Risk of Fatal Arrhythmic Events in Long QT Syndrome Patients After Syncope

Christian Jons*, Arthur J. Moss, Ilan Goldenberg, Judy Liu, Scott McNitt, Wojciech Zareba, Ming Qi, Jennifer L. Robinson

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Objectives: The aim of this study was to identify risk factors for fatal arrhythmias in long QT syndrome (LQTS) patients presenting with syncope. Background: Syncope is highly predictive for future fatal arrhythmias in the LQTS. However, there are no data regarding risk stratification and management strategies in the high-risk subset of LQTS patients presenting with syncope. Methods: A total of 1,059 LQTS patients with a corrected QT interval ≥450 ms presenting with syncope as a first symptom were drawn from the International LQTS Registry. Cox proportional hazards regression was used to identify risk factors for a severe arrhythmic events comprising aborted cardiac arrest, appropriate implantable cardioverter-defibrillator therapy, and sudden cardiac death. Results: The lowest risk was found in patients with only 1 syncopal episode occurring before the start of beta-blocker therapy. In contrast, patients experiencing syncope after starting beta-blocker therapy had a 3.6-fold increase in the risk of severe arrhythmic events (p < 0.001) relative to this low-risk group and displayed a risk of severe arrhythmic events similar to that of patients not treated with beta-blockers. Multiple syncopal episodes occurring before initiation of beta-blocker therapy were associated with an intermediate risk (hazard ratio: 1.8, p < 0.001). The risk of syncope during beta-blocker therapy is high during childhood in both sexes but is higher in women than in men (hazard ratio: 2.3, p < 0.001). Conclusions: Patients with syncope during beta-blocker therapy are at high risk of life-threatening events, and implantable cardioverter-defibrillator therapy should be considered in these patients. The risk of beta-blocker failure is highest in young children and in women.

Original languageEnglish
Pages (from-to)783-788
Number of pages6
JournalJournal of the American College of Cardiology
Issue number8
StatePublished - 23 Feb 2010
Externally publishedYes


  • beta-blockers
  • long QT syndrome
  • sudden cardiac death
  • syncope


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