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[Electrocardiography and electrophysiologic findings in patients with myotonic muscular dystrophy].

作者信息

Podczeck A, Graf M, Hief C, Kaltenbrunner W, Nürnberg M, Steinbach K

机构信息

3. Medizinische Abteilung (Kardiologie), Ludwig-Boltzmann-Institut für Arrhythmieforschung, Wilhelminespital, Wien.

出版信息

Z Kardiol. 1993 Aug;82(8):474-6.

PMID:8212780
Abstract

Myotonic dystrophy is associated with diffuse cardiac conduction disturbances. Seven consecutive patients, all asymptomatic with respect to cardiac abnormalities, were investigated by means of ECG, Holter monitoring, and invasive electrophysiologic studies (EPS). During Holter monitoring, no abnormalities were found in any patient, except for one patient who showed single monomorphic VEBs. During EPS three patients showed conduction disturbances in the AV-node (AVN) as well as in the His-Purkinje-system (HPS) in another three patients. Except for two patients ventricular vulnerability was normal during programmed ventricular stimulation. A considerable number of asymptomatic patients with myotonic dystrophy reveals AVN- and HPS-conduction disturbances as shown during EPS.

摘要

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Myotonic dystrophy: correlation of clinical symptoms with the size of the CTG trinucleotide repeat.强直性肌营养不良:临床症状与CTG三核苷酸重复序列大小的相关性。
J Neurol. 1995 Jan;242(2):99-104. doi: 10.1007/BF00887824.