Right ventricular remodeling in the hearts of patients with Duchenne muscular dystrophy (DMD) occurs in the context of preserved right ventricular ejection fraction, according to a new study published in the Journal of Magnetic Resonance Imaging.

This abnormal right ventricular deformation is associated with older age and decreased left ventricular ejection fraction, Nicholas K. Brown, MD, of the Seattle Children’s Hospital in Washington, and colleagues noted.

The right ventricle is relatively well preserved in patients with DMD. However, its structure and function are difficult to characterize because of its complex architecture, the study authors added.


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The researchers set out to clarify the link between the clinical variables of DMD and the structure and function of the myocardium. In doing so, the study team conducted a retrospective study in 20 patients with DMD and 18 age-matched controls. Patients were 8 to 21 years of age.

The investigators conducted tissue phase mapping in the basal, mid, and apical short-axis planes and collected clinical data from the patients. 

They quantified the radial, circumferential, and longitudinal velocities and corresponding time to peak velocities from the tissue phase mapping. They used these values to calculate any right ventricular twist and intraventricular and interventricular dyssynchrony.

The results showed that patients with DMD had preserved right ventricular ejection fraction. However, their interventricular dyssynchrony was significantly increased compared to controls. 

When they compared the patients with DMD with each other, the researchers found that the right ventricular dyssynchrony was significantly increased with lower left ventricular ejection fraction. Moreover, right ventricular intraventricular dyssynchrony significantly increased with older age.

“Further studies evaluating the role of [the right ventricle] in clinical outcomes in DMD are warranted,” the authors concluded.

Reference

Brown N, Berhane H, Gambetta K, et al. Right ventricular remodeling assessed by MRI in Duchenne muscular dystrophy. J Magn Reson Imaging. Published online November 10, 2022. doi:10.1002/jmri.28521