[埃默里-德赖富斯肌营养不良症的心脏受累：一例报告]

[Cardiac involvement in Emery-Dreifuss muscular dystrophy: a case report].

作者信息

Chabrak S, Ammar S, Ammar N, Ouali S, Mghaieth F, Larbi N, Kafsi N, Hentati F, Mechmeche R

机构信息

Service des Explorations Fonctionnelles et de Réanimatin Cardiologique, La Rabta, Tunis.

出版信息

Tunis Med. 2006 Jun;84(6):361-4.

PMID:17042210

Abstract

Emery Dreifuss muscular dystrophy (EDMD) is an uncommon hereditary myopathy characterized by 3 symptoms: slow progressive muscular atrophy, muscular contractures and cardiac disease which affect prognosis. We report a 22 year-old patient with EDMD which shows the typical features of the associated dilated cardiomyopathy, ventricular arrhythmia, atrio-ventricular block, atrial standstill then atrial paralysis.

摘要

埃默里-德赖富斯肌营养不良症（EDMD）是一种罕见的遗传性肌病，其特征有三种症状：缓慢进行性肌肉萎缩、肌肉挛缩和影响预后的心脏病。我们报告一名22岁的EDMD患者，其表现出扩张型心肌病、室性心律失常、房室传导阻滞、心房停搏继而心房麻痹的典型特征。