Effects of steroids and angiotensin converting enzyme inhibition on circumferential strain in boys with Duchenne muscula
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RESEARCH
Open Access
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 Kan N Hor1*, Wojciech Mazur2, Michael D Taylor1, Hussein R Al-Khalidi3, Linda H Cripe1, John L Jefferies1, Subha V Raman4, Eugene S Chung2, Kathi J Kinnett1, Katelyn Williams1, William M Gottliebson1 and D Woodrow Benson1
Abstract 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.
Background Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder affecting approximately 1:3,500 males [1-3]. Boys with DMD are currently treated with corticosteroids at a young age to prolong ambulation. This therapy combined with improvements in respiratory care * Correspondence: [email protected] 1 The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA Full list of author information is available at the end of the article
have resulted in increased survival [4-6] such that DMDassociated heart disease is now the leading cause of mortality [7-11]. Myocardial changes, as a result of the lack of dystrophin, consist of cell membrane degradation, interstitial inflammation, edema, fatty replacement and fibrosis [12-15]. Despite reports in small cohorts of the beneficial cardiovascular effects of corticosteroids [6,16] and angiotensin converting enzyme inhibitors (ACEI)/ angiotensin recept
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