STATE-OF-THE-ART PAPER
Brugada syndrome: 19922002
A historical perspective
Charles Antzelevitch, PhD, FACC*,*,
Pedro Brugada, MD, PhD ,
Josep Brugada, MD, PhD ,
Ramon Brugada, MD, FACC*,
Jeffrey A. Towbin, MD, FACC and
Kolawanee Nademanee, MD, FACC||
* Masonic Medical Research Laboratory, Utica, New York, USA
Cardiovascular Center, OLV Hospital, Aalst, Belgium
Cardiovascular Institute, Hospital Clinic, University of Barcelona, Barcelona, Spain
Pediatrics Department, Section of Cardiology, Baylor College of Medicine, Houston, Texas, USA
|| Pacific Rim Cardiac Electrophysiology and Research, Inglewood, California, USA
Manuscript received October 30, 2002;
revised manuscript received December 20, 2002,
accepted January 24, 2003.
* Reprint requests and correspondence: Dr. Charles Antzelevitch, Masonic Medical Research Laboratory, 2150 Bleecker Street, Utica, New York 13501, USA. ca{at}mmrl.edu
An intriguing new clinical entity characterized by ST-segment elevation in the right precordial electrocardiographic leads and a high incidence of sudden death in individuals with structurally normal hearts was described by Pedro and Josep Brugada in 1992. The past decade has witnessed an exponential rise in the number of reported cases and a dramatic proliferation of papers serving to define the clinical, genetic, cellular, ionic, and molecular aspects of this disease. The purpose of this brief review is to chronicle the historical highlights that have brought us to our present understanding of Brugada syndrome.
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Abbreviations and Acronyms
| | ARVD/C | | arrhythmogenic right ventricular dysplasia/cardiomyopathy | | ECG | | electrocardiogram/electrocardiographic | | EPS | | electrophysiologic study | | ICD | | implantable cardioverter-defibrillator | | INa | | sodium channel current | | Ito | | transient outward current | | RBBB | | right bundle branch block | | RV | | right ventricle/ventricular | | RVOT | | right ventricular outflow tract | | SAECG | | signal-averaged electrocardiogram | | VF | | ventricular fibrillation | | VT | | ventricular tachycardia |
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 |
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|
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|
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|
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|
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|
 |
|

|
 |

|
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|
 |
|

|
 |

|
 |
 
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|
 |
|

|
 |

|
 |
 
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|
 |
|

|
 |

|
 |
 
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|
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|
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|

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|
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