Regular Holter electrocardiographic monitoring is warranted in patients at high risk of NSVT, as nonsustained ventricular tachycardia was detected in 8.3% of recordings and reduced left ventricular function was identified as the strongest associated factor.
Nonsustained ventricular tachycardia (NSVT) was detected in 8.3% of Holter ECG recordings from patients with Duchenne muscular dystrophy.
Premature ventricular complexes at a frequency of ≥720 per 24 hours were detected in 12.2% of Holter ECG recordings.
Bradyarrhythmia and supraventricular tachyarrhythmia were rare findings in DMD patients undergoing Holter monitoring.
Reduced left ventricular function was identified as the strongest associated factor for NSVT in patients with DMD.
Older age, nonuse of noninvasive positive-pressure ventilation, and a Dp140-deficient genotype were associated with increased risk of NSVT, though these associations were statistically nonsignificant.
A Dp116-deficient genotype was associated with lower odds of NSVT in patients with DMD.
Cardiac causes account for approximately 30% of all deaths in DMD, providing the clinical rationale for the study.
This research suggests that dangerous heart rhythm abnormalities, particularly a type called nonsustained ventricular tachycardia (NSVT), occur in a meaningful proportion of people with Duchenne muscular dystrophy (DMD). By analyzing over 1,000 heart monitor recordings from 167 patients aged 9 to 44 years, the researchers found NSVT in about 8% of recordings and high rates of abnormal extra heartbeats (called premature ventricular complexes) in about 12% of recordings. In contrast, other types of arrhythmias such as slow heart rhythms or fast rhythms originating in the upper chambers of the heart were uncommon. The most important factor linked to NSVT was reduced heart pumping function (left ventricular dysfunction). Older age, not using breathing support machines (noninvasive positive-pressure ventilation), and having a specific genetic subtype (Dp140-deficient) were also associated with higher NSVT risk, though these associations did not reach statistical significance — possibly because the study was not large enough to detect them definitively. Interestingly, patients with a different genetic subtype (Dp116-deficient) appeared to have lower NSVT risk, suggesting that the specific genetic makeup of DMD may influence heart rhythm risk differently. This research suggests that regular Holter ECG monitoring (a portable heart monitor worn over 24 hours or longer) is important for DMD patients, particularly those who already show signs of weakened heart function. Since NSVT can potentially trigger sudden cardiac death in people with underlying heart disease, identifying high-risk patients early may allow for closer monitoring and timely medical management. The study also highlights a gap in the existing evidence, as large studies including adult DMD patients have been lacking.
Sugiyama R, Takeshita E, Aihara Y, Shimizu-Motohashi Y, Izumi S, Oba M, et al.. (2026). Prevalence and Characteristics of Cardiac Arrhythmia in Duchenne Muscular Dystrophy: A Retrospective, Single-Center Holter Electrocardiographic Monitoring Study.. European journal of neurology. https://doi.org/10.1111/ene.70621