Atrial Arrhythmias Common in DMD, but More Data on Treatment Are Needed

Cases of atrial arrhythmia in patients with Duchenne muscular dystrophy (DMD) are common but lack a standard solution, highlighting the challenging balance between risk and benefit and a need for more long-term data on the management of arrhythmia in patients with DMD, according to the authors of a case series published in HeartRhythm Case Reports.¹

“While the risk of atrial arrhythmias has been recognized in DMD patients, to date there is no standard approach to management,” the authors wrote. “DMD is rare, and patients are often excluded from trials looking at the benefits and risks of antiarrhythmic therapy and catheter ablation. The most recent expert consensus statement on the evaluation and management of arrhythmias in neuromuscular disorders comments on the risk of atrial arrhythmias in DMD patients, but the only management discussed is anticoagulation.”

Recent research shows that DMD affects both the atrial and ventricular myocardium, meaning patients are at an increased risk of arrhythmias and cardiac dysfunction in general.² The authors of the case series also noted that prevalent comorbidities among patients with DMD, such as sleep disordered breathing and body fat accumulation due to chronic steroid use, increase the risk of arrhythmia even further.¹

The case series included 5 patients with DMD who had developed atrial arrhythmias by their early adult years that were challenging to manage. The authors emphasized the tendency for such arrhythmias to develop in patients with DMD, as well as reviewed the management of atrial arrhythmia in these patients.

The authors of the case series noted that prevalent comorbidities among patients with DMD increase the risk of arrhythmias even further. | Image credit: Elena - stock.adobe.com

In the series, 3 patients underwent ablation to address recurrent arrhythmias in addition to maximally tolerated medical therapy, but atrial arrhythmias returned within days or months of ablation. In the general adult population, the authors noted, 12-month atrial arrhythmia recurrence following ablation ranges from 20% to 50%, suggesting patients with DMD may have a higher failure rate vs the general population.

“In patients with DMD, ablation may prove to be less successful because of the diffuse changes seen in the atrium as opposed to focal areas of disease,” the authors explained. “While not observed in our cohort, the risk of complications such as perforation during a catheter-based procedure may be higher in this population given the thin atrial walls in the setting of pathologic fatty replacement.”

While none of the patients in the case series experienced stroke or thromboembolic complications, most received some type of anticoagulation and most were found to have decreased ejection fraction related to frequent arrhythmia at some point in time. One of the patients died due to multiorgan dysfunction linked to decompensated heart failure, with rapid progression after atrial fibrillation onset 2 years prior to his death, the authors noted.

“The patients included in this case series demonstrate that atrial arrhythmias can be problematic in patients with DMD and can contribute to morbidity and mortality,” the authors concluded. “However, there is not an easy solution, as most cases prove to be recalcitrant with medical management and unresponsive to ablations. This lack of solutions highlights the difficult balance between risk and benefit when it comes to treating arrhythmias in this patient population and the need for long-term, multicenter studies given the rarity of this disease.”

References

1. Greiner E, Villa CR, Ryan TD, Spar DS. Refractory atrial arrhythmias in Duchenne muscular dystrophy: a case series. HeartRhythm Case Rep. Published online August 2, 2024. doi:10.1016/j.hrcr.2024.07.023

2. Greiner E, Breaux A, Kasten J, et al. Cardiac atrial pathology in Duchenne muscular dystrophy. Muscle Nerve. 2024;69(5):572-579. doi:10.1002/mus.28072