Ann Rehabil Med.  2024 Dec;48(6):405-412. 10.5535/arm.240006.

Muscle Pathology Associated With Cardiac Function in Duchenne Muscular Dystrophy

• Yoon JA
• Lee H
• Lee IS
• Song YS
• Lee BJ
• Kim SY
• Shin YB

• KMID: 2562971
• DOI: http://doi.org/10.5535/arm.240006

Abstract

Objective
To compare the progression of muscle fibrosis of various site and its relation between cardiac deterioration in Duchenne muscular dystrophy (DMD). In this study aimed to examine the associations between echocardiogram-based cardiac function indices and fibrosis of the abdominal and lower extremity muscles in patients with DMD to facilitate early detection of cardiac dysfunction and identify its predictors.
Methods
Twenty-one patients with DMD patients were enrolled in the study. The association between cardiac dysfunction and fibrosis of the abdominal and lower extremity muscles was determined by analyzing the echocardiography and elastography. Non-parametric Spearman rank correlation coefficients were used to examine the pairwise relationships between cardiac function and muscle elasticity.
Results
All patients were male and non-ambulant. Their mean age was 18.45±4.28 years. The strain ratios of the abdominal muscle and quadriceps muscles were significantly higher than those of the medial gastrocnemius. The strain ratio of the rectus abdominis muscle has a significant negative correlation with left ventricular ejection fraction. Cardiac function and valvular insufficiency were not significantly correlated with muscle strain ratio. According to the result of our study, the only skeletal muscle which showed significant correlation with cardiac dysfunction was degree abdominal muscle fibrosis.
Conclusion
The degree of fibrosis of respiratory muscles was also significantly associated with cardiac dysfunction; therefore, it can be used as a predictor of cardiac dysfunction in patients with DMD in clinical practice.

Keyword

Duchenne muscular dystrophy; Muscles; Left ventricular dysfunction; Fibrosis; Elastography

Figure

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  Fig. 1. B-mode ultrasound and dynamic sonoelastography (DS) for measuring strain ratio including region of interest placement on muscle and subcutaneous tissue (right) and DS score (Score 3) (left).

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  Fig. 2. Box-plot of the Kruskal–Wallis rank-sum test result for the muscle strain ratio. GCM, gastrocnemius medialis. *Significant difference (p<0.05).

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  Fig. 3. Left ventricular ejection fraction showing negative correlation with abdominal muscle strain ratio.

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