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Steroid therapy and cardiac function in Duchenne muscular dystrophy
L W Markham1, R L Spicer, P R Khoury
1Department of Pediatrics, Division of Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA. Larry.Markham@cchmc.org
Pediatric Cardiology
|July 2, 2005
Summary
Steroid treatment may stabilize cardiac muscle function in Duchenne muscular dystrophy (DMD) patients. This study suggests a sustained benefit on cardiac function, independent of steroid type, even after treatment cessation.
Area of Science:
- Cardiology
- Neurology
- Genetics
Background:
- Duchenne muscular dystrophy (DMD) causes progressive skeletal and cardiac muscle deterioration.
- Steroids are known to improve motor function and respiratory strength in DMD patients.
- The impact of steroid treatment on cardiac function in DMD is not fully understood.
Purpose of the Study:
- To investigate the effect of steroid treatment on cardiac muscle function in Duchenne muscular dystrophy patients.
- To determine if steroid therapy can stabilize or improve cardiac function as measured by echocardiogram parameters.
- To assess the long-term effects and steroid type independence of treatment.
Main Methods:
- Retrospective review of echocardiograms from 111 DMD subjects (≤21 years) between 1997-2004.
- Analysis of medical records for steroid treatment history (prednisone, deflazacort).
- Comparison of cardiac function metrics (shortening fraction) between treated and untreated groups, and across different treatment durations and types.
Main Results:
- No significant differences in baseline characteristics between treated and untreated groups.
- Untreated subjects were significantly more likely to have a reduced shortening fraction (<28%) compared to treated subjects, particularly older children.
- No difference in shortening fraction was observed between prednisone and deflazacort subgroups, or between subjects currently on treatment versus those who had completed it.
Conclusions:
- Steroid treatment appears to alter the progressive decline in cardiac function in Duchenne muscular dystrophy.
- The beneficial effect on cardiac function is sustained beyond the treatment period and is independent of the specific steroid used.
- These findings support the use of steroids to manage cardiac complications in DMD.
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