Prevalence and Characteristics of Cardiac Arrhythmia in Duchenne Muscular Dystrophy: A Retrospective, Single-Center Holter Electrocardiographic Monitoring Study.

BACKGROUND

In Duchenne muscular dystrophy (DMD), cardiac causes account for approximately 30% of all deaths. Therefore, evaluating cardiac function in older patients is important. In addition, current recommendations advise screening for arrhythmias when left ventricular dysfunction is identified; however, the evidence base underpinning this recommendation in DMD is limited. Furthermore, large-scale cohort studies with substantial adult representation are lacking.

METHODS

To characterize arrhythmia profiles of patients with DMD, we analyzed 1018 Holter electrocardiographic recordings from 167 patients with DMD (age: 9-44 years).

RESULTS

Bradyarrhythmia and supraventricular tachyarrhythmia were rare; nonsustained ventricular tachycardia (NSVT) and ≥ 720 premature ventricular complexes per 24 h were detected in 85 (8.3%) and 124 (12.2%) recordings, respectively. Logistic regression identified reduced left ventricular function as the strongest associated factor for NSVT. Although statistically nonsignificant, risk increased with age, nonuse of noninvasive positive-pressure ventilation, and a Dp140-deficient genotype. A Dp116-deficient genotype was associated with lower odds of NSVT.

CONCLUSIONS

Given that NSVT may trigger sudden cardiac death in susceptible patients with underlying cardiac disease, regular Holter electrocardiographic monitoring is warranted in patients at high risk of NSVT.