Patients with Duchenne or Becker muscular dystrophy assigned ACE inhibitor therapy had slower progression of myocardial fibrosis compared with patients assigned the usual care, according to new findings.
The researchers randomly assigned 42 patients with muscular dystrophy, myocardial fibrosis and normal ventricular function to receive or not receive ACE inhibitors.
The primary outcome was progression of myocardial fibrosis from baseline to 2 years as assessed by cardiac MR.
In an assessment of 76 patients (mean age, 13 years; 100% male; 92% with Duchenne muscular dystrophy), some of whom were not included in the randomized comparison because they had abnormal ventricular function or no myocardial fibrosis, baseline myocardial fibrosis predicted lower left ventricular ejection fraction at follow-up (coefficient, –0.16; P = .03), Marly Conceição Silva, MD, PhD, from the Heart Institute (InCor), University of São Paulo Medical School, Brazil, and colleagues wrote.
Among those in the randomized comparison, patients assigned ACE inhibitors had slower progression of myocardial fibrosis at 2 years compared with the untreated group (mean increase for intervention group, 3.1% of LV mass; mean increase for control group, 10% of LV mass; P = .001), according to the researchers.
When Silva and colleagues conducted a multivariable analysis, they found treatment with an ACE inhibitor independently predicted decreased progression of myocardial fibrosis (coefficient, –4.51; P = .04).
Among those in the entire cohort, patients with myocardial fibrosis as confirmed by cardiac MR were more likely to have CV events than those who did not (18.2% vs. 0%; log-rank P = .04), the researchers wrote.
In an accompanying editor’s note, Elizabeth M. McNally, MD, PhD, director of the Center for Genetic Medicine at Northwestern University Feinberg School of Medicine and associate editor for translational science of JAMA Cardiology, wrote, “The findings provide support for the concept of pretreating cardiomyopathy before the onset of LV dysfunction.”
Although there has been debate over whether young people with a genetic mutation that puts them at elevated risk for dilated cardiomyopathy and HF should be treated prophylactically, “the present investigation indicates that this issue should be addressed,” McNally wrote. – by Erik Swain
The researchers and McNally report no relevant financial disclosures.