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 Towbin, G. Michael Vincent, Li Zhang

Research output: Contribution to journalArticle

54 Citations (Scopus)

Abstract

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 <.01), history of syncope (hazard ratio 6.1, P <.01), and gender were strongly associated with risk of ACA or LQTS-related death. Conclusion: Sudden death of a sibling prompted more aggressive treatment but did not predict risk of death or ACA, whereas QTc ≥ 0.53 s, gender, and syncope predicted this risk. All subjects should receive appropriate beta-blocker therapy. The decision to implant an ICD should be based on an individual's own risk characteristics (QTc, gender, and history of syncope).

Original languageEnglish (US)
Pages (from-to)831-836
Number of pages6
JournalHeart Rhythm
Volume5
Issue number6
DOIs
StatePublished - Jun 1 2008
Externally publishedYes

Fingerprint

Long QT Syndrome
Siblings
Syncope
Heart Arrest
Sudden Death
Implantable Defibrillators
Therapeutics
Registries
Parturition

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Kaufman, E. S., McNitt, S., Moss, A. J., Zareba, W., Robinson, J. L., Hall, W. J., ... Zhang, L. (2008). Risk of death in the long QT syndrome when a sibling has died. Heart Rhythm, 5(6), 831-836. https://doi.org/10.1016/j.hrthm.2008.02.029

Risk of death in the long QT syndrome when a sibling has died. / Kaufman, Elizabeth S.; McNitt, Scott; Moss, Arthur J.; Zareba, Wojciech; Robinson, Jennifer L.; Hall, W. Jackson; Ackerman, Michael J.; Benhorin, Jesaia; Locati, Emanuela T.; Napolitano, Carlo; Priori, Silvia G.; Schwartz, Peter J.; Towbin, Jeffrey; Vincent, G. Michael; Zhang, Li.

In: Heart Rhythm, Vol. 5, No. 6, 01.06.2008, p. 831-836.

Research output: Contribution to journalArticle

Kaufman, ES, McNitt, S, Moss, AJ, Zareba, W, Robinson, JL, Hall, WJ, Ackerman, MJ, Benhorin, J, Locati, ET, Napolitano, C, Priori, SG, Schwartz, PJ, Towbin, J, Vincent, GM & Zhang, L 2008, 'Risk of death in the long QT syndrome when a sibling has died', Heart Rhythm, vol. 5, no. 6, pp. 831-836. https://doi.org/10.1016/j.hrthm.2008.02.029
Kaufman ES, McNitt S, Moss AJ, Zareba W, Robinson JL, Hall WJ et al. Risk of death in the long QT syndrome when a sibling has died. Heart Rhythm. 2008 Jun 1;5(6):831-836. https://doi.org/10.1016/j.hrthm.2008.02.029
Kaufman, Elizabeth S. ; McNitt, Scott ; Moss, Arthur J. ; Zareba, Wojciech ; Robinson, Jennifer L. ; Hall, W. Jackson ; Ackerman, Michael J. ; Benhorin, Jesaia ; Locati, Emanuela T. ; Napolitano, Carlo ; Priori, Silvia G. ; Schwartz, Peter J. ; Towbin, Jeffrey ; Vincent, G. Michael ; Zhang, Li. / Risk of death in the long QT syndrome when a sibling has died. In: Heart Rhythm. 2008 ; Vol. 5, No. 6. pp. 831-836.
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abstract = "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 <.01), history of syncope (hazard ratio 6.1, P <.01), and gender were strongly associated with risk of ACA or LQTS-related death. Conclusion: Sudden death of a sibling prompted more aggressive treatment but did not predict risk of death or ACA, whereas QTc ≥ 0.53 s, gender, and syncope predicted this risk. All subjects should receive appropriate beta-blocker therapy. The decision to implant an ICD should be based on an individual's own risk characteristics (QTc, gender, and history of syncope).",
author = "Kaufman, {Elizabeth S.} and Scott McNitt and Moss, {Arthur J.} and Wojciech Zareba and Robinson, {Jennifer L.} and Hall, {W. Jackson} and Ackerman, {Michael J.} and Jesaia Benhorin and Locati, {Emanuela T.} and Carlo Napolitano and Priori, {Silvia G.} and Schwartz, {Peter J.} and Jeffrey Towbin and Vincent, {G. Michael} and Li Zhang",
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AU - Kaufman, Elizabeth S.

AU - McNitt, Scott

AU - Moss, Arthur J.

AU - Zareba, Wojciech

AU - Robinson, Jennifer L.

AU - Hall, W. Jackson

AU - Ackerman, Michael J.

AU - Benhorin, Jesaia

AU - Locati, Emanuela T.

AU - Napolitano, Carlo

AU - Priori, Silvia G.

AU - Schwartz, Peter J.

AU - Towbin, Jeffrey

AU - Vincent, G. Michael

AU - Zhang, Li

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N2 - 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 <.01), history of syncope (hazard ratio 6.1, P <.01), and gender were strongly associated with risk of ACA or LQTS-related death. Conclusion: Sudden death of a sibling prompted more aggressive treatment but did not predict risk of death or ACA, whereas QTc ≥ 0.53 s, gender, and syncope predicted this risk. All subjects should receive appropriate beta-blocker therapy. The decision to implant an ICD should be based on an individual's own risk characteristics (QTc, gender, and history of syncope).

AB - 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 <.01), history of syncope (hazard ratio 6.1, P <.01), and gender were strongly associated with risk of ACA or LQTS-related death. Conclusion: Sudden death of a sibling prompted more aggressive treatment but did not predict risk of death or ACA, whereas QTc ≥ 0.53 s, gender, and syncope predicted this risk. All subjects should receive appropriate beta-blocker therapy. The decision to implant an ICD should be based on an individual's own risk characteristics (QTc, gender, and history of syncope).

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