Effects of steroids and angiotensin converting enzyme inhibition on circumferential strain in boys with Duchenne muscular dystrophy: a cross-sectional and longitudinal study utilizing cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2011 Oct 19;13(1):60
Date
10/21/2011Pubmed ID
22011358Pubmed Central ID
PMC3207955DOI
10.1186/1532-429X-13-60Scopus ID
2-s2.0-84857668964 (requires institutional sign-in at Scopus site) 47 CitationsAbstract
BACKGROUND: Steroid use has prolonged ambulation in Duchenne muscular dystrophy (DMD) and combined with advances in respiratory care overall management has improved such that cardiac manifestations have become the major cause of death. Unfortunately, there is no consensus for DMD-associated cardiac disease management. Our purpose was to assess effects of steroid use alone or in combination with angiotensin converting enzyme inhibitors (ACEI) or angiotension receptor blocker (ARB) on cardiovascular magnetic resonance (CMR) derived circumferential strain (εcc).
METHODS: We used CMR to assess effects of corticosteroids alone (Group A) or in combination with ACEI or ARB (Group B) on heart rate (HR), left ventricular ejection fraction (LVEF), mass (LVM), end diastolic volume (LVEDV) and circumferential strain (εcc) in a cohort of 171 DMD patients >5 years of age. Treatment decisions were made independently by physicians at both our institution and referral centers and not based on CMR results.
RESULTS: Patients in Group A (114 studies) were younger than those in Group B (92 studies)(10 ± 2.4 vs. 12.4 ± 3.2 years, p < 0.0001), but HR, LVEF, LVEDV and LVM were not different. Although εcc magnitude was lower in Group B than Group A (-13.8 ± 1.9 vs. -12.8 ± 2.0, p = 0.0004), age correction using covariance analysis eliminated this effect. In a subset of patients who underwent serial CMR exams with an inter-study time of ~15 months, εcc worsened regardless of treatment group.
CONCLUSIONS: These results support the need for prospective clinical trials to identify more effective treatment regimens for DMD associated cardiac disease.
Author List
Hor KN, Mazur W, Taylor MD, Al-Khalidi HR, Cripe LH, Jefferies JL, Raman SV, Chung ES, Kinnett KJ, Williams K, Gottliebson WM, Benson DWMESH terms used to index this publication - Major topics in bold
AdolescentAdrenal Cortex Hormones
Analysis of Variance
Angiotensin II Type 1 Receptor Blockers
Angiotensin-Converting Enzyme Inhibitors
Cardiomyopathies
Child
Cross-Sectional Studies
Disease Progression
Heart Rate
Humans
Longitudinal Studies
Magnetic Resonance Imaging, Cine
Male
Muscular Dystrophy, Duchenne
Myocardial Contraction
Ohio
Predictive Value of Tests
Retrospective Studies
Stroke Volume
Time Factors
Treatment Outcome
Ventricular Function, Left
