| Trigger-Specific Ion-channel Mechanisms, Risk Factors, and Response to Therapy in Type 1 Long QT Syndrome. | |
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MedLine Citation:
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PMID: 21871251 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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BACKGROUND: Arrhythmic events in long-QT syndrome type-1 (LQT1) may be associated with exercise, acute arousal, or rest/sleep. OBJECTIVES: We aimed to identify trigger-specific risk factors for cardiac events in LQT1 patients. METHODS: The study population comprised 721 genetically-confirmed LQT1 patients from the LQTS Registry. Multivariate analysis was used to assess the independent contribution of pre-specified clinical and mutation-specific factors to the development of a first reported triggered event, associated with exercise, arousal, or sleep/rest. RESULTS: Cardiac events occurred in 221 study patients, of whom 121 (55%) were associated with exercise, 30 (14%) with arousal, 47 (21%) with sleep/rest, and 23 (10%) associated with other triggers. Multivariate analysis showed that males <13 years had a 2.8-fold (p<0.001) increase in the risk for exercise-triggered events, whereas females ≥13 years showed 3.5-fold (p=0.002) increase in the risk for sleep/rest non-arousal events. Cytoplasmic-loop mutations within the transmembrane region, involved in adrenergic channel regulation, were associated with increased risk for both exercise- and arousal- triggered events (HR=6.19 [p<0.001] and 4.99 [p<0.001], respectively), but were not associated with events during sleep/rest (HR=0.72, p=0.46). Beta-blocker therapy was associated with a pronounced 78% (p<0.001) reduction in the risk of exercise-triggered events, but did not have a significant effect on events associated with arousal or sleep/rest. CONCLUSIONS: In LQT1 patients, cardiac events triggered by exercise, arousal, or rest/sleep, are associated with distinctive risk factors and response to medical therapy. These findings can be used for improved recommendations for lifestyle modifications and therapeutic management in this population. |
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Authors:
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Ilan Goldenberg; Princy Thottathil; Coeli M Lopes; Arthur J Moss; Scott McNitt; Jin O-Uchi; Jennifer L Robinson; Wojciech Zareba; Michael J Ackerman; Elizabeth S Kaufman; Jeffrey A Towbin; Michael Vincent; Alon Barsheshet |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-8-23 |
Journal Detail:
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Title: Heart rhythm : the official journal of the Heart Rhythm Society Volume: - ISSN: 1556-3871 ISO Abbreviation: - Publication Date: 2011 Aug |
Date Detail:
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Created Date: 2011-8-29 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101200317 Medline TA: Heart Rhythm Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2011. Published by Elsevier Inc. |
Affiliation:
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Cardiology Division, University of Rochester Medical Center, Rochester, NY. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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