Brugada's syndrome

A lethal arrhythmia occurring during sleep, mainly in healthy men in their 4th decade. Both sporadic and familial cases have been reported and pedigree analysis suggests an autosomal dominant pattern of inheritance.

Martini et al in 1989 described six patients with apparently idiopathic ventricular fibrillation, 3 of whom had a distinctive ECG pattern characterized by an upsloping ST segment in the right precordial leads ('early repolarization') in association with right bundle branch block and T-wave inversion. In these patients, they documented subtle structural abnormalities of the right ventricle after detailed clinical investigation. Pedro and Josep Brugada in 1992 described 8 additional patients with the same ECG changes who experienced cardiac arrest due to ventricular fibrillation. They introduced the term 'right bundle branch block, ST segment elevation, and sudden death syndrome' to describe a seemingly new clinical entity.

• Bortolo Martini, A. Nava, G. Thiene, G. F. Buja, B. Canciani, R. Scognamiglio, L. Daliento, S. Dalla Volta:
  Ventricular fibrillation without apparent heart disease: description of six cases.
  American Heart Journal, St. Louis, December 1989, 118 (6): 1203-1209.
• P. Brugada, J. Brugada:
  A distinct clinical and electrocardiographic syndrome: right bundle branch block, persistent ST segment elevation with normal QT interval and sudden cardiac death.
  Pacing and Clinical Electrophysiology, Mount Kisco NY, 1991, 14: 746.
• P. Brugada, J. Brugada:
  Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report.
  Journal of the American College of Cardiology, November 15, 1992, 20: 1391-1396.
• J. Brugada. R. Brugada, P. Brugada:
  Right bundle branch block and persistent ST segment elevation in leads V1 through V3: a marker for sudden death in patients with demonstrable structural heart disease. Circulation, 1998, 97: 457-460.
• J. Brugada, P. Brugada, R. Brugada:
  The syndrome of right bundle branch block ST segment elevation in V1 to V3 and sudden death--the Brugada syndrome. Europace, London, 1999, 1: 156-166.
• P. Brugada, Peter Geelen, R. Brugada:
  Prognostic value of electrophysiological investigations in Brugada syndrome.
  Journal of Cardiovascular Electrophysiology, Mt. Kisco, NY, September 2001, 12: 1004-1007.
• J. Brugada, P. Brugada, R. Brugada:
  The Syndrome of Right Bundle Branch Block, ST segment Elevation in V1 to V3 and Sudden Death. Indian Pacing and Electrophysiology Journal, 2001, 1 (1): 6-11.
• J. Brugada, R. Brugada, P. Brugada:
  Brugada syndrome.
  Archives des maladies du coeur et des vaisseaux, Paris, 1999, 92: 847-850.