Right ventricular (RV) systolic dysfunction is common in adult patients with Duchenne muscular dystrophy (DMD) and it is associated with a higher risk of cardiac events according to results of a clinical trial published in the Journal of the American Heart Association.
The prevalence of RV systolic dysfunction was fairly high in the studied population with it being present in a total of 46 out of the 90 patients (51%) included in the study. The presence of RV systolic dysfunction significantly increased the risk of cardiac events in patients. A total of 11 cardiac events (23.9%) occurred in the patients with RV dysfunction compared to only 2 in those without the dysfunction (4.5%; P =.008).
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Using a multivariable Cox model that included RV systolic dysfunction, left ventricular (LV) ejection fraction, and 24-hour home mechanical ventilation, the RV dysfunction had an adjusted hazard ratio (aHR) of 4.96 (P =.04). Using the same model, the aHR for LV ejection fraction was .93 (P = .003). Death due to cardiac complications occurred in 6 of the 11 RV systolic dysfunction patients and 1 of those without dysfunction.
“The coexistence of LV systolic dysfunction and restrictive pulmonary failure is well described in patients with DMD. In the present study, we clearly demonstrated that RV systolic impairment is also present in half of patients with DMD and that this feature is associated with increased risk of cardiac events,” the authors wrote.
Additional analysis found no relationship between the existence of RV systolic dysfunction at study inclusion and the development of new LV dysfunction. Patients with an LV ejection fraction <50% were at an increased risk of developing RV dysfunction during follow-up, however (odds ratio, 4.5; P =.03).
The 90 patients with genetically confirmed DMD were enrolled in the study between 2010 and 2019. The median age of patients was 27.5 years and 99% were wheelchair-bound. Home mechanical ventilation was utilized in 83 of the patients including 51 who were ventilated 24 hours per day.
Fayssoil A, Mansencal N, Nguyen LS, et al. Prognosis of right ventricular systolic dysfunction in patients with Duchenne muscular dystrophy. J Am Heart Assoc. Published online August 10, 2023. doi:10.1161/jaha.122.027231