Severe cardiac arrhythmias in young patients with myotonic dystrophy type 1

Neurology. 2004 Nov 23;63(10):1939-41. doi: 10.1212/01.wnl.0000144343.91136.cf.

Abstract

Cardiac tachyarrhythmias have rarely been studied in young patients with myotonic dystrophy type 1 (DM1). The authors observed major cardiac rhythm disturbances in 11 patients aged 10 to 18 years. Tachyarrhythmic events were more frequent than impulse conduction disorders. Wide variations in CTG expansion were observed among the population. Since physical exercise was a prominent arrhythmogenic factor, systematic exercise tests with EKG monitoring may be indicated in young patients with DM1.

Publication types

  • Case Reports
  • Multicenter Study
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Adrenergic beta-Antagonists / therapeutic use
  • Arrhythmias, Cardiac / etiology*
  • Atrial Flutter / etiology
  • Atrial Flutter / surgery
  • Catheter Ablation
  • Child
  • Chromosomes, Human, Pair 19 / genetics
  • Death, Sudden, Cardiac / etiology
  • Defibrillators, Implantable
  • Exercise Test
  • Female
  • Heart Arrest / etiology
  • Humans
  • Male
  • Myotonic Dystrophy / complications*
  • Myotonic Dystrophy / diagnosis
  • Myotonic Dystrophy / genetics
  • Retrospective Studies
  • Tachycardia, Ventricular / drug therapy
  • Tachycardia, Ventricular / etiology
  • Trinucleotide Repeats
  • Ventricular Fibrillation / etiology
  • Ventricular Fibrillation / surgery

Substances

  • Adrenergic beta-Antagonists