In a large retrospective study, DM1 patients with conduction abnormalities had lower all-cause mortality and sudden death with electrophysiological testing and prophylactic pacing than with a noninvasive strategy.
Myotonic dystrophy is the most common adult-onset muscular dystrophy. It is a multisystem disease with frequent and substantial cardiac involvement, including asymptomatic electrocardiogram (ECG) abnormalities (commonly prolongation of the PR interval or QRS duration), rhythm disturbances (progressive heart block; atrial tachycardia, flutter, and fibrillation; and ventricular tachycardia and fibrillation), and sudden death from asystole after atrioventricular (AV) block or ventricular tachyarrhythmia. Permanent pacing even for first-degree AV block has been recommended by the American College of Cardiology and the American Heart Association, but empirical data supporting this recommendation have been relatively scant. Now, researchers have …
Reviewing Author
DisclosuresGrant / Research supportNIH NeuroBioBank; ALS Association; NIH/National Institute of Neurological Disorders and Stroke; NIH/National Center for Advancing Translational Sciences; FDA; Department of Defense
Editorial boardsCochrane Collaboration
Leadership positions in professional societiesMuscle Study Group Executive Committee
DisclosuresGrant / Research supportNIH NeuroBioBank; ALS Association; NIH/National Institute of Neurological Disorders and Stroke; NIH/National Center for Advancing Translational Sciences; FDA; Department of Defense
Editorial boardsCochrane Collaboration
Leadership positions in professional societiesMuscle Study Group Executive Committee