1型强直性肌营养不良患者的心率变异性随年龄增长和CTG重复长度增加而下降。
Heart rate variability declines with increasing age and CTG repeat length in patients with myotonic dystrophy type 1.
作者信息
Hardin Bradley A, Lowe Miriam R, Bhakta Deepak, Groh William J
机构信息
Krannert Institute of Cardiology, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA.
出版信息
Ann Noninvasive Electrocardiol. 2003 Jul;8(3):227-32. doi: 10.1046/j.1542-474x.2003.08310.x.
BACKGROUND
Cardiac myopathy manifesting as arrhythmias is common in the neurological disease, myotonic dystrophy type 1 (DM1). The purpose of the present study was to evaluate heart rate variability (HRV) in patients with DM1.
METHODS
In a multicenter study, history, ECG, and genetic testing were performed in DM1 patients.
RESULTS
In 289 patients in whom the diagnosis of DM1 was confirmed by a prolonged cytosine-thymine-guanine (CTG) repeat length the most common ambulatory ECG abnormality was frequent ventricular ectopy (16.3%). The 24-hour time domain parameters of SDNN (SD of the NN interval) and SDANN (SD of the mean NN, 5-minute interval) declined as age and CTG repeat length increased (SDNN: -8.5 ms per decade, 95% confidence intervals [CI]-12.9, -4.2, -8.7 ms per 500 CTG repeats, CI -15.7, -1.8, r=0.24, P<0.001; SDANN: -8.1 ms per decade, CI -12.4, -3.8, -8.8 ms per 500 CTG repeats, CI -15.7, -1.9, r=0.23, P<0.001). Short-term frequency domain parameters declined with age only (total power: -658 ms2 per decade, CI: -984, -331, r=0.23, P<0.001; low frequency (LF) power -287 ms2 per decade, CI: -397, -178, r=0.30, P<0.001; high frequency (HF) power: -267 ms2 per decade, CI: -386, -144, r=0.25, P<0.001). The LF/HF ratio increased as the patient aged (0.5 per decade, CI: 0.1, 0.9, r=0.13, P=0.03).
CONCLUSIONS
In DM1 patients a decline in HRV is observed as the patient ages and CTG repeat length increases.
背景
在神经疾病1型强直性肌营养不良症（DM1）中，表现为心律失常的心肌病很常见。本研究的目的是评估DM1患者的心率变异性（HRV）。
方法
在一项多中心研究中，对DM1患者进行了病史、心电图和基因检测。
结果
在289例通过延长的胞嘧啶 - 胸腺嘧啶 - 鸟嘌呤（CTG）重复长度确诊为DM1的患者中，最常见的动态心电图异常是频发室性早搏（16.3%）。随着年龄和CTG重复长度增加，24小时时域参数正常NN间期标准差（SDNN）和平均NN间期标准差（SDANN，5分钟间隔）下降（SDNN：每十年 -8.5毫秒，95%置信区间[CI] -12.9， -4.2；每500个CTG重复 -8.7毫秒，CI -15.7， -1.8，r = 0.24，P < 0.001；SDANN：每十年 -8.1毫秒，CI -12.4， -3.8；每500个CTG重复 -8.8毫秒，CI -15.7， -1.9，r = 0.23，P < 0.001）。短期频域参数仅随年龄下降（总功率：每十年 -658毫秒²，CI： -984， -331，r = 0.23，P < 0.001；低频（LF）功率每十年 -287毫秒²，CI： -397， -178，r = 0.30，P < 0.001；高频（HF）功率：每十年 -267毫秒²，CI： -386， -144，r = 0.25，P < 0.001）。随着患者年龄增长，LF/HF比值增加（每十年0.5，CI：0.1，0.9，r = 0.13，P = 0.03）。
结论
在DM1患者中，随着患者年龄增长和CTG重复长度增加，观察到HRV下降。
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