Corticosteroids improve the efficacy of preventive treatment of left ventricular failure in DMD

A retrospective study of the occurrence of left ventricular damage in 455 boys with Duchenne muscular dystrophy, born between 1982 and 2009 and followed for an average of 10 years with a mean age at last check-up of 14.8 years shows that :

  • 40.7% had left ventricular failure (ejection fraction less than 55% or shortening of ejection fraction less than 28%), which began on average at the age of 14.2 years;
  • 91 (20%) boys received preventive treatment, in 76% of cases with angiotensin-converting enzyme (ACE) inhibitors, starting at a mean age of 12.5 years; less than 20% died during the study, on average at 18.9 years;
  • among boys without prophylactic treatment of LV failure, 8.5% survived to age 25 without signs of LV failure, while 85.7% of those with prophylactic treatment had no LV failure at age 16 and 52.5% at age 26.9;
  • corticosteroid treatment does not alter the age of onset of left ventricular failure, which is also around age 16, as in untreated patients;
  • finally, the onset of left ventricular failure was significantly delayed in the cardiac prophylaxis and corticosteroid group,compared with the other groups.

 

Prophylactic use of cardiac medications for delay of left ventricular dysfunction in Duchenne muscular dystrophy. Conway KM, Thomas S, Ciafaloni E et al. Birth Defects Res. 2023 Oct 18.