Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 1999 Jul;82(1):105-8.
doi: 10.1136/hrt.82.1.105.

Cardiac involvement in Emery Dreifuss muscular dystrophy: a case series

A E Buckley  1 , J Dean, I R Mahy
Affiliations
Case Reports

Cardiac involvement in Emery Dreifuss muscular dystrophy: a case series

A E Buckley et al. Heart. 1999 Jul.

Abstract

Three patients with Emery Dreifuss muscular dystrophy are reported. Emery Dreifuss muscular dystrophy is an X linked muscular dystrophy, in which locomotor involvement is characteristically mild and slowly progressive. The effect on the heart becomes apparent in the teenage years and is characterised by cardiac conduction defects and infiltration of the myocardium by fibrous and adipose tissue. It first affects the atria, which results in atrial paralysis; treatment with ventricular pacing is usually needed. Female carriers can develop heart problems and are at risk of sudden death. Relatives of affected patients should be offered screening with electrocardiography and echocardiography.

PubMed Disclaimer

Figures

Figure 1
Figure 1
12 lead ECG of case 1 showing a junctional bradycardia of 37 beats/min. P waves are absent.
Figure 2
Figure 2
Transthoracic echocardiogram of case 1 (apical four chamber view) showing marked right atrial dilatation. The echodense area in the region of the tricuspid valve is due to reflected echoes from a ventricular pacing lead. RA, right atrium.
See this image and copyright information in PMC

Publication types

MeSH terms