Risk factors for recurrent syncope and subsequent fatal or near-fatal events in children and adolescents with long QT syndrome
Open accessAbstract
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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The annual rate of sudden cardiac death in patients with untreated long QT syndrome is around 0.9% overall and 5% for those with syncope.
“The annual rate of SCD in patients with untreated LQTS is around 0.9% overall and 5% for those with syncope. 352,364”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [352]
The Results section (page unavailable) supports the general point that syncope predicts greater risk of a subsequent fatal or near-fatal event. It reports five-year probabilities for the combined outcome of aborted cardiac arrest or sudden cardiac death, not annual sudden-death rates of 0.9% overall or 5% after syncope in untreated patients. The Results also note that at least 88% of patients who experienced syncope used beta-blockers. (LLM, conf=0.84)
Experimental — accuracy not yet measuredSyncopal events in long QT syndrome are associated with an increased risk of subsequent cardiac arrest.
“Syncopal events in long QT syndrome (LQTS) are associated with an increased risk of subsequent cardiac arrest.”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [352]
The Results section reports that syncopal episodes in children and adolescents with LQTS were associated with increased risk of subsequent aborted cardiac arrest or sudden cardiac death. The claim omits the study’s age restriction and describes cardiac arrest alone, whereas the reported risk was for a combined outcome. (LLM, conf=0.87)
Experimental — accuracy not yet measuredA history of syncope in patients with structural heart disease or inheritable arrhythmia syndromes is associated with a two- to four-fold increased risk of death, but this varies between specific conditions.
“In general, a history of syncope in patients with structural heart disease or inheritable arrhythmia syndromes is associated with a two- to four-fold increased risk of death, 348,350-353 but varies between specific conditions. 354-356”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [352]
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- Authors
- Liu, J. F., Jons, C., Moss, A. J., McNitt, S., Peterson, D. R., Qi, M., Zareba, W., Robinson, J. L., Barsheshet, A., Ackerman, M. J., Benhorin, J., Kaufman, E. S., Locati, E. H., Napolitano, C., Priori, S. G., Schwartz, P. J., Towbin, J., Vincent, M., Zhang, L., & Goldenberg, I.
- Affiliations
- University of Rochester Medicine (US)
- Mayo Clinic (US)
- Tel Aviv Sourasky Medical Center (IL)
- Case Western Reserve University (US)
- Azienda Socio Sanitaria Territoriale Grande Ospedale Metropolitano Niguarda (IT)
- University of Pavia (IT)
- NYU Langone Health (US)
- Cincinnati Children's Hospital Medical Center (US)
- University of Utah (US)
- Main Line Health (US)
- Corresponding author
- Goldenberg, Ilan
- ORCID
- Venue
- Journal of the American College of Cardiology
- Publisher
- Elsevier BV
- Published
- 2011-02
- Volume
- 57
- Issue
- 8
- Pages
- 941-950
- ISSN
- 0735-1097 (ISSN-L)
- PMC
- PMC3052409
- OpenAlex
- W2075417967
- Cited by
- 126
- References
- 20
- Citations by year
- 2026: 2 · 2025: 2 · 2024: 5 · 2023: 5 · 2022: 9 · 2021: 10 · 2020: 2 · 2019: 5 · 2018: 12 · 2017: 12 · 2016: 11 · 2015: 11 · 2014: 9 · 2013: 14 · 2012: 9
- FWCI
- 8.55
- Type
- journal article
- Language
- en
- Update notices
- erratum (2011-04-19)
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of the American College of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institutes of Health (HL-33843, HL-51618)
- BioReference Laboratories, Inc.
- Bioreference Labs
- CardioDx
- Cambridge Heart Inc.
- St. Jude Medical
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Health Sciences › Medicine › Surgery › Cardiovascular Syncope and Autonomic Disorders
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
- Keywords
- long QT syndrome, recurrent syncope, risk factors, QTc interval, LQT1, long QT syndrome type 2, sex differences, beta-blocker therapy, children and adolescents
- MeSH terms
- Adolescent, Cause of Death, Child, Electrocardiography, Female, Humans, Long QT Syndrome, Male, Prognosis, Recurrence, Registries, Severity of Illness Index, Syncope, Cohort Studies, Incidence, Multivariate Analysis, Proportional Hazards Models, Death, Sudden, Cardiac, Age Distribution, Sex Distribution, Risk Assessment, Kaplan-Meier Estimate
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