Determinants of Risk for Sudden Death in Myotonic Dystrophy

In a multicenter case series, an abnormal ECG and atrial tachycardia were independent predictors of sudden cardiac death.

Myotonic dystrophy, the most common adult-onset muscular dystrophy, is associated with cardiac conduction system disease and sudden cardiac death. In patients with this syndrome, both heart block and ventricular arrhythmias may cause sudden death; however, the clinical predictors of sudden death are not well characterized.

In the largest case series to date, investigators studied 406 adults with myotonic dystrophy at 23 neuromuscular centers for a mean of 5.7 years. Ninety-six participants had abnormal electrocardiograms at baseline, including 29 with atrial arrhythmias; other abnormal ECG findings were first-, second-, and third-degree heart block, and prolonged QRS duration. During follow-up, 81 patients died, including 27 of sudden cardia…

Reviewing Author

Mark S. Link, MD

Disclosures

Editorial BoardsCirculation; UpToDate

Mark S. Link, MD

Disclosures

Editorial BoardsCirculation; UpToDate