Which cardiac findings are characteristic of Emery-Dreifuss muscular dystrophy (EDMD)?

Updated: May 23, 2019
  • Author: Eli S Neiman, DO, FACN; Chief Editor: Amy Kao, MD  more...
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  • Cardiac disease usually begins after onset of weakness and manifests as syncope in the second or third decade.

  • Pacemakers are often needed by age 30 years.

  • Cardiac disease may present with sudden cardiac death.

  • Bradycardia, atrial arrhythmias (including atrial fibrillation/flutter), AV conduction defect, and atrial paralysis have all been reported.

  • Late findings may include atrial or ventricular cardiomyopathy.

  • Of female carriers, 10-20% have atrial arrhythmias or conduction defects and need to be monitored with yearly ECG to try to prevent sudden cardiac death.

  • Conduction defects with minimal muscle and joint involvement may occur. [3]

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