Copyright © 2001 by the European Society of Cardiology.
Diagnosis and long-term follow-up of the Brugada syndrome in patients with idiopathic ventricular fibrillation
a Department of Cardiology, Heart-Lung Centre Utrecht, The Netherlands
b Experimental and Molecular Cardiology Group, Academic Medical Centre, University of Amsterdam, The Netherlands
revised July 3, 2000; accepted July 12, 2000
Abstract
Aims Some patients with idiopathic ventricular fibrillation may suffer from the Brugada syndrome. The diagnostic criteria for the Brugada syndrome are uncertain and arbitrarily set. Therefore, we studied the prevalence of the Brugada syndrome using various diagnostic criteria and long-term follow-up in 37 idiopathic ventricular fibrillation patients.
Methods and Results Idiopathic ventricular fibrillation was diagnosed after thorough clinical evaluation in 37 survivors of an out-of-hospital cardiac arrest referred to our institute (UMC Utrecht). Retrospectively, nine patients (24%, group I) were classified as potentially having the Brugada syndrome based on the presence of (in)complete right bundle branch block and ST-segment elevation in leads V1–V3of 
1mm. Only three patients (8%, group II) showed (in)complete right bundle branch block and 2mm ST-segment elevation. With the intermittent presence of these ECG features and/or their (re)appearance with class I antiarrhythmic drugs included as criteria, the percentage of the Brugada syndrome was attenuated in group I (2/37; 5%) and group II (1/37; 3%). Sixteen (43%) of all idiopathic ventricular fibrillation patients (mean follow-up 77±41 months) had a recurrent episode of syncope, ventricular tachyarrhythmias or sudden death. Recurrence rate was 3/9 (33%) in Brugada patients group I, 2/3 (66%) in group II and 13/28 (46%) in patients without the Brugada syndrome (P=ns).
Conclusions Depending on the diagnostic criteria used, the Brugada syndrome was observed in 3% to 24% of patients with idiopathic ventricular fibrillation, underlining the importance of defining the precise diagnostic criteria in these patients. For all idiopathic ventricular fibrillation patients, the ventricular tachyarrhythmia recurrence rate was substantial during an average follow-up of more than 6 years.
Key Words: Sudden cardiac death, idiopathic ventricular fibrillation, Brugada syndrome, follow-up
f1 Correspondence: C. A. Remme, MD, Laboratory of Experimental Cardiology, Academic Medical Centre, Room M0-052, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
References
- Wever EFD, Hauer RNW, Oomen A, Peters RHJ, Bakker PFA, Robles de Medina EO. Unfavourable outcome in patients with primary electrical disease who survived an episode of ventricular fibrillation. Circulation. 1993;88:1021–1029
[Abstract/Free Full Text] - Belhassen B, Viskin S. Idiopathic ventricular tachycardia and fibrillation. J Cardiovasc Electrophysiol. 1993;4:356–368[Web of Science][Medline]
- Tung RT, Shen WK, Hammill SC, Gersh BJ. Idiopathic ventricular fibrillation in out-of-hospital cardiac arrest survivors. PACE. 1994;17:1405–1412
- Priori SG, Borggrefe M, Camm AJ. Unexplained cardiac arrest: the need of a prospective registry. Eur Heart J. 1992;13:1445–1446
[Free Full Text] - Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol. 1992;20:1391–1396[Abstract]
- Chen Q, Kirsch GE, Zhang D. Genetic basis and molecular mechanism for idiopathic ventricular fibrillation. Nature. 1998;392:293–296[CrossRef][Medline]
- Alings AMW, Wilde AAM. ‘Brugada syndrome’. Clinical data and suggested pathophysiological mechanism. Circulation. 1999;99:666–673
[Free Full Text] - Atarashi H, Ogawa S, Harumi K. Characteristics of patients with right bundle branch block and ST-segment elevation in right precordial leads. Am J Cardiol. 1996;78:581–583[CrossRef][Web of Science][Medline]
- Antzelevitch C. The Brugada syndrome. J Cardiovasc Electrophysiol. 1998;9:513–516[Web of Science][Medline]
- Alshinawi C, Mannens M, Wilde A. Mutations in the human cardiac sodium channel gene (SCN5A) in patients with Brugada's syndrome (Abstr). Eur Heart J. 1998;19:78
- Rook MB, Bezzina Alshinawi C, Groenewegen WA. Human SCN5A gene mutations alter cardiac sodium channel kinetics and are associated with the Brugada syndrome. Cardiovasc Res. 1999;44:507–517
[Abstract/Free Full Text] - Denker S, Lehmann M, Mahmud R, Gilbert C, Akhtar M. Facilitation of ventricular tachycardia induction with abrupt changes in cycle length. Am J Cardiol. 1984;53:508–515[CrossRef][Web of Science][Medline]
- Circulation. 1997;95:265–272
[Abstract/Free Full Text] - Aizawa Y, Naitoh N, Washizuka T. Electrophysiological findings in idiopathic recurrent ventricular fibrillation: special reference to mode of induction, drug testing, and long-term outcomes. PACE. 1996;19:929–939
- Meissner MD, Lehmann MH, Steinman RT. Ventricular fibrillation in patients without significant structural heart disease: a multicentre experience with implantable cardioverter-defibrillator therapy. J Am Coll Cardiol. 1993;21:1406–1412[Abstract]
- J Am Coll Cardiol. 1998;31:1175–1209
[Free Full Text] - Brugada J, Brugada R, Brugada P. 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. Circulation. 1998;97:457–460
[Abstract/Free Full Text] - Brugada J, Brugada P. Further characterisation of the syndrome of right bundle branch block, ST segment elevation, and sudden cardiac death. J Cardiovasc Electrophysiol. 1997;8:325–331[Web of Science][Medline]
- Miyazaki T, Mitamura H, Miyoshi S, Soejima K, Aizawa Y, Ogawa S. Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome. J Am Coll Cardiol. 1996;27:1061–1070[Abstract]
- Brugada R, Brugada J, Antzelevitch C, Kirsch GE, Potenza D, Towbin JA, Brugada P. Sodium channel blockers identify risk for sudden death in patients with ST-segment elevation and right bundle branch block but structurally normal hearts. Circulation. 2000;101:510–515
[Abstract/Free Full Text] - Proiri SG, Napolitano C, Terrence L. Incomplete penetrance and variable response to sodium channel blockade in Brugada's syndrome (Abstr). Eur Heart J. 1999;20:465
[Abstract/Free Full Text]
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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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