Congenital long QT syndromes and Brugada syndrome: The arrhythmogenic ion channel disorders

J. Nemec, W. K. Shen

Research output: Contribution to journalReview articlepeer-review

3 Scopus citations


Congenital long QT syndromes (LQTS) and Brugada syndrome are hereditary disorders of cardiac ion channels which result in life-threatening cardiac arrhythmias or sudden cardiac death in patients with anatomically normal hearts. The pathogenesis of these dramatic events has been partially elucidated with the identification of the individual ion channels involved and understanding of the effect of some disease-causing mutations on the membrane currents and action potential. The clinical spectrum of congenital LQTS is broader than previously thought and involves certain patients previously diagnosed with idiosyncratic drug-induced proarrhythmia. The initial treatment for congenital LQTS patients involves β-blockers in most cases. Indications for implantable cardioverter-defibrillator (ICD) or pace-maker (PM) implantation in selected individuals continue to evolve.

Original languageEnglish (US)
Pages (from-to)773-797
Number of pages25
JournalExpert Opinion on Pharmacotherapy
Issue number5
StatePublished - Aug 27 2001


  • Brugada syndrome
  • Implantable cardioverter-defibrillator
  • Long QT syndrome
  • Torsade de pointes
  • l
  • l
  • l
  • β-Blockers

ASJC Scopus subject areas

  • Pharmacology
  • Pharmacology (medical)


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