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July 25, 2012

Arrhythmia Management in Myotonic Dystrophy Type 1

Author Affiliations

Letters Section Editor: Jody W. Zylke, MD, Senior Editor.

Author Affiliations: Division of Cardiology (Krannert Institute), Indiana University, Indianapolis (wgroh@iupui.edu).

JAMA. 2012;308(4):337-338. doi:10.1001/jama.2012.6807

To the Editor: Dr Wahbi and colleagues1 concluded that sudden death mortality was improved in patients with myotonic dystrophy type 1 (DM1) and moderate conduction abnormalities on electrocardiogram who underwent an invasive strategy consisting of an electrophysiological study followed by prophylactic pacing if the HV interval exceeded 70 milliseconds. In our opinion, there are uncertainties in how the retrospective observational trial was conducted and reported that limit the ability to determine if their invasive strategy was associated with a survival advantage.