Prevalence and Characteristics of Cardiac Arrhythmia in Duchenne Muscular Dystrophy: A Retrospective, Single-Center

Ryo Sugiyama1, Eri Takeshita1, Yu Aihara1

  • 1Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan.

Insights

Nonsustained ventricular tachycardia (NSVT) is a risk in Duchenne muscular dystrophy (DMD) patients, particularly those with reduced left ventricular function. Regular Holter monitoring is recommended for high-risk individuals to prevent sudden cardiac death.

Area of Science:

  • Cardiology
  • Neurology
  • Genetics

Background:

  • Cardiac complications are a leading cause of death in Duchenne muscular dystrophy (DMD).
  • Current guidelines recommend arrhythmia screening in DMD patients with left ventricular dysfunction, but evidence is limited.
  • Large cohort studies including adult DMD patients are scarce.

Purpose of the Study:

  • To characterize the arrhythmia profiles in a large cohort of Duchenne muscular dystrophy patients.
  • To identify factors associated with arrhythmias, particularly nonsustained ventricular tachycardia (NSVT).

Main Methods:

  • Analysis of 1018 Holter electrocardiographic recordings from 167 DMD patients (aged 9-44 years).
  • Logistic regression was used to identify factors associated with NSVT.

Main Results:

  • Bradyarrhythmia and supraventricular tachyarrhythmia were infrequent.
  • Nonsustained ventricular tachycardia (NSVT) was detected in 8.3% of recordings.
  • Reduced left ventricular function was the strongest predictor of NSVT, with increased risk associated with older age and nonuse of ventilation.

Conclusions:

  • NSVT poses a risk for sudden cardiac death in DMD patients with cardiac disease.
  • Regular Holter electrocardiographic monitoring is crucial for identifying high-risk patients.
  • Further research is needed to refine screening protocols for cardiac arrhythmias in DMD.
Abstract

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