OBJECTIVES: The aim of this study was to identify risk factors for fatal arrhythmias in long QT syndrome (LQTS) patients presenting with syncope. BACKGROUND: Syncope is highly predictive for future fatal arrhythmias in the LQTS. However, there are no data regarding risk stratification and management strategies in the high-risk subset of LQTS patients presenting with syncope. METHODS: A total of 1,059 LQTS patients with a corrected QT interval > or =450 ms presenting with syncope as a first symptom were drawn from the International LQTS Registry. Cox proportional hazards regression was used to identify risk factors for a severe arrhythmic events comprising aborted cardiac arrest, appropriate implantable cardioverter-defibrillator therapy, and sudden cardiac death. RESULTS: The lowest risk was found in patients with only 1 syncopal episode occurring before the start of beta-blocker therapy. In contrast, patients experiencing syncope after starting beta-blocker therapy had a 3.6-fold increase in the risk of severe arrhythmic events (p < 0.001) relative to this low-risk group and displayed a risk of severe arrhythmic events similar to that of patients not treated with beta-blockers. Multiple syncopal episodes occurring before initiation of beta-blocker therapy were associated with an intermediate risk (hazard ratio: 1.8, p < 0.001). The risk of syncope during beta-blocker therapy is high during childhood in both sexes but is higher in women than in men (hazard ratio: 2.3, p < 0.001). CONCLUSIONS: Patients with syncope during beta-blocker therapy are at high risk of life-threatening events, and implantable cardioverter-defibrillator therapy should be considered in these patients. The risk of beta-blocker failure is highest in young children and in women. Copyright (c) 2010 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
OBJECTIVES: The aim of this study was to identify risk factors for fatal arrhythmias in long QT syndrome (LQTS) patients presenting with syncope. BACKGROUND:Syncope is highly predictive for future fatal arrhythmias in the LQTS. However, there are no data regarding risk stratification and management strategies in the high-risk subset of LQTS patients presenting with syncope. METHODS: A total of 1,059 LQTS patients with a corrected QT interval > or =450 ms presenting with syncope as a first symptom were drawn from the International LQTS Registry. Cox proportional hazards regression was used to identify risk factors for a severe arrhythmic events comprising aborted cardiac arrest, appropriate implantable cardioverter-defibrillator therapy, and sudden cardiac death. RESULTS: The lowest risk was found in patients with only 1 syncopal episode occurring before the start of beta-blocker therapy. In contrast, patients experiencing syncope after starting beta-blocker therapy had a 3.6-fold increase in the risk of severe arrhythmic events (p < 0.001) relative to this low-risk group and displayed a risk of severe arrhythmic events similar to that of patients not treated with beta-blockers. Multiple syncopal episodes occurring before initiation of beta-blocker therapy were associated with an intermediate risk (hazard ratio: 1.8, p < 0.001). The risk of syncope during beta-blocker therapy is high during childhood in both sexes but is higher in women than in men (hazard ratio: 2.3, p < 0.001). CONCLUSIONS:Patients with syncope during beta-blocker therapy are at high risk of life-threatening events, and implantable cardioverter-defibrillator therapy should be considered in these patients. The risk of beta-blocker failure is highest in young children and in women. Copyright (c) 2010 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
Authors: Jared W Magnani; Eiran Z Gorodeski; Victor M Johnson; Lisa M Sullivan; Naomi M Hamburg; Emelia J Benjamin; Patrick T Ellinor Journal: Heart Rhythm Date: 2010-09-22 Impact factor: 6.343
Authors: Mark S Link; Derek V Exner; Mark Anderson; Michael Ackerman; Amin Al-Ahmad; Bradley P Knight; Steven M Markowitz; Elizabeth S Kaufman; David Haines; Samuel J Asirvatham; David J Callans; J Paul Mounsey; Frank Bogun; Sanjiv M Narayan; Andrew D Krahn; Suneet Mittal; Jagmeet Singh; John D Fisher; Sumeet S Chugh Journal: Heart Rhythm Date: 2011-06-14 Impact factor: 6.343
Authors: Judy F Liu; Christian Jons; Arthur J Moss; Scott McNitt; Derick R Peterson; Ming Qi; Wojciech Zareba; Jennifer L Robinson; Alon Barsheshet; Michael J Ackerman; Jesaia Benhorin; Elizabeth S Kaufman; Emanuela H Locati; Carlo Napolitano; Silvia G Priori; Peter J Schwartz; Jeffrey Towbin; Michael Vincent; Li Zhang; Ilan Goldenberg Journal: J Am Coll Cardiol Date: 2011-02-22 Impact factor: 24.094
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