Cardiac Conduction Disorders as Markers of Cardiac Events in Myotonic Dystrophy Type 1

J Am Heart Assoc. 2020 Sep;9(17):e015709. doi: 10.1161/JAHA.119.015709. Epub 2020 Aug 19.

Abstract

Background Myotonic dystrophy type 1 involves cardiac conduction disorders. Cardiac conduction disease can cause fatal arrhythmias or sudden death in patients with myotonic dystrophy type 1. Methods and Results This study enrolled 506 patients with myotonic dystrophy type 1 (aged ≥15 years; >50 cytosine-thymine-guanine repeats) and was treated in 9 Japanese hospitals for neuromuscular diseases from January 2006 to August 2016. We investigated genetic and clinical backgrounds including health care, activities of daily living, dietary intake, cardiac involvement, and respiratory involvement during follow-up. The cause of death or the occurrence of composite cardiac events (ie, ventricular arrhythmias, advanced atrioventricular blocks, and device implantations) were evaluated as significant outcomes. During a median follow-up period of 87 months (Q1-Q3, 37-138 months), 71 patients expired. In the univariate analysis, pacemaker implantations (hazard ratio [HR], 4.35; 95% CI, 1.22-15.50) were associated with sudden death. In contrast, PQ interval ≥240 ms, QRS duration ≥120 ms, nutrition, or respiratory failure were not associated with the incidence of sudden death. The multivariable analysis revealed that a PQ interval ≥240 ms (HR, 2.79; 95% CI, 1.9-7.19, P<0.05) or QRS duration ≥120 ms (HR, 9.41; 95% CI, 2.62-33.77, P < 0.01) were independent factors associated with a higher occurrence of cardiac events than those observed with a PQ interval <240 ms or QRS duration <120 ms; these cardiac conduction parameters were not related to sudden death. Conclusions Cardiac conduction disorders are independent markers associated with cardiac events. Further investigation on the prediction of occurrence of sudden death is warranted.

Keywords: conduction disturbance; myotonic dystrophy; sudden death; ventricular tachycardia.

Publication types

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

MeSH terms

  • Activities of Daily Living
  • Adult
  • Aftercare
  • Arrhythmias, Cardiac / physiopathology*
  • Atrioventricular Block / epidemiology
  • Atrioventricular Block / therapy
  • Cardiac Conduction System Disease / complications*
  • Death, Sudden, Cardiac / epidemiology
  • Death, Sudden, Cardiac / prevention & control*
  • Eating
  • Female
  • Health Status
  • Humans
  • Incidence
  • Japan / epidemiology
  • Male
  • Middle Aged
  • Myotonic Dystrophy / complications*
  • Myotonic Dystrophy / genetics
  • Myotonic Dystrophy / mortality
  • Pacemaker, Artificial / statistics & numerical data*
  • Proportional Hazards Models
  • Retrospective Studies