What is the role of ECG in the workup 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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Answer

ECG should be obtained in all patients with EDMD.

Early changes include low amplitude P waves and a prolonged PR interval.

Progression to bradycardia, absent P waves, irregular atrial rhythm, atrial fibrillation and flutter, AV-conduction defects, and a late cardiomyopathy all have been reported.

A classic pattern is of a junctional escape rhythm at 40-50 beats per minute without P waves.

Confirmation of the diagnosis is obtained by demonstration of a lack of all electrical and mechanical activity of the atria and an inability to pace the atria confirming that the myocardium, not the conduction system, is affected.


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