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

Abstract

Introduction : 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. Purpose To assess effects of steroid use alone or in combination with angiotensin converting enzyme inhibitors (ACEI) or angiotension receptor blocker (ARB) on cardiac magnetic resonance imaging (CMR) derived circumferential strain (ecc). Methods We reviewed our DMD CMR database and medical records from February 2006 to 2010. The study cohort was divided into patients receiving steroids alone (Group A) or steroids plus ACEI or ARB (Group B). Analysis of covariance was used to assess the effect of medication on heart rate (HR), left ventricular ejection fraction (LVEF), mass (LVM), end diastolic volume (LVEDV) and ecc with age as a continuous covariate. Results A total of 206 studies from 136 DMD subjects were included in the analysis. Group A (114 studies) was younger than Group B (92 studies)(10±2.4 vs. 12.4±3.2 years, p < 0.0001). However, HR, LVEF, LVEDV and LVM were not different between the two groups (Table 1). ecc magnitude was significantly lower in Group B (-13.8±1.9 vs -12.8 ±2.0, p= 0.0004), but age correction using covariance analysis eliminated this effect (Table 2). After mean follow-up of 15 months, ecc of neither group improved compared with baseline (Table 3). Conclusions In a large consecutive database of DMD patients, serial CMR studies demonstrate that current and standard treatment strategy at our institution has little effect on DMD-associated cardiac disease. The failure of current regimens supports the need for rigorous prospective clinical trials to identify effective treatment regimens. (Table Presented0.