High efficacy of β-blockers in long-QT syndrome type 1: Contribution of noncompliance and QT-prolonging drugs to the occurrence of β-blocker treatment "failures"

G. Michael Vincent, Peter J. Schwartz, Isabelle Denjoy, Heikki Swan, Candice Bithell, Carla Spazzolini, Lia Crotti, Kirsi Piippo, Jean Marc Lupoglazoff, Elizabeth Villain, Silvia G. Priori, Carlo Napolitano, Li Zhang

Research output: Contribution to journalArticle


Background - β-Blocker efficacy in long-QT syndrome type 1 is good but variably reported, and the causes of cardiac events despite β-blocker therapy have not been ascertained. Methods and Results - This was a retrospective study of the details surrounding cardiac events in 216 genotyped long-QT syndrome type 1 patients treated with β-blocker and followed up for a median time of 10 years. Before β-blocker, cardiac events occurred in 157 patients (73%) at a median age of 9 years, with cardiac arrest (CA) in 26 (12%). QT-prolonging drugs were used by 17 patients; 9 of 17 (53%) had CA compared with 17 of 199 nonusers (8.5%; odds ratio, 12.0; 95% confidence interval, 4.1 to 35.3; P

Original languageEnglish
Pages (from-to)215-221
Number of pages7
Issue number2
Publication statusPublished - Jan 20 2009



  • Arrhythmia
  • Death
  • Heart arrest
  • Long-QT syndrome
  • Sudden
  • Syncope
  • Torsade de pointes

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

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