3型长QT综合征的临床方面:国际多中心研究
Arthur A M Wilde1, Arthur J Moss2, Elizabeth S Kaufman2
1From AMC Heart Centre, Department of Clinical and Experimental Cardiology (A.A.M.W., C.R.B.) and Department of Clinical Genetics (N.H., M.A.), Academic Medical Center, Amsterdam, The Netherlands; Cardiology Division of the Department of Medicine (A.J.M., C.L., W.Z., I.G., J.L.R., M.L.A., S.M., B.P.), the Department of Biostatistics (D.R.P.), and the Department of Pathology (M.Q.), University of Rochester School of Medicine and Dentistry, Rochester, NY; Heart and Vascular Research Center, MetroHealth Campus of Case Western Reserve University, Cleveland, OH (E.S.K.); Department of Cardiovascular Medicine, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan (W.S.); Department of Cardiology Bikur Cholim Hospital, Jerusalem, Israel (J.B.); Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH (J.A.T.); IRCCS Istituto Auxologico Italiano, Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Milan, Italy (C.P. L.C., P.J.S.); Department of Cardiology, Gentofte University Hospital, Copenhagen, Denmark (J.K.K.); Departments of Medicine, Pediatrics, and Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, MN (D.J.T., M.J.A.); Division of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine (W.S.) and Department of Preventive Cardiology (Y.M.), National Cerebral and Cardiovascular Center, Suita, Japan; Department of Molecular Medicine, University of Pavia, Italy (L.C.); Princess Al-Jawhara Al-Brahim Centre of Excellence in Research of Hereditary Disorders, Jeddah, Kingdom of Saudi Arabia (A.A.M.W.); and Department of Cardiovascular, Neural and Metabolic Sciences, San Luca Hospital IRCCS Istituto Auxologico Italiano, Milan, Italy (L.C., P.J.S.). a.a.wilde@amc.nl.
长QT综合征3型 (LQT3) 的风险分层确定了延长的QTc和作为关键风险. 在LQT3的女性中,β- 阻断剂治疗显著降低了心脏事件.
科学领域:
- 心脏病学
- 遗传学
- 药理学
背景情况:
- 在大量人群中缺乏长QT综合征3型 (LQT3) 的风险分层.
- 之前的研究没有评估LQT3患者的临床,遗传和治疗因素.
研究的目的:
- 研究LQT3患者心脏事件的危险因素.
- 在LQT3患者中评估β抑制剂治疗的有效性.
主要方法:
- 来自406名LQT3患者的临床,心电图和遗传数据的分析.
- 考克斯回归分析以确定心脏事件 (CE) 的预测因素.
- 在LQT3患者中对时间依赖的β- 阻断剂治疗的评估.
主要成果:
- 在391名LQT3患者中,有30%的患者经历过至少一次CE.
- 增加的QTc持续时间和先前的昏迷与较高的CE风险有关.
- 在女性中,β- 阻断剂治疗降低了CE的83% (P=0. 015),但在男性中没有明确的结果.
结论:
- 延长的QTc和昏迷是LQT3中危及生命的CE的重要危险因素.
- 在女性LQT3患者中,β抑制剂疗法有效降低了CE.
- 由于有限的事件,需要进一步研究以确定男性LQT3患者的β抑制剂疗效.
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