Abstract
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 …
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