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The Correlation of Skeletal and Cardiac Muscle Dysfunction in Duchenne Muscular Dystrophy
Andrew D Posner1, Jonathan H Soslow2, W Bryan Burnette3
1Vanderbilt University, Nashville, TN, United States.
Insights
In Duchenne muscular dystrophy (DMD), maintained skeletal muscle strength correlates with better cardiac function. This relationship is significant in non-ambulatory patients, highlighting the need for cardiac monitoring in DMD research.
Area of Science:
- Neurology
- Cardiology
- Genetics
Background:
- Duchenne muscular dystrophy (DMD) involves progressive skeletal and cardiac muscle decline.
- The link between skeletal and cardiac dysfunction progression in DMD is not fully understood.
- Understanding this relationship is crucial for evaluating new DMD therapies' cardiac effects.
Purpose of the Study:
- To investigate the association between worsening skeletal muscle weakness and the development of cardiac dysfunction in DMD patients.
- To determine if skeletal muscle function predicts cardiac function in Duchenne muscular dystrophy.
Main Methods:
- Studied 77 Duchenne muscular dystrophy patients, collecting data on muscle strength, cardiac function (fractional shortening), and medications.
- Employed Spearman rank correlation and generalized least squares to analyze the relationship between muscle strength (subjective and quantitative muscle testing) and cardiac function.
- Adjusted analyses for age, ambulatory status, and medication duration.
Main Results:
- A significant correlation was found between maintained arm/leg muscle strength and preserved cardiac function (fractional shortening).
- Quantitative muscle testing showed a significant association with cardiac function in non-ambulatory DMD subjects.
- No significant association was observed between muscle testing and cardiac function in ambulatory DMD subjects.
Conclusions:
- A significant relationship exists between skeletal muscle and cardiac function in non-ambulatory Duchenne muscular dystrophy patients.
- This association suggests careful cardiac monitoring is warranted in DMD studies, even when cardiac function is not the primary outcome.
- While not implying causation, the findings emphasize the interconnectedness of muscle systems in DMD.
Background:
Duchenne muscular dystrophy (DMD) is characterized by progressive skeletal muscle and cardiac dysfunction. While skeletal muscle dysfunction precedes cardiomyopathy, the relationship between the progressive decline in skeletal and cardiac muscle function is unclear. This relationship is especially important given that the myocardial effects of many developing DMD therapies are largely unknown.
Objective:
Our objective was to assess the relationship between progression of skeletal muscle weakness and onset of cardiac dysfunction in DMD.
Methods:
A total of 77 DMD subjects treated at a single referral center were included. Demographic information, quantitative muscle testing (QMT), subjective muscle strength, cardiac function, and current and retrospective medications were collected. A Spearman rank correlation was used to evaluate for an association between subjective strength and fractional shortening. The effects of total QMT and arm QMT on fractional shortening were examined in generalized least square with and without adjustments for age, ambulatory status, and duration of corticosteroids and cardiac specific medications.
Results:
We found a significant correlation between maintained subjective skeletal muscle arm and leg strength and maintained cardiac function as defined by fractional shortening (rho=0.47, p=0.004 and rho=0.48, p=0.003, respectively). We also found a significant association between QMT and fractional shortening among non-ambulatory DMD subjects (p=0.03), while this association was not significant in ambulatory subjects.
Conclusions:
Our findings allow us to conclude that in this population, there exists a significant relationship between skeletal muscle and cardiac function in non-ambulatory DMD patients. While this does not imply a causal relationship, a possible association between skeletal and cardiac muscle function suggests that researchers should carefully monitor cardiac function, even when the primary outcome measures are not cardiac in nature.
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