New echocardiographic insights in short QT syndrome: More than a channelopathy?
New echocardiographic insights in short QT syndrome: More than a channelopathy?
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DOI：
10.1016/j.hrthm.2015.05.024
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发表时间：
2015-10-01
期刊：
影响因子：
5.5
通讯作者：
Gaita, Fiorenzo
中科院分区：
文献类型：
--
作者：
Frea, Simone;Giustetto, Carla;Gaita, Fiorenzo
短QT综合征（SQTS）是一种先天性离子通道疾病，其特征是心源性猝死风险增加。很少有人知道的可能性，加速复极改变机械功能在SQTS. EQUITIVES研究的存在下，左心室功能障碍和机械分散，评估组织多普勒成像（TDI）和斑点跟踪超声心动图（STE），其相关性与QT间期和genetics.METHODS十五SOTS患者（7与HERG和3与KCNQ 1突变）进行了研究。将心电图和超声心动图参数与年龄和性别匹配的健康对照组进行比较。结果与对照组相比，SQTS患者显示左心室收缩减少（整体纵向应变：-16.0% +/- 3.4%对-22.6% +/-1.7%，P < .001;心肌功能指数0.59 ± 0.17 vs 0.34 ± 0.08，P <0.001），射血分数发生率较高
BACKGROUND Short QT syndrome (SQTS) is a congenital ion channel disease characterized by an increased risk of sudden cardiac death. Little is known about the possibility that accelerated repolarization alters mechanical function in SQTS.OBJECTIVES The study investigated the presence of left ventricular dysfunction and mechanical dispersion, assessed by tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE), and their correlation with QT interval duration and genetics.METHODS Fifteen SOTS patients (7 with HERG and 3 with KCNQ1 mutation) were studied. Electrocardiographic and echocardiographic parameters were compared with age- and sex-matched healthy controls.RESULTS When compared to the control group, SQTS patients showed reduced left ventricular contraction (global longitudinal strain: -16.0% +/- 3.4% vs -22.6% +/- 1.7%, P < .001; myocardial performance index 0.59 +/- 0.17 vs 0.34 +/- 0.08, P < .001) and a higher incidence of ejection fraction