Department of Cardiorespiratory Sciences, Arrhythmologic Unit, Second University of Naples, Italy.
Kardiol Pol. 2012;70(11):1154-9.
The development of malignant ventricular arrhythmias is a possible feature in Emery-Dreifuss muscular dystrophy (EDMD) patients with normal left ventricular systolic function. This event may be the cause of sudden cardiac death in EDMD patients. QTc dispersion (QTc-D), JTc dispersion (JTc-D) and Tpeak-end dispersion (TDR) could reflect the physiological variability of regional and transmural ventricular repolarisation and could provide a substrate for life-threatening ventricular arrhythmias.
The current study was designed to evaluate the heterogeneity of ventricular repolarisation in EDMD patients.
Echocardiograms and electrocardiograms from 40 EDMD patients (age 20 ± 13) were evaluated and compared to those of 40 healthy age-matched controls.
The EDMD group, compared to the healthy control group, presented increased values of QTc-D (82.8 ± 44.1 vs. 53.3 ± 13.9, p = 0.003), JTc-D (73.6 ± 32.3 vs. 60.4 ± 11.1 ms, p = 0.001) and TDR (100.54 ± 19.06 vs. 92.15 ± 15.5 ms, p = 0.004). No correlation between QTc dispersion and ejection fraction (R = 0.2, p = 0.3) was found.
EDMD is associated with significantly increased regional and transmural heterogeneity of ventricular repolarisation, in the absence of impaired systolic and diastolic cardiac function.
恶性室性心律失常的发展是左心室收缩功能正常的 Emery-Dreifuss 肌营养不良（EDMD）患者的可能特征。这一事件可能是 EDMD 患者心源性猝死的原因。QTc 离散度（QTc-D）、JTc 离散度（JTc-D）和 T 波峰末离散度（TDR）可反映区域性和跨壁心室复极的生理变异性，并为危及生命的室性心律失常提供基质。
本研究旨在评估 EDMD 患者的心室复极异质性。
评估并比较 40 名 EDMD 患者（年龄 20±13 岁）和 40 名年龄匹配的健康对照组的超声心动图和心电图。
与健康对照组相比，EDMD 组的 QTc-D（82.8±44.1 比 53.3±13.9，p=0.003）、JTc-D（73.6±32.3 比 60.4±11.1 ms，p=0.001）和 TDR（100.54±19.06 比 92.15±15.5 ms，p=0.004）值增加。QTc 离散度与射血分数之间无相关性（R=0.2，p=0.3）。
EDMD 与区域性和跨壁心室复极的明显异质性相关，而收缩和舒张心功能不受影响。
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