[斯坦纳特肌强直性营养不良症中的心脏受累情况]
[Cardiac involvement in Steinert myotonic dystrophy].
作者信息
Miladi M I, Charfeddine H, Feki I, Turki E, Elleuch N, Trabelsi I, Krichène S, Kammoun S, Mhiri C
机构信息
Service de neurologie, CHU Habib Bourguiba, route El Ain km 0,5, 3029 Sfax, Tunisia.
出版信息
Rev Med Interne. 2009 Jul;30(7):573-7. doi: 10.1016/j.revmed.2009.01.017. Epub 2009 Mar 14.
INTRODUCTION
Myotonic dystrophy type 1 (DM1) is an autosomal dominant multisystemic disease with frequent cardiac involvement that may cause sudden death. This study was performed to determine the various cardiac manifestations in DM1, their frequency and the relevance of cardiac electrophysiological study in this disease.
METHODS
Ten patients with DM1, five men and five women, mean age 44.3+/-7.8 years underwent neurological and cardiac assessments.
RESULTS
The most frequent electrocardiographic findings were conduction abnormalities, essentially by intraventricular conduction defects (eight out of ten cases) such as bundle branch or fascicular blocks. Echocardiography showed alterations in systolic left ventricular function in two cases. Invasive electrophysiology testing showed sub-hisien block in three patients, requiring cardiac pacemaker implantation. These three patients had normal duration of PR interval and normal width of QRS complex.
CONCLUSIONS
We recommend that all patients with DM1 should undergo cardiac investigation to detect subclinical cardiac involvement.
引言
1型强直性肌营养不良症（DM1）是一种常染色体显性多系统疾病，常累及心脏，可导致猝死。本研究旨在确定DM1患者的各种心脏表现、其发生率以及心脏电生理检查在该疾病中的相关性。
方法
10例DM1患者，5男5女，平均年龄44.3±7.8岁，接受了神经学和心脏评估。
结果
最常见的心电图表现为传导异常，主要是室内传导缺陷（10例中有8例），如束支或分支阻滞。超声心动图显示2例患者左心室收缩功能改变。有创电生理检查显示3例患者希氏束下阻滞，需要植入心脏起搏器。这3例患者的PR间期持续时间和QRS波群宽度正常。
结论
我们建议所有DM1患者都应接受心脏检查，以检测亚临床心脏受累情况。
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