Risk of death in the long QT syndrome when a sibling has died

Elizabeth S. Kaufman, Scott McNitt, Arthur J. Moss, Wojciech Zareba, Jennifer L. Robinson, W. Jackson Hall, Michael J. Ackerman, Jesaia Benhorin, Emanuela T. Locati, Carlo Napolitano, Silvia G. Priori, Peter J. Schwartz, Jeffrey A. Towbin, G. Michael Vincent, Li Zhang

Research output: Contribution to journalArticlepeer-review


Background: Sudden death of a sibling is thought to be associated with greater risk of death in long QT syndrome (LQTS). However, there is no evidence of such an association. Objective: This study sought to test the hypothesis that sudden death of a sibling is a risk factor for death or aborted cardiac arrest (ACA) in patients with LQTS. Methods: We examined all probands and first-degree and second-degree relatives in the International Long QT Registry from birth to age 40 years with QTc ≥ 0.45 s. Covariates included sibling death, QTc, gender by age, syncope, and implantable cardioverter-defibrillator (ICD) and beta-blocker treatment. End points were (1) severe events (ACA, LQTS-related death) and (2) any cardiac event (syncope, ACA, or LQTS-related death). Results: Of 1915 subjects, 270 had a sibling who died. There were 213 severe events and 829 total cardiac events. More subjects with history of sibling death received beta-blocker therapy. Sibling death was not significantly associated with risk of ACA or LQTS-related death, but was associated with increased risk of syncope. QTc ≥ 0.53 s (hazard ratio 2.5, P

Original languageEnglish
Pages (from-to)831-836
Number of pages6
JournalHeart Rhythm
Issue number6
Publication statusPublished - Jun 2008


  • Long QT syndrome
  • Risk stratification
  • Sudden cardiac death
  • Syncope
  • Torsades

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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