Current Cardiology Reports

, Volume 7, Issue 5, pp 349–356

Sudden cardiac death without structural heart disease: Update on the long QT and brugada syndromes

  • Ilan Goldenberg
  • Arthur J. Moss
  • Wojciech Zareba
Article

DOI: 10.1007/s11886-005-0088-1

Cite this article as:
Goldenberg, I., Moss, A.J. & Zareba, W. Curr Cardiol Rep (2005) 7: 349. doi:10.1007/s11886-005-0088-1

Abstract

The long QT syndrome (LQTS) and the Brugada syndrome (BrS) are the most common genetic causes of malignant ventricular arrhythmias and sudden cardiac death in young patients with normal cardiac morphology. To date, more than 250 different mutations in seven genes have been identified as causing LQTS, whereas the only gene identified to be linked to BrS is SCN5A. In both syndromes, genespecific mutations have been shown to be associated with specific phenotypic expressions. Risk stratification in LQTS and BrS is based mainly upon a constellation of electrocardiographic findings and a history of prior symptoms. In patients identified as high risk for arrhythmic mortality, the implantable cardioverter defibrillator is the most effective treatment and has been shown to provide near-complete protection during long-term follow-up.

Copyright information

© Current Science Inc 2005

Authors and Affiliations

  • Ilan Goldenberg
    • 1
  • Arthur J. Moss
  • Wojciech Zareba
  1. 1.Heart Research Follow-up ProgramUniversity of Rochester Medical CenterRochesterUSA

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