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
This article has been cited by other articles:
 |
|
 |
|
  M. A. Babaee Bigi, A. Aslani, and A. Aslani Significance of cardiac autonomic neuropathy in risk stratification of Brugada syndrome Europace, July 1, 2008; 10(7): 821 - 824. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Haissaguerre, N. Derval, F. Sacher, L. Jesel, I. Deisenhofer, L. de Roy, J.-L. Pasquie, A. Nogami, D. Babuty, S. Yli-Mayry, et al. Sudden Cardiac Arrest Associated with Early Repolarization N. Engl. J. Med., May 8, 2008; 358(19): 2016 - 2023. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Hayashi, S. Takatsuki, P. Maison-Blanche, A. Messali, A. Haggui, P. Milliez, A. Leenhardt, and F. Extramiana Ventricular Repolarization Restitution Properties in Patients Exhibiting Type 1 Brugada Electrocardiogram With and Without Inducible Ventricular Fibrillation J. Am. Coll. Cardiol., March 25, 2008; 51(12): 1162 - 1168. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. F. Kusano, M. Taniyama, K. Nakamura, D. Miura, K. Banba, S. Nagase, H. Morita, N. Nishii, A. Watanabe, T. Tada, et al. Atrial Fibrillation in Patients With Brugada Syndrome: Relationships of Gene Mutation, Electrophysiology, and Clinical Backgrounds J. Am. Coll. Cardiol., March 25, 2008; 51(12): 1169 - 1175. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Surber, S. Hensellek, D. Prochnau, G. S. Werner, K. Benndorf, H. R. Figulla, and T. Zimmer Combination of cardiac conduction disease and long QT syndrome caused by mutation T1620K in the cardiac sodium channel Cardiovasc Res, March 1, 2008; 77(4): 740 - 748. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Furushima, M. Chinushi, K. Okamura, K. Iijima, S. Komura, Y. Tanabe, S. Okada, D. Izumi, and Y. Aizawa Comparison of conduction delay in the right ventricular outflow tract between Brugada syndrome and right ventricular cardiomyopathy: investigation of signal average ECG in the precordial leads Europace, October 1, 2007; 9(10): 951 - 956. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Paul, J. Gerss, E. Schulze-Bahr, T. Wichter, C. Vahlhaus, A. A.M. Wilde, G. Breithardt, and L. Eckardt Role of programmed ventricular stimulation in patients with Brugada syndrome: a meta-analysis of worldwide published data Eur. Heart J., September 1, 2007; 28(17): 2126 - 2133. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Viskin and O. Rogowski Asymptomatic Brugada syndrome: a cardiac ticking time-bomb? Europace, September 1, 2007; 9(9): 707 - 710. [Full Text] [PDF]
|
|
|
|
 |
|
 E. A. Stephenson and C. I. Berul Electrophysiological Interventions for Inherited Arrhythmia Syndromes Circulation, August 28, 2007; 116(9): 1062 - 1080. [Full Text] [PDF]
|
|
|
|
|
|
S. Poelzing, C. Forleo, M. Samodell, L. Dudash, S. Sorrentino, M. Anaclerio, R. Troccoli, M. Iacoviello, R. Romito, P. Guida, et al. SCN5A Polymorphism Restores Trafficking of a Brugada Syndrome Mutation on a Separate Gene Circulation, August 1, 2006; 114(5): 368 - 376. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. A. Cesario and G. W. Dec Implantable Cardioverter- Defibrillator Therapy in Clinical Practice J. Am. Coll. Cardiol., April 18, 2006; 47(8): 1507 - 1517. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M C S Hall and D M Todd Modern management of arrhythmias Postgrad. Med. J., February 1, 2006; 82(964): 117 - 125. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. D. Krahn, M. Gollob, R. Yee, L. J. Gula, A. C. Skanes, B. D. Walker, and G. J. Klein Diagnosis of Unexplained Cardiac Arrest: Role of Adrenaline and Procainamide Infusion Circulation, October 11, 2005; 112(15): 2228 - 2234. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E Aksay, T Okan, and S Yanturali Brugada syndrome, manifested by propafenone induced ST segment elevation Emerg. Med. J., October 1, 2005; 22(10): 748 - 750. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S Chakrabarti and A G Stuart Understanding cardiac arrhythmias Arch. Dis. Child., October 1, 2005; 90(10): 1086 - 1090. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Haghjoo, A. Arya, Z. Emkanjoo, and M. A. Sadr-Ameli Clinical and Electrophysiologic Profile of Brugada Syndrome in Iranian Patients Asian Cardiovasc Thorac Ann, September 1, 2005; 13(3): 241 - 246. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. G. Meregalli, A. A.M. Wilde, and H. L. Tan Pathophysiological mechanisms of Brugada syndrome: Depolarization disorder, repolarization disorder, or more? Cardiovasc Res, August 15, 2005; 67(3): 367 - 378. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. C.M. Beaufort-Krol, M. P. van den Berg, A. A.M. Wilde, J. P. van Tintelen, J. W. Viersma, C. R. Bezzina, and M. Th.E. Bink-Boelkens Developmental Aspects of Long QT Syndrome Type 3 and Brugada Syndrome on the Basis of a Single SCN5A Mutation in Childhood J. Am. Coll. Cardiol., July 19, 2005; 46(2): 331 - 337. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Brugada, R. Brugada, J. Brugada, S. G. Priori, C. Napolitano, P. Brugada, R. Brugada, J. Brugada, S. G. Priori, and C. Napolitano Should patients with an asymptomatic Brugada electrocardiogram undergo pharmacological and electrophysiological testing? Circulation, July 12, 2005; 112(2): 279 - 292. [Full Text] [PDF]
|
|
|
|
 |
|
 M. Inamura, H. Okamoto, M. Kuroiwa, and S. Hoka General anesthesia for patients with Brugada syndrome. A report of six cases: [L'anesthesie generale chez des patients atteints du syndrome de Brugada. Presentation de six cas] Can J Anesth, April 1, 2005; 52(4): 409 - 412. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Antzelevitch, P. Brugada, M. Borggrefe, J. Brugada, R. Brugada, D. Corrado, I. Gussak, H. LeMarec, K. Nademanee, A. R. Perez Riera, et al. Brugada Syndrome: Report of the Second Consensus Conference: Endorsed by the Heart Rhythm Society and the European Heart Rhythm Association Circulation, February 8, 2005; 111(5): 659 - 670. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. E. Clancy and R. S. Kass Inherited and Acquired Vulnerability to Ventricular Arrhythmias: Cardiac Na+ and K+ Channels Physiol Rev, January 1, 2005; 85(1): 33 - 47. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. A. Pyles, J. Knapp, and and the Committee on Pediatric Emergency Medicine Role of Pediatricians in Advocating Life Support Training Courses for Parents and the Public Pediatrics, December 1, 2004; 114(6): e761 - e765. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J.-S. Hermida, I. Denjoy, J. Clerc, F. Extramiana, G. Jarry, P. Milliez, P. Guicheney, S. Di Fusco, J.-L. Rey, B. Cauchemez, et al. Hydroquinidine therapy in Brugada syndrome J. Am. Coll. Cardiol., May 19, 2004; 43(10): 1853 - 1860. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Brugada, R. Brugada, and P. Brugada Determinants of Sudden Cardiac Death in Individuals With the Electrocardiographic Pattern of Brugada Syndrome and No Previous Cardiac Arrest Circulation, December 23, 2003; 108(25): 3092 - 3096. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. Krittayaphong, G. Veerakul, K. Nademanee, and C. Kangkagate Heart rate variability in patients with Brugada syndrome in Thailand Eur. Heart J., October 1, 2003; 24(19): 1771 - 1778. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. Phillips, M. Priestley, A. R. Denniss, and J. B. Uther Brugada-Type Electrocardiographic Pattern Induced by Epidural Bupivacaine Anesth. Analg., July 1, 2003; 97(1): 264 - 267. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J Brugada, P Brugada, and R Brugada The ajmaline challenge in Brugada syndrome: A useful tool or misleading information? Eur. Heart J., June 2, 2003; 24(12): 1085 - 1086. [Full Text] [PDF]
|
|
|
|
|
|
C. Antzelevitch, P. Brugada, J. Brugada, R. Brugada, J. A. Towbin, and K. Nademanee Brugada syndrome: 1992-2002: A historical perspective J. Am. Coll. Cardiol., May 21, 2003; 41(10): 1665 - 1671. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Plunkett, J A Hulse, B Mishra, and J Gill Variable presentation of Brugada syndrome: lessons from three generations with syncope BMJ, May 15, 2003; 326(7398): 1078 - 1079. [Full Text] [PDF]
|
|
|
|
 |
|
 M Takagi, N Aihara, S Kuribayashi, A Taguchi, T Kurita, K Suyama, S Kamakura, and M Takamiya Abnormal response to sodium channel blockers in patients with Brugada syndrome: augmented localised wall motion abnormalities in the right ventricular outflow tract region detected by electron beam computed tomography Heart, February 1, 2003; 89(2): 169 - 174. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. Patruno, D. Pontillo, A. Achilli, G. Ruggeri, and G. Critelli Electrocardiographic pattern of Brugada syndrome disclosed by a febrile illness: clinical and therapeutic implications Europace, January 1, 2003; 5(3): 251 - 255. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. H. Dinckal, V. Davutoglu, I. Akdemir, S. Soydinc, A. Kirilmaz, and M. Aksoy Incessant monomorphic ventricular tachycardia during febrile illness in a patient with Brugada syndrome: fatal electrical storm Europace, January 1, 2003; 5(3): 257 - 261. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Moric, E. Herbert, M. Trusz-Gluza, A. Filipecki, U. Mazurek, and T. Wilczok The implications of genetic mutations in the sodium channel gene (SCN5A) Europace, January 1, 2003; 5(4): 325 - 334. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. M Morgan PATIENTS WITH VENTRICULAR ARRHYTHMIAS: WHO SHOULD BE REFERRED TO AN ELECTROPHYSIOLOGIST? Heart, December 1, 2002; 88(5): 544 - 550. [Full Text] [PDF]
|
|
|
|
|
|
T. Noda, W. Shimizu, A. Taguchi, K. Satomi, K. Suyama, T. Kurita, N. Aihara, and S. Kamakura ST-segment elevation and ventricular fibrillation without coronary spasm by intracoronary injection of acetylcholine and/or ergonovine maleate in patients with Brugada syndrome J. Am. Coll. Cardiol., November 20, 2002; 40(10): 1841 - 1847. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P.J. Schwartz, A. Garson Jr, T. Paul, M. Stramba-Badiale, V.L. Vetter, E. Villain, and C. Wren Guidelines for the interpretation of the neonatal electrocardiogram Eur. Heart J., September 1, 2002; 23(17): 1329 - 1344. [Full Text] [PDF]
|
|
|
|
|
|
L. Eckardt, P. Kirchhof, E. Schulze-Bahr, S. Rolf, M. Ribbing, P. Loh, H.-J. Bruns, A. Witte, P. Milberg, M. Borggrefe, et al. Electrophysiologic investigation in Brugada syndrome. Yield of programmed ventricular stimulation at two ventricular sites with up to three premature beats Eur. Heart J., September 1, 2002; 23(17): 1394 - 1401. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Kurita, W. Shimizu, M. Inagaki, K. Suyama, A. Taguchi, K. Satomi, N. Aihara, S. Kamakura, J. Kobayashi, and Y. Kosakai The electrophysiologic mechanism of ST-segment elevation in Brugada syndrome J. Am. Coll. Cardiol., July 17, 2002; 40(2): 330 - 334. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. P. P. Smits, L. Eckardt, V. Probst, C. R. Bezzina, J. J. Schott, C. A. Remme, W. Haverkamp, G.u. Breithardt, D. Escande, E. Schulze-Bahr, et al. Genotype-phenotype relationship in Brugada syndrome: electrocardiographic features differentiate SCN5A-related patients from non-SCN5A-related patients J. Am. Coll. Cardiol., July 17, 2002; 40(2): 350 - 356. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. Paydak, E. A. Telfer, R. F. Kehoe, P. Balasubramanian, L. Narra, M. Ezri, T. Zheutlin, P. Maheshwari, J. Nazari, and T. Wang Brugada Syndrome: An Unusual Cause of Convulsive Syncope Arch Intern Med, June 24, 2002; 162(12): 1416 - 1419. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Nagase, K. F. Kusano, H. Morita, Y. Fujimoto, M. Kakishita, K. Nakamura, T. Emori, H. Matsubara, and T. Ohe Epicardial electrogram of the right ventricular outflow tract in patients with the brugada syndrome: Using the epicardial lead J. Am. Coll. Cardiol., June 19, 2002; 39(12): 1992 - 1995. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. J. Callans Management of the Patient Who Has Been Resuscitated From Sudden Cardiac Death Circulation, June 11, 2002; 105(23): 2704 - 2707. [Full Text] [PDF]
|
|
|
|
|
|
M. Kanda, W. Shimizu, K. Matsuo, N. Nagaya, A. Taguchi, K. Suyama, T. Kurita, N. Aihara, and S. Kamakura Electrophysiologic characteristics andimplications of induced ventricular fibrillationin symptomatic patients with brugada syndrome J. Am. Coll. Cardiol., June 5, 2002; 39(11): 1799 - 1805. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Nademanee Prognostic value of electrophysiologic studies in brugada syndrome J. Am. Coll. Cardiol., June 5, 2002; 39(11): 1806 - 1807. [Full Text] [PDF]
|
|
|
|
|
|
H.-J. Bruns, L. Eckardt, C. Vahlhaus, E. Schulze-Bahr, W. Haverkamp, M. Borggrefe, G. Breithardt, and T. Wichter Body surface potential mapping in patients with Brugada syndrome: right precordial ST segment variations and reverse changes in left precordial leads Cardiovasc Res, April 1, 2002; 54(1): 58 - 66. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. G. Priori, C. Napolitano, M. Gasparini, C. Pappone, P. D. Bella, U. Giordano, R. Bloise, C. Giustetto, R. De Nardis, M. Grillo, et al. Natural History of Brugada Syndrome: Insights for Risk Stratification and Management Circulation, March 19, 2002; 105(11): 1342 - 1347. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. G. Priori, E. Aliot, C. Blomstrom-Lundqvist, L. Bossaert, G. Breithardt, P. Brugada, J. A. Camm, R. Cappato, S. M. Cobbe, C. Di Mario, et al. TASK FORCE ON SUDDEN CARDIAC DEATH, EUROPEAN SOCIETY OF CARDIOLOGY: Summary of Recommendations Europace, January 1, 2002; 4(1): 3 - 18. [Abstract] [PDF]
|
|
|
|
|
|
J. Brugada, R. Brugada, C. Antzelevitch, J. Towbin, K. Nademanee, and P. Brugada Long-Term Follow-Up of Individuals With the Electrocardiographic Pattern of Right Bundle-Branch Block and ST-Segment Elevation in Precordial Leads V1 to V3 Circulation, January 1, 2002; 105(1): 73 - 78. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. Brugada and R. Roberts Brugada Syndrome: Why Are There Multiple Answers to a Simple Question? Circulation, December 18, 2001; 104(25): 3017 - 3019. [Full Text] [PDF]
|
|
|
|
|
|
S H Wong, N T Mulvihill, M Norton, and R. HALL Assessing the risk of sudden cardiac death Heart, December 1, 2001; 86(6): 624 - 625. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Y. Miyasaka, H. Tsuji, K. Yamada, S. Tokunaga, D. Saito, Y. Imuro, N. Matsumoto, and T. Iwasaka Prevalence and mortality of the Brugada-type electrocardiogram in one city in Japan J. Am. Coll. Cardiol., September 1, 2001; 38(3): 771 - 774. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. Surawicz Brugada syndrome: manifest, concealed, "asymptomatic," suspected and simulated J. Am. Coll. Cardiol., September 1, 2001; 38(3): 775 - 777. [Full Text] [PDF]
|
|
|
|
|
|
S.G. Priori, E. Aliot, C. Blomstrom-Lundqvist, L. Bossaert, G. Breithardt, P. Brugada, A.J. Camm, R. Cappato, S.M. Cobbe, C. Di Mario, et al. Task Force on Sudden Cardiac Death of the European Society of Cardiology Eur. Heart J., August 2, 2001; 22(16): 1374 - 1450. [PDF]
|
|
|
|
|
|
M Furuhashi, K Uno, K Tsuchihashi, D Nagahara, M Hyakukoku, T Ohtomo, S Satoh, T Nishimiya, and K Shimamoto Prevalence of asymptomatic ST segment elevation in right precordial leads with right bundle branch block (Brugada-type ST shift) among the general Japanese population Heart, August 1, 2001; 86(2): 161 - 166. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Baroudi, V. Pouliot, I. Denjoy, P. Guicheney, A. Shrier, and M. Chahine Novel Mechanism for Brugada Syndrome : Defective Surface Localization of an SCN5A Mutant (R1432G) Circ. Res., June 22, 2001; 88 (12): e78 - e83. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
|