Article

Ventricular arrhythmia in X-linked Emery-Dreifuss muscular dystrophy: a lesson from an autopsy case.

Department of Cardiology, Okazaki City Hospital, Japan.
Internal Medicine (impact factor: 0.94). 01/2011; 50(5):459-62. pp.459-62
Source: PubMed

ABSTRACT Emery-Dreifuss muscular dystrophy (EDMD) is a distinctive form of muscular dystrophy which is often associated with cardiac abnormalities. Conduction disturbances are frequently observed, and may necessitate pacemaker implantation to prevent sudden death. In this case report, we present an autopsy of a 31-year-old man with X-linked EDMD who developed only minimal skeletal muscle symptoms, and who died from ventricular arrhythmia despite undergoing a previous pacemaker implantation. Ventricular arrhythmias in X-linked EDMD patients are also discussed.

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Keywords

31-year-old man
 
cardiac abnormalities
 
case report
 
Emery-Dreifuss muscular dystrophy
 
minimal skeletal muscle symptoms
 
muscular dystrophy
 
pacemaker implantation
 
previous pacemaker implantation
 
ventricular arrhythmia
 
Ventricular arrhythmias
 
X-linked EDMD patients