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Canadian Journal of Cardiology

From a Polish 3-Year-Old Boy Who Visited Maastricht to Automatic Detection Using Deep Learning: Brugada Syndrome Is Being Revolutionised

  • Hidde Bleijendaal
    Correspondence
    Corresponding author: Dr Hidde Bleijendaal or Dr Arthur A.M. Wilde, Heart Center, Department of Clinical Cardiology, Amsterdam Cardiovascular Sciences, Amsterdam University Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands. Tel.: +31205663072; fax: +31206971385.
    Affiliations
    Heart Center, Department of Clinical Cardiology, Amsterdam Cardiovascular Sciences, Amsterdam University Medical Center, University of Amsterdam, Amsterdam, The Netherlands
    Search for articles by this author
  • Arthur A.M. Wilde
    Affiliations
    Heart Center, Department of Clinical Cardiology, Amsterdam Cardiovascular Sciences, Amsterdam University Medical Center, University of Amsterdam, Amsterdam, The Netherlands
    Search for articles by this author
Published:September 23, 2021DOI:https://doi.org/10.1016/j.cjca.2021.09.016
      In 1992, Pedro and Josep Brugada described a typical electrocardiographic (ECG) pattern including right bundle branch block (RBBB) and persistent ST-segment elevation associated with sudden cardiac disease, without underlying electrolyte disturbances, ischemia, or structural heart disease. The syndrome, which later became widely known as the Brugada syndrome (BrS), first came to their attention in 1986 when a 3-year-old Polish boy named Lech and his father Andreas were referred to Professor Wellens in Maastricht, The Netherlands, owing to multiple episodes of syncope. On the ECG they observed the typical ECG pattern that later came to be associated with the syndrome. Despite the language barrier, they discovered that the boy had a sister who died at the age of 2 years, probably owing to cardiac arrest.
      • Brugada P.
      • Brugada J.
      Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report.
      ,
      • Brugada P.
      • Brugada J.
      • Roy D.
      Brugada syndrome 1992-2012: 20 years of scientific excitement, and more.
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