TY - JOUR
T1 - Risk factors for recurrent syncope and subsequent fatal or near-fatal events in children and adolescents with long QT syndrome
AU - Liu, Judy F.
AU - Jons, Christian
AU - Moss, Arthur J.
AU - McNitt, Scott
AU - Peterson, Derick R.
AU - Qi, Ming
AU - Zareba, Wojciech
AU - Robinson, Jennifer L.
AU - Barsheshet, Alon
AU - Ackerman, Michael J.
AU - Benhorin, Jesaia
AU - Kaufman, Elizabeth S.
AU - Locati, Emanuela H.
AU - Napolitano, Carlo
AU - Priori, Silvia G.
AU - Schwartz, Peter J.
AU - Towbin, Jeffrey
AU - Vincent, Michael
AU - Zhang, Li
AU - Goldenberg, Ilan
N1 - Funding Information:
This work was supported in part by research grants HL-33843 and HL-51618 from the National Institutes of Health , Bethesda, Maryland, and by an unrestricted grant from BioReference Laboratories, Inc. , Elmwood Park, New Jersey. Dr. Moss has received a research grant from Bioreference Labs . Dr. Ackerman is a consultant for Biotronik, Boston Scientific, Medtronic, PGx Health, and St. Jude Medical; and has intellectual property in PGx Health. Dr. Kaufman receives grant support from CardioDx , Cambridge Heart Inc. , and St. Jude Medical . Dr. Locati is a consultant for Sorin. All other authors have reported that they have no relationships to disclose.
PY - 2011/2/22
Y1 - 2011/2/22
N2 - Objectives: We aimed to identify risk factors for recurrent syncope in children and adolescents with congenital long QT syndrome (LQTS). Background: Data regarding risk assessment in LQTS after the occurrence of the first syncope episode are limited. Methods: The Prentice-Williams-Peterson conditional gap time model was used to identify risk factors for recurrent syncope from birth through age 20 years among 1,648 patients from the International Long QT Syndrome Registry. Results: Multivariate analysis demonstrated that corrected QT interval (QTc) duration (<500 ms) was a significant predictor of a first syncope episode (hazard ratio: 2.16), whereas QTc effect was attenuated when the end points of the second, third, and fourth syncope episodes were evaluated (hazard ratios: 1.29, 0.99, 0.90, respectively; p < 0.001 for the null hypothesis that all 4 hazard ratios are identical). A genotype-specific subanalysis showed that during childhood (0 to 12 years), males with LQTS type 1 had the highest rate of a first syncope episode (p = 0.001) but exhibited similar rates of subsequent events as other genotype-sex subsets (p = 0.63). In contrast, in the age range of 13 to 20 years, long QT syndrome type 2 females experienced the highest rate of both first and subsequent syncope events (p < 0.001 and p = 0.01, respectively). Patients who experienced <1 episodes of syncope had a 6- to 12-fold (p < 0.001 for all) increase in the risk of subsequent fatal/near-fatal events independently of QTc duration. Beta-blocker therapy was associated with a significant reduction in the risk of recurrent syncope and subsequent fatal/near-fatal events. Conclusions: Children and adolescents who present after an episode of syncope should be considered to be at a high risk of the development of subsequent syncope episodes and fatal/near-fatal events regardless of QTc duration.
AB - Objectives: We aimed to identify risk factors for recurrent syncope in children and adolescents with congenital long QT syndrome (LQTS). Background: Data regarding risk assessment in LQTS after the occurrence of the first syncope episode are limited. Methods: The Prentice-Williams-Peterson conditional gap time model was used to identify risk factors for recurrent syncope from birth through age 20 years among 1,648 patients from the International Long QT Syndrome Registry. Results: Multivariate analysis demonstrated that corrected QT interval (QTc) duration (<500 ms) was a significant predictor of a first syncope episode (hazard ratio: 2.16), whereas QTc effect was attenuated when the end points of the second, third, and fourth syncope episodes were evaluated (hazard ratios: 1.29, 0.99, 0.90, respectively; p < 0.001 for the null hypothesis that all 4 hazard ratios are identical). A genotype-specific subanalysis showed that during childhood (0 to 12 years), males with LQTS type 1 had the highest rate of a first syncope episode (p = 0.001) but exhibited similar rates of subsequent events as other genotype-sex subsets (p = 0.63). In contrast, in the age range of 13 to 20 years, long QT syndrome type 2 females experienced the highest rate of both first and subsequent syncope events (p < 0.001 and p = 0.01, respectively). Patients who experienced <1 episodes of syncope had a 6- to 12-fold (p < 0.001 for all) increase in the risk of subsequent fatal/near-fatal events independently of QTc duration. Beta-blocker therapy was associated with a significant reduction in the risk of recurrent syncope and subsequent fatal/near-fatal events. Conclusions: Children and adolescents who present after an episode of syncope should be considered to be at a high risk of the development of subsequent syncope episodes and fatal/near-fatal events regardless of QTc duration.
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U2 - 10.1016/j.jacc.2010.10.025
DO - 10.1016/j.jacc.2010.10.025
M3 - Article
C2 - 21329841
AN - SCOPUS:79951588052
SN - 0735-1097
VL - 57
SP - 941
EP - 950
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
IS - 8
ER -