Nonsustained ventricular tachycardia was detected in 8.3% of Holter recordings from patients with Duchenne muscular dystrophy, with reduced left ventricular function being the strongest associated factor.
Observational (n=167)
No
What is the prevalence of cardiac arrhythmias and their associated factors in patients with Duchenne muscular dystrophy?
Reduced left ventricular function is strongly associated with nonsustained ventricular tachycardia in patients with Duchenne muscular dystrophy, highlighting the importance of regular Holter monitoring in this high-risk population.
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.
Sugiyama et al. (2026) conducted an observational in Duchenne muscular dystrophy (n=167). Duchenne muscular dystrophy was evaluated on Nonsustained ventricular tachycardia (NSVT). Nonsustained ventricular tachycardia was detected in 8.3% of Holter recordings from patients with Duchenne muscular dystrophy, with reduced left ventricular function being the strongest associated factor.