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

M. Josefina Sanchez, William Scott, Franco Pessana, Diana P Castro, Claudio Ramaciotti

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Steroid therapy of patients with Duchenne muscular dystrophy (DMD) delays the onset of cardiac dysfunction. Objective: The aim of this study was to compare the effects of 3 different steroid regimens on cardiac function assessed by echocardiography. Methods: Patients 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. Results: Development 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. Conclusions: Our 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.

Original languageEnglish (US)
Article number101557
JournalProgress in Pediatric Cardiology
Volume67
DOIs
StatePublished - Dec 2022

Keywords

  • Cardiomyopathy
  • Duchenne muscular dystrophy
  • Heart failure
  • Steroids

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

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