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Risk for Life-Threa...
Risk for Life-Threatening Cardiac Events in Patients With Genotype-Confirmed Long-QT Syndrome and Normal-Range Corrected QT Intervals
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Goldenberg, Ilan (author)
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Horr, Samuel (author)
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Moss, Arthur J. (author)
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Lopes, Coeli M. (author)
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Barsheshet, Alon (author)
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McNitt, Scott (author)
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Zareba, Wojciech (author)
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Andrews, Mark L. (author)
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Robinson, Jennifer L. (author)
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Locati, Emanuela H. (author)
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Ackerman, Michael J. (author)
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Benhorin, Jesaia (author)
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Kaufman, Elizabeth S. (author)
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Napolitano, Carlo (author)
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- Platonov, Pyotr (author)
- Lund University,Lunds universitet,Kardiologi,Sektion II,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Cardiology,Section II,Department of Clinical Sciences, Lund,Faculty of Medicine
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Priori, Silvia G. (author)
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Qi, Ming (author)
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Schwartz, Peter J. (author)
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Shimizu, Wataru (author)
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Towbin, Jeffrey A. (author)
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Vincent, G. Michael (author)
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Wilde, Arthur A. M. (author)
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Zhang, Li (author)
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(creator_code:org_t)
- Elsevier BV, 2010
- 2010
- English.
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In: Journal of the American College of Cardiology. - : Elsevier BV. - 0735-1097. ; 57:1, s. 51-59
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Abstract
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- Objectives This study was designed to assess the clinical course and to identify risk factors for life-threatening events in patients with long-QT syndrome (LQTS) with normal corrected QT (QTc) intervals. Background Current data regarding the outcome of patients with concealed LQTS are limited. Methods Clinical and genetic risk factors for aborted cardiac arrest (ACA) or sudden cardiac death (SCD) from birth through age 40 years were examined in 3,386 genotyped subjects from 7 multinational LQTS registries, categorized as LQTS with normal-range QTc (<= 440 ms [n = 469]), LQTS with prolonged QTc interval (>440 ms [ n = 1,392]), and unaffected family members (genotyped negative with <= 440 ms [ n = 1,525]). Results The cumulative probability of ACA or SCD in patients with LQTS with normal-range QTc intervals (4%) was significantly lower than in those with prolonged QTc intervals (15%) (p < 0.001) but higher than in unaffected family members (0.4%) (p < 0.001). Risk factors ACA or SCD in patients with normal-range QTc intervals included mutation characteristics (transmembrane-missense vs. nontransmembrane or nonmissense mutations: hazard ratio: 6.32; p = 0.006) and the LQTS genotypes (LQTS type 1: LQTS type 2, hazard ratio: 9.88; p = 0.03; LQTS type 3: LQTS type 2, hazard ratio: 8.04; p = 0.07), whereas clinical factors, including sex and QTc duration, were associated with a significant increase in the risk for ACA or SCD only in patients with prolonged QTc intervals (female age >13 years, hazard ratio: 1.90; p = 0.002; QTc duration, 8% risk increase per 10-ms increment; p = 0.002). Conclusions Genotype-confirmed patients with concealed LQTS make up about 25% of the at-risk LQTS population. Genetic data, including information regarding mutation characteristics and the LQTS genotype, identify increased risk for ACA or SCD in this overall lower risk LQTS subgroup. (J Am Coll Cardiol 2011;57:51-9) (C) 2011 by the American College of Cardiology Foundation
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Kardiologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cardiac and Cardiovascular Systems (hsv//eng)
Keyword
- sudden cardiac death
- long-QT syndrome
- corrected QT interval
Publication and Content Type
- art (subject category)
- ref (subject category)
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- By the author/editor
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Goldenberg, Ilan
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Horr, Samuel
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Moss, Arthur J.
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Lopes, Coeli M.
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Barsheshet, Alon
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McNitt, Scott
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show more...
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Zareba, Wojciech
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Andrews, Mark L.
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Robinson, Jennif ...
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Locati, Emanuela ...
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Ackerman, Michae ...
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Benhorin, Jesaia
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Kaufman, Elizabe ...
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Napolitano, Carl ...
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Platonov, Pyotr
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Priori, Silvia G ...
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Qi, Ming
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Schwartz, Peter ...
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Shimizu, Wataru
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Towbin, Jeffrey ...
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Vincent, G. Mich ...
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Wilde, Arthur A. ...
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Zhang, Li
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show less...
- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Cardiac and Card ...
- Articles in the publication
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Journal of the A ...
- By the university
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Lund University