*6.1. Early DCM*

This group includes all cases in which LVEF is normal or ≥50%. At this stage of the disease, the aim is to delay the onset of ventricular dysfunction. Because of lack of specific therapy for DMD DCM, 2018 DMD Care Considerations [4] recommend traditional first line HF with ACE-I or angiotensin receptor blockers (ARBs).

In 2005, Duboc [50] for the first time reported a two-phase study conducted over five years for the prophylactic use of perindopril for DMD-DCM. This study was designed to evaluate the effect of perindopril on the development and progression to LV dysfunction. In a multicenter study, 57 children aged 9.5 to 13 years with normal cardiac examination and LVEF of more than 55% at baseline as measured by radionuclide ventriculography, were randomized to perindopril 2–4 mg versus placebo. Chi-squared analysis showed a significant benefit for patients treated in order to prevent the progression of DCM, defined as reduction of LVEF below 45%. After this study, ACEi have been prescribed for prevention.
