present here a clinical case of teenager (age 17 years) with BS manifested by syncopal state at the background of fever...
February 2017: Kardiologiia
https://www.readbyqxmd.com/read/28274576/long-term-follow-up-of-probands-with-brugada-syndrome
#11
Carlo de Asmundis, Giacomo Mugnai, Gian-Battista Chierchia, Juan Sieira, Giulio Conte, Moises Rodriguez-Mañero, Gudrun Pappaert, Jens Czapla, Jan Nijs, Mark La Meir, Ruben Casado, Erwin Ströker, Valentina De Regibus, Pedro Brugada
This study analyzes the natural history of a large cohort of probands with Brugada syndrome (BrS) to assess the predictive value of different clinical and electrocardiographic parameters for the development of ventricular fibrillation (VF) or sudden cardiac death (SCD) during a long-term follow-up. Baseline characteristics of 289 consecutive probands (203 men; mean age 45 ± 16 years) with a Brugada type 1 electrocardiogram were analyzed. After a mean follow-up of 10.1 ± 4.6 years, 29 malignant arrhythmias occurred...
February 10, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28269522/analysis-of-a-cardiovascular-model-for-the-study-of-the-autonomic-response-of-brugada-syndrome-patients
#12
Mireia Calvo, Virginie Le Rolle, Daniel Romero Perez, Nathalie Behar, Pedro Gomis, Philippe Mabo, Alfredo I Hernandez
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/28266935/genetic-causes-of-sudden-cardiac-death-in-the-young
#13
Andrea Mazzanti, Riccardo Maragna, Silvia G Priori
PURPOSE OF REVIEW: In this article, we discuss the most recent and relevant studies published in the field of inherited arrhythmogenic disorders, focusing in particular on channelopathies (Long QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia) and arrhythmogenic right ventricular cardiomyopathy (ARVC). RECENT FINDINGS: We discuss the updated diagnostic criteria for channelopathies released by the European Society of Cardiology, the new results on the value of programmed electrical stimulation in patients with Brugada syndrome, and the recent evidences supporting a genotype-specific therapy for Long QT syndrome type 3...
March 6, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28258845/noninvasive-ecg-imaging-ecgi-mapping-the-arrhythmic-substrate-of-the-human-heart
#14
Yoram Rudy
This short communication accompanies my presentation at the International Congress on Sudden Cardiac Death held in Prague, March 30-April 1, 2017. It summarizes briefly studies of the cardiac electrophysiological substrate in patients with hereditary arrhythmogenic syndromes - the Long QT and Brugada syndromes - conducted noninvasively, in situ, using Electrocardiographic Imaging (ECGI). The same noninvasive approach was used to map the electrophysiological substrate of a post-infarction myocardial scar and to relate this substrate to the pattern of activation during reentrant ventricular tachycardia...
February 27, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28228734/%C3%AE-subunits-functionally-differentiate-human-kv4-3-potassium-channel-splice-variants
#15
Geoffrey W Abbott
The human ventricular cardiomyocyte transient outward K(+) current (Ito) mediates the initial phase of myocyte repolarization and its disruption is implicated in Brugada Syndrome and heart failure (HF). Human cardiac Ito is generated primarily by two Kv4.3 splice variants (Kv4.3L and Kv4.3S, diverging only by a C-terminal, S6-proximal, 19-residue stretch unique to Kv4.3L), which are differentially remodeled in HF, but considered functionally alike at baseline. Kv4.3 is regulated in human heart by β subunits including KChIP2b and KCNEs, but their effects were previously assumed to be Kv4...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28227777/analysis-of-a-cardiovascular-model-for-the-study-of-the-autonomic-response-of-brugada-syndrome-patients
#16
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
#17
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
#18
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
#19
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
#20
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
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