Effects of steroids and angiotensin converting enzyme inhibition on circumferential strain in boys with duchenne muscular dystrophy: a cross-sectional and longitudinal study utilizing cardiac magnetic resonance imaging
2011

Effects of Steroids and ACE Inhibition on Heart Function in Boys with Duchenne Muscular Dystrophy

Sample size: 136 publication Evidence: moderate

Author Information

Author(s): Williams Katelyn, Hor Kan N, Mazur Wojciech, Al-Khalidi Hussein R, Chung Eugene S, Cripe Linda S, Kinnett Kathi, Taylor Michael D, Akula Nandakishore, Gottliebson William M, Benson D Woodrow

Primary Institution: CCHMC, Cincinnati, OH, USA

Hypothesis

The study aims to assess the effects of steroid use alone or in combination with ACE inhibitors on cardiac function in boys with Duchenne muscular dystrophy.

Conclusion

Current treatment strategies have little effect on cardiac disease in Duchenne muscular dystrophy patients.

Supporting Evidence

  • Steroid use has prolonged ambulation in Duchenne muscular dystrophy.
  • Cardiac manifestations have become the major cause of death in Duchenne muscular dystrophy.
  • Analysis showed no significant differences in heart rate, left ventricular ejection fraction, or mass between treatment groups.
  • Circumferential strain was lower in the group receiving steroids plus ACE inhibitors, but this effect was eliminated after age correction.

Takeaway

The study looked at how steroids and other medications affect the hearts of boys with a muscle disease, and found that these treatments don't really help their heart health.

Methodology

The study reviewed medical records and CMR data from 2006 to 2010, comparing patients on steroids alone versus those on steroids plus ACE inhibitors or ARBs.

Potential Biases

Potential biases may arise from the retrospective nature of the study and the lack of randomization.

Limitations

The study is limited by its retrospective design and the lack of improvement in circumferential strain over time.

Participant Demographics

Participants were boys diagnosed with Duchenne muscular dystrophy, aged approximately 10 to 12 years.

Statistical Information

P-Value

p=0.0004

Statistical Significance

p<0.0001

Digital Object Identifier (DOI)

10.1186/1532-429X-13-S1-P284

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