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Updated: Mar 21, 2026
Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Comparison of left ventricular function assessment between echocardiography and MRI in Duchenne muscular dystrophy
Sujatha Buddhe1, Mark Lewin2, Aaron Olson2
1Division of Cardiology, Department of Pediatrics, University of Washington School of Medicine and Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA, 98105, USA. sujatha.buddhe@seattlechildrens.org.
Insights
Echocardiography shows weak correlation with cardiac MRI for assessing heart function in Duchenne muscular dystrophy (DMD) patients. Cardiac MRI is recommended for early and routine functional assessment in children with DMD.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Medical Imaging
Background:
- Cardiomyopathy is a major cause of mortality in Duchenne muscular dystrophy (DMD).
- Echocardiography is the standard for assessing left ventricular (LV) function but has limitations in DMD patients.
- Cardiac MRI offers advanced imaging capabilities for evaluating cardiac function.
Purpose of the Study:
- To compare echocardiographic measures of cardiac function with cardiac MRI in children and young adults with DMD.
- To evaluate the accuracy of echocardiography in assessing LV function and strain compared to MRI.
- To determine the utility of MRI for functional assessment and late gadolinium enhancement (LGE) imaging in DMD.
Main Methods:
- Retrospective analysis of DMD patients who underwent both echocardiography and MRI between 2010-2015.
- Measurement of echocardiographic and MRI parameters, including LV ejection fraction (LVEF) and myocardial strain.
- Categorization of subjects into two groups based on MRI LVEF (≥55% and <55%).
Main Results:
- Echocardiographic assessment showed diminished function in only 50% of patients with reduced LVEF by MRI.
- Suboptimal echocardiographic image quality was noted in 39% of subjects.
- Weak correlation was found between echocardiographic parameters and MRI-derived LVEF; MRI-derived strain showed better correlation.
Conclusions:
- Echocardiography demonstrates a weak correlation with MRI for ventricular functional assessment in DMD.
- Despite improvements with adequate image quality, echocardiography's correlation remains modest and potentially insufficient for clinical management.
- Routine and early cardiac MRI is recommended for both LGE imaging and functional assessment in pediatric DMD patients.
Background:
Cardiomyopathy in Duchenne muscular dystrophy (DMD) is associated with death in approximately 40% of patients. Echocardiography is routinely used to assess left ventricular (LV) function; however, it has limitations in these patients.
Objective:
We compared echocardiographic measures of cardiac function assessment to cardiac MRI.
Materials And Methods:
We included children and young adults with DMD who had MRI performed between January 2010 and July 2015. We measured echocardiographic and MRI parameters of function assessment, including strain. Presence of late gadolinium enhancement (LGE) was assessed by MRI. Subjects were divided into two groups based on MRI left ventricular ejection fraction (LVEF): group I, LVEF ≥55% and group II, LVEF <55%.
Results:
We included 41 studies in 33 subjects, with 25 in group I and 16 in group II. Mean age of subjects was 13.6 ± 2.8 years and mean duration between echocardiogram and MRI was 7.6 ± 4.1 months. Only 8 of 16 (50%) patients in group II had diminished function on echocardiogram. Echocardiographic images were suboptimal in 16 subjects (39%). Overall, echocardiographic parameters had weak correlation with MRI-derived ejection fraction percentage. MRI-derived myocardial strain assessment has better correlation with MRI ejection fraction as compared to echocardiography-derived strain parameters.
Conclusion:
Echocardiography-based ventricular functional assessment has weak correlation with MRI parameters in children and young adults with Duchenne muscular dystrophy. While this correlation improves in the subset of subjects with adequate echocardiographic image quality, it remains modest and potentially suboptimal for clinical management. Accordingly, we conclude that MRI should be performed routinely and early in children with DMD, not only for LGE imaging but also for functional assessment.
