Electrocardiogram vs Electrophysiological Study and Major Conduction Delays in Myotonic Dystrophy Type 1

Should the identification of patients with myotonic dystrophy type 1 (; DM1) who are at risk for major bradyarrhythmic events and eligible for prophylactic pacing be based on electrocardiogram (ECG) or electrophysiological study criteria?In a multicenter cohort of 706 patients with DM1, a His-ventricular interval of 70 milliseconds or greater during electrophysiological study proved more sensitive and accurate than ECG-based criteria for predicting major bradyarrhythmic events.These findings suggest that electrophysiological study offers greater accuracy than ECG in selecting patients with DM1 for prophylactic permanent pacing.

Corresponding Author: Karim Wahbi, MD, PhD, Department of Cardiology, Cochin Hospital, 27 Rue du Faubourg Saint Jacques, 75679 Paris Cedex 14, France (karim.wahbi@aphp.fr).

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