“Brugada Syndrome”: A Structural Cardiomyopathy or a Functional Electrical Disease?

  • P. Brugada
  • J. Brugada
  • R. Brugada


Five years ago we described a new syndrome characterised by an electrocardiographic pattern consisting of right bundle branch block with elevation of the ST segment in leads V1–V3 and sudden death caused by rapid polymorphic ventricular arrhythmias. None of the initially described patients had any demonstrable form of heart disease. Nowadays, thanks to international cooperation, we have obtained long-term information on a large cohort of patients with this syndrome. Data on 63 patients (57 males, mean age 38 ± 17 years) were analysed. Twenty-seven patients (43%) had a family history of sudden death. Extensive noninvasive and invasive examinations, including echocardiography, left and right ventricular angiography, heart biopsies, ergonovine tests, and nuclear magnetic resonance failed to show any form of structural heart abnormality. Findings during these tests were characteristically different from those in right ventricular dysplasia. The electrocardiographic response to ajmaline or procainamide and the transient normalisation of the electrocardiogram during follow-up were characteristic of this syndrome and not found in right ventricular dysplasia. Genetic testing in a large family with approximately 50% affected members excluded by linkage analysis the gene abnormalities so far described in right ventricular dysplasia. During follow-up up to 10 years, no patient developed or was suspected to develop any form of structural heart disease. In terms of prognosis, this syndrome is associated to a high recurrence of ventricular fibrillation in patients who have already suffered from one or multiple episodes of aborted sudden death. Most worrying is, however, that asymptomatic persons with this electrocardiographic pattern are also at high risk of sudden death. Amiodarone and β-blockers do not protect against sudden death. The only valuable therapy at present is an implantable cardioverter-defibrillator.


Sudden Death Ventricular Fibrillation Structural Heart Disease Brugada Syndrome Arrhythmic Event 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Brugada P, Brugada J (1992) Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. J Am Coll 20: 1391–1396CrossRefGoogle Scholar
  2. 2.
    Miyazaki T, Mitamura H, Miyoshi S, Soejima K, Aizawa Y, Ogawa S (1996) Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome. J Am Coll Cardiol 27: 1061–1070PubMedCrossRefGoogle Scholar
  3. 3.
    Proclemer A, Facchin D, Feruglio GA, Nucifora R (1993) Fibrillazione ventricolare recidivante, blocco di branca destra, persistente sopraslivellamento del tratto ST in V1–V3; una nuova sindrome aritmica? G Ital Cardiol 23: 1211–1218PubMedGoogle Scholar
  4. 4.
    Bjerregaard P, Gussak I, Kotar SL, Gessler JE, Janosik D (1994) Recurrent syncope in a patient with a prominent J wave. Am Heart J 127: 1426–1430CrossRefGoogle Scholar
  5. 5.
    Atarashi H, Ogawa S, Harumi K et al (1996) Characteristics of patients with right bundle branch block and ST segment elevation in right precordial leads. Am J Cardiol 78: 581–583PubMedCrossRefGoogle Scholar
  6. 6.
    Weyer EFD, Hauer RNW, Oomen A, Peters RHJ, Bakker PFA, Robles de Medina EO (1993) Unfavourable outcome in patients with primary electrical disease who survived an episode of ventricular fibrillation. Circulation 88: 1021–1029CrossRefGoogle Scholar
  7. 7.
    Brugada J, Brugada P (1997) Further characterisation of the syndrome of right bundle branch block, ST segment elevation, and sudden cardiac death. J Cardiovasc Electrophysiol 8: 325–331PubMedCrossRefGoogle Scholar
  8. 8.
    Corrado D, Nava A, Buja G et al (1996) Familial cardiomyopathy underlies syndrome of right bundle branch block, ST segment elevation and sudden cardiac death. J Am Coll Cardiol 27: 443–448PubMedCrossRefGoogle Scholar
  9. 9.
    Scheinmann MM (1997) Is the Brugada syndrome a distinct clinical entity? J Cardiovasc Electrophysiol 8: 332–334CrossRefGoogle Scholar
  10. 10.
    Rampazzo A, Nava A, Danieli GA et al (1994) The gene for arrhythmogenic right ventricular cardiomyopathy maps to chromosome 14q23-q24. Hum Mol Genet 3: 959–962PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 1998

Authors and Affiliations

  • P. Brugada
    • 1
  • J. Brugada
    • 2
  • R. Brugada
    • 3
  1. 1.The Cardiovascular Research and Teaching Institute AalstBelgium
  2. 2.Arrhythmia Unit, Hospital ClinicUniversity of BarcelonaSpain
  3. 3.Cardiology DepartmentBaylor College of MedicineHoustonUSA

Personalised recommendations