Comparison of the effect of three steroid regimens on cardiac function in Duchenne muscular dystrophy

Abstract

BackgroundSteroid therapy of patients with Duchenne muscular dystrophy (DMD) delays the onset of cardiac dysfunction. ObjectiveThe aim of this study was to compare the effects of 3 different steroid regimens on cardiac function assessed by echocardiography. MethodsPatients diagnosed with DMD, between 8 and 23 years of age were reviewed between January 2000 and September 2018. Participants were classified into four groups. Control group (no steroid treatment), Prednisone 10 mg/kg/weekend day, Prednisone 0.40 mg - 0.75 mg/kg/day and Deflazacort 0.9 mg/kg/day.Demographic data, echocardiographic measurements of shortening fraction (FS) and left ventricular end diastolic diameter z-score were collected and compared between cohorts. ResultsDevelopment of cardiac dysfunction in groups receiving prednisone daily or 3 day prednisone was less common in comparison to the control group (p = 0.005). No patient on the Deflazacort group had abnormal left ventricular FS. There was no difference in the age of initiation of steroid therapy among groups (p = 0.08). Age of development of systolic dysfunction by echocardiography between daily prednisone and weekend prednisone was not significantly different (p = 0.07). There was no difference in the number of patients developing of cardiac dysfunction between patients receiving prednisone daily or 3 day prednisone (p = 0.4). The entire cohort (77 patients) demonstrated a decline in the cardiac function over time. ConclusionsOur study confirms the delay in development of left ventricular dysfunction related to steroid therapy. Deflazacort seems to show a significant beneficial effect on cardiac function, compared to daily and weekend prednisone therapies.