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).
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).
Authors: J Kimbrough; A J Moss; W Zareba; J L Robinson; W J Hall; J Benhorin; E H Locati; A Medina; C Napolitano; S Priori; P J Schwartz; K Timothy; J A Towbin; G M Vincent; L Zhang Journal: Circulation Date: 2001-07-31 Impact factor: 29.690
Authors: Wojciech Zareba; Arthur J Moss; Emanuela H Locati; Michael H Lehmann; Derick R Peterson; W Jackson Hall; Peter J Schwartz; G Michael Vincent; Silvia G Priori; Jesaia Benhorin; Jeffrey A Towbin; Jennifer L Robinson; Mark L Andrews; Carlo Napolitano; Katherine Timothy; Li Zhang; Aharon Medina Journal: J Am Coll Cardiol Date: 2003-07-02 Impact factor: 24.094
Authors: A J Moss; P J Schwartz; R S Crampton; D Tzivoni; E H Locati; J MacCluer; W J Hall; L Weitkamp; G M Vincent; A Garson Journal: Circulation Date: 1991-09 Impact factor: 29.690
Authors: Peter J Schwartz; Silvia G Priori; Marina Cerrone; Carla Spazzolini; Attilio Odero; Carlo Napolitano; Raffaella Bloise; Gaetano M De Ferrari; Catherine Klersy; Arthur J Moss; Wojciech Zareba; Jennifer L Robinson; W Jackson Hall; Paul A Brink; Lauri Toivonen; Andrew E Epstein; Cuilan Li; Dayi Hu Journal: Circulation Date: 2004-03-29 Impact factor: 29.690
Authors: Wojciech Zareba; Arthur J Moss; James P Daubert; W Jackson Hall; Jennifer L Robinson; Mark Andrews Journal: J Cardiovasc Electrophysiol Date: 2003-04
Authors: Ilan Goldenberg; Samuel Horr; Arthur J Moss; Coeli M Lopes; Alon Barsheshet; Scott McNitt; Wojciech Zareba; Mark L Andrews; Jennifer L Robinson; Emanuela H Locati; Michael J Ackerman; Jesaia Benhorin; Elizabeth S Kaufman; Carlo Napolitano; Pyotr G Platonov; Silvia G Priori; Ming Qi; Peter J Schwartz; Wataru Shimizu; Jeffrey A Towbin; G Michael Vincent; Arthur A M Wilde; Li Zhang Journal: J Am Coll Cardiol Date: 2011-01-04 Impact factor: 24.094
Authors: Marilene Pavan; Viviane F Ruiz; Fábio A Silva; Tiago J Sobreira; Roberta M Cravo; Michelle Vasconcelos; Lívia P Marques; Sonia M F Mesquita; José E Krieger; Antônio A B Lopes; Paulo S Oliveira; Alexandre C Pereira; José Xavier-Neto Journal: BMC Med Genet Date: 2009-11-03 Impact factor: 2.103