The Brugada syndrome: clinical, electrophysiologic and genetic aspects ☆
TLDR
This review deals with the clinical, basic and genetic aspects of a recently highlighted form of idiopathic ventricular fibrillation known as the Brugada syndrome and attempts to correlate the electrocardiographic manifestations with cellular and ionic heterogeneity known to exist within the heart under normal and pathophysiologic conditions so as to identify the cellular basis and thus potential diagnostic and therapeutic approaches.About:
This article is published in Journal of the American College of Cardiology.The article was published on 1999-01-01 and is currently open access. It has received 492 citations till now. The article focuses on the topics: Brugada syndrome & Ajmaline.read more
Citations
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Journal ArticleDOI
Cellular Basis for the Brugada Syndrome and Other Mechanisms of Arrhythmogenesis Associated With ST-Segment Elevation
Gan-Xin Yan,Charles Antzelevitch +1 more
TL;DR: Depression or loss of the action potential dome in RV epicardium creates a transmural voltage gradient that may be responsible for the ST-segment elevation observed in the Brugada syndrome and other syndromes exhibiting similar ECG manifestations.
Journal ArticleDOI
Natural History of Brugada Syndrome Insights for Risk Stratification and Management
Silvia G. Priori,Carlo Napolitano,Maurizio Gasparini,Carlo Pappone,Paolo Della Bella,Umberto Giordano,Raffaella Bloise,Carla Giustetto,Roberto De Nardis,Massimiliano Grillo,Elena Ronchetti,Giovanna Faggiano,Janni Nastoli +12 more
TL;DR: The information on the natural history of patients obtained in this study allowed elaboration of a risk-stratification scheme to quantify the risk for sudden cardiac death and to target the use of the implantable cardioverter-defibrillator.
Journal ArticleDOI
Sodium Channel Blockers Identify Risk for Sudden Death in Patients With ST-Segment Elevation and Right Bundle Branch Block but Structurally Normal Hearts
Ramon Brugada,Josep Brugada,Charles Antzelevitch,Glenn E. Kirsch,Domenico Potenza,Jeffrey A. Towbin,Pedro Brugada +6 more
TL;DR: A similar incidence of potentially lethal arrhythmias in patients displaying transient versus persistent ST-segment elevation and right bundle branch block is demonstrated, as well as the effectiveness of sodium channel blockers to unmask the syndrome and, thus, identify patients at risk.
Journal ArticleDOI
Ionic Mechanisms Responsible for the Electrocardiographic Phenotype of the Brugada Syndrome Are Temperature Dependent
Robert Dumaine,Jeffrey A. Towbin,Pedro Brugada,Matteo Vatta,Dmitri V. Nesterenko,Vladislav V. Nesterenko,Josep Brugada,Ramon Brugada,Charles Antzelevitch +8 more
TL;DR: The findings explain the features of the ECG of Brugada patients, illustrate for the first time a cardiac sodium channel mutation of which the arrhythmogenicity is revealed only at temperatures approaching the physiological range, and suggest that some patients may be more at risk during febrile states.
Journal ArticleDOI
The M cell: its contribution to the ECG and to normal and abnormal electrical function of the heart.
Charles Antzelevitch,Wataru Shimizu,Gan-Xin Yan,Serge Sicouri,J Weissenburger,Vladislav V. Nesterenko,Alexander Burashnikov,J. M. Di Diego,Jeffrey E. Saffitz,George Thomas +9 more
TL;DR: A comprehensive characterization of the M cell, its contribution to transmural heterogeneity, and its role in the normal electrical function of the heart, in the inscription of the ECG, and in the development of QT dispersion, T wave alternans, long QT intervals, and cardiac arrhythmias, such as torsades de pointes are provided.
References
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Journal ArticleDOI
Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report.
Pedro Brugada,Josep Brugada +1 more
TL;DR: Common clinical and ECG features define a distinct syndrome in this group of patients with recurrent episodes of aborted sudden death unexplainable by currently known diseases, not explainable by electrolyte disturbances, ischemia or structural heart disease.
Journal ArticleDOI
Sudden Cardiac Death
TL;DR: Total mortality, rather than classifications of cardiac and arrhythmic mortality, should be used as primary objectives for many outcome studies.
Book
Cardiac Electrophysiology : From Cell to Bedside
Douglas P. Zipes,José Jalife +1 more
TL;DR: Part I - Molecular Bases Of Ion Channel Activity PART II - Biophysics and Regulation of Cardiac Ion Channels PART III - Pharmacology of Card cardiac Ion Ch channels PART IV - Cellular Electrophysiology PART V - Models of Cardiopulmonary Excitation PART VI - Neural Control ofCardiac Electrical Activity
Journal ArticleDOI
Genetic basis and molecular mechanism for idiopathic ventricular fibrillation
Qiuyun Chen,Glenn E. Kirsch,Danmei Zhang,Ramon Brugada,Josep Brugada,Pedro Brugada,Domenico Potenza,Angel Moya,Martin Borggrefe,Günter Breithardt,Rocio Ortiz-Lopez,Zhiqing Wang,Charles Antzelevitch,Richard E. O'Brien,Eric Schulze-Bahr,Mark T. Keating,Jeffrey A. Towbin,Qing Wang +17 more
TL;DR: It is shown that sodium channels with the missense mutation recover from inactivation more rapidly than normal and that the frameshift mutation causes the sodium channel to be non-functional.
Journal ArticleDOI
Cellular Basis for the Electrocardiographic J Wave
Gan-Xin Yan,Charles Antzelevitch +1 more
TL;DR: The results provide the first direct evidence in support of the hypothesis that heterogeneous distribution of a transient outward current-mediated spike-and-dome morphology of the action potential across the ventricular wall underlies the manifestation of the electrocardiographic J wave.
Related Papers (5)
Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report.
Pedro Brugada,Josep Brugada +1 more
Genetic basis and molecular mechanism for idiopathic ventricular fibrillation
Cellular Basis for the Brugada Syndrome and Other Mechanisms of Arrhythmogenesis Associated With ST-Segment Elevation
Gan-Xin Yan,Charles Antzelevitch +1 more