From the Arrhythmia Unit (J.B.), Cardiovascular Institute, Hospital
Clínic, University of Barcelona, Spain; Cardiology Department (R.B.),
Baylor College of Medicine, Houston, Tex; and Cardiovascular Research and
Teaching Institute (P.B.), OLV Hospital, Aalst, Belgium.
Correspondence to Josep Brugada, MD, Arrhythmia Unit, Cardiovascular Institute, Hospital Clínic, University of Barcelona, Villarroel 170, 08036, Barcelona, Spain. E-mail jepbrugada{at}grn.es
Background—Five years ago, we
described a specific ECG pattern of right bundle-branch block and
ST-segment elevation in leads V1 through V3
associated with sudden death in patients without demonstrable
structural heart disease. Information on long-term outcome has become
available due to pooled data on a large cohort of patients with this
syndrome who are followed at 33 centers worldwide.
Methods and Results—Data on 63 patients (57 men; mean age,
38±17 years) with the described ECG pattern were analyzed in
terms of arrhythmic events and sudden death. Events were
analyzed for patients with at least one episode of aborted
sudden death or syncope of unknown origin before recognition of the
syndrome (symptomatic patients, n=41) and for patients in
whom the ECG pattern was recognized by chance or because of screening
related to sudden death of a relative (asymptomatic
patients, n=22). During a mean follow-up of 34±32 months, an
arrhythmic event occurred in 14 symptomatic patients (34%)
and 6 asymptomatic patients (27%). An automatic
defibrillator was implanted in 35 patients, 15 received pharmacological
therapy with ß-blockers and/or amiodarone, and 13 did not
receive treatment The incidence of arrhythmic events was similar in all
therapy groups (log-rank 0.86); however, total mortality was 0% in the
implantable defibrillator group, 26% in the pharmacological group, and
31% in the no therapy group (log-rank 0.0005). All mortality was due
to sudden death.
Conclusions—Patients without demonstrable structural heart
disease and an ECG pattern of right bundle-branch block and ST-segment
elevation in leads V1 through V3 are at risk
for sudden death. Amiodarone and/or ß-blockers do not protect
them against sudden death, and an implantable defibrillator seems to be
the present treatment of choice.
© 1998 American Heart Association, Inc.
Clinical Investigation and Reports
Right Bundle-Branch Block and ST-Segment Elevation in Leads V1 Through V3
A Marker for Sudden Death in Patients Without Demonstrable Structural Heart Disease
Key Words: death, sudden • electrocardiography • fibrillation
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J M Butler Brugada syndrome--the missed epidemic Emerg. Med. J., November 1, 2000; 17(6): 426 - 428. [Abstract] [Full Text] [PDF]
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D. W. Wang, N. Makita, A. Kitabatake, J. R. Balser, and A. L. George Jr Enhanced Na+ Channel Intermediate Inactivation in Brugada Syndrome Circ. Res., October 13, 2000; 87 (8): e37 - e43. [Abstract] [Full Text] [PDF]
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S Viskin, R Fish, M Eldar, D Zeltser, M D Lesh, A Glick, and B Belhassen Prevalence of the Brugada sign in idiopathic ventricular fibrillation and healthy controls Heart, July 1, 2000; 84(1): 31 - 36. [Abstract] [Full Text] [PDF]
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W. G. Stevenson and H. T. Aretz Case 20-2000- A 61-Year-Old Man with a Wide-Complex Tachycardia N. Engl. J. Med., June 29, 2000; 342(26): 1979 - 1987. [Full Text] [PDF]
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C Wren, J J O'Sullivan, and C Wright Sudden death in children and adolescents Heart, April 1, 2000; 83(4): 410 - 413. [Abstract] [Full Text]
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R. Brugada, J. Brugada, C. Antzelevitch, G. E. Kirsch, D. Potenza, J. A. Towbin, and P. Brugada Sodium Channel Blockers Identify Risk for Sudden Death in Patients With ST-Segment Elevation and Right Bundle Branch Block but Structurally Normal Hearts Circulation, February 8, 2000; 101(5): 510 - 515. [Abstract] [Full Text] [PDF]
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J. Farre The Brugada syndrome: do we need more than the 12-lead ECG? Eur. Heart J., February 2, 2000; 21(4): 264 - 265. [PDF]
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P Brugada, R Brugada, and J Brugada Sudden death in patients and relatives with the syndrome of right bundle branch block, ST segment elevation in the precordial leads V1to V3and sudden death Eur. Heart J., February 2, 2000; 21(4): 321 - 326. [Abstract] [PDF]
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C. Bezzina, M. W. Veldkamp, M. P. van den Berg, A. V. Postma, M. B. Rook, J.-W. Viersma, I. M. van Langen, G. Tan-Sindhunata, M. Th. E. Bink-Boelkens, A. H. van der Hout, et al. A Single Na+ Channel Mutation Causing Both Long-QT and Brugada Syndromes Circ. Res., December 3, 1999; 85(12): 1206 - 1213. [Abstract] [Full Text] [PDF]
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J. D. Fisher, D. Krikler, and K. A. Hallidie-Smith Familial polymorphic ventricular arrhythmias: A quarter century of successful medical treatment based on serial exercise-pharmacologic testing J. Am. Coll. Cardiol., December 1, 1999; 34(7): 2015 - 2022. [Abstract] [Full Text] [PDF]
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M. B. Rook, C. Bezzina Alshinawi, W.A. Groenewegen, I. C. van Gelder, A. C.G. van Ginneken, H. J. Jongsma, M. M.A.M. Mannens, and A. A.M. Wilde Human SCN5A gene mutations alter cardiac sodium channel kinetics and are associated with the Brugada syndrome Cardiovasc Res, December 1, 1999; 44(3): 507 - 517. [Abstract] [Full Text] [PDF]
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R. Dumaine, J. A. Towbin, P. Brugada, M. Vatta, D. V. Nesterenko, V. V. Nesterenko, J. Brugada, R. Brugada, and C. Antzelevitch Ionic Mechanisms Responsible for the Electrocardiographic Phenotype of the Brugada Syndrome Are Temperature Dependent Circ. Res., October 29, 1999; 85(9): 803 - 809. [Abstract] [Full Text] [PDF]
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G.-X. Yan and C. Antzelevitch Cellular Basis for the Brugada Syndrome and Other Mechanisms of Arrhythmogenesis Associated With ST-Segment Elevation Circulation, October 12, 1999; 100(15): 1660 - 1666. [Abstract] [Full Text] [PDF]
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C. WREN Cardiac causes for syncope or sudden death in childhood Arch. Dis. Child., October 1, 1999; 81(4): 289 - 291. [Full Text]
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X. Jouven, M. Desnos, C. Guerot, and P. Ducimetiere Predicting Sudden Death in the Population : The Paris Prospective Study I Circulation, April 20, 1999; 99(15): 1978 - 1983. [Abstract] [Full Text] [PDF]
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K. Matsuo, T. Kurita, M. Inagaki, M. Kakishita, N. Aihara, W. Shimizu, A. Taguchi, K. Suyama, S. Kamakura, and K. Shimomura The circadian pattern of the development of ventricular fibrillation in patients with Brugada syndrome Eur. Heart J., March 2, 1999; 20(6): 465 - 470. [Abstract] [PDF]
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M. Alings and A. Wilde "Brugada" Syndrome : Clinical Data and Suggested Pathophysiological Mechanism Circulation, February 9, 1999; 99(5): 666 - 673. [Full Text] [PDF]
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I. Gussak, C. Antzelevitch, P. Bjerregaard, J. A. Towbin, and B. R. Chaitman The Brugada syndrome: clinical, electrophysiologic and genetic aspects J. Am. Coll. Cardiol., January 1, 1999; 33(1): 5 - 15. [Abstract] [Full Text] [PDF]
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J. Brugada, P. Brugada, and R. Brugada The syndrome of right bundle branch block ST segment elevation in V1 to V3 and sudden death--the Brugada syndrome Europace, January 1, 1999; 1(3): 156 - 166. [Abstract] [PDF]
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G. Baroudi, S. Acharfi, C. Larouche, and M. Chahine Expression and Intracellular Localization of an SCN5A Double Mutant R1232W/T1620M Implicated in Brugada Syndrome Circ. Res., January 11, 2002; 90 (1): e11 - e16. [Abstract] [Full Text] [PDF]
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