When PPMD convened the Contemporary Issues in Duchenne Cardiology meeting with National Heart, Lung, and Blood Institute (NHLBI) in July 2014, there were several recommendations.
The strongest recommendations included the use of cardiac MRI as the gold standard for cardiac surveillance and starting ACE inhibitors with either the the first findings of cardiac fibrosis or dysfunction (or by age 10yo if findings remained normal).
The two-year study described in a new paper titled "Myocardial Fibrosis Progression in Duchenne and Becker Muscular Dystrophy" substantiates those recommendations. The randomized study of 76 Duchenne (70) and Becker (6) patients showed the importance of cardiac MRI in detecting cardiac fibrosis and demonstrated that starting ACE inhibitor therapy significantly slowed the progression of fibrosis, thus preserving cardiac function longer.
Because fibrosis occurs much sooner than cardiac dysfunction, and because it is not possible to see fibrosis by imaging the heart with echosonography, we hope that this paper will further validate the need for at least annual cardiac surveillance using MRI in patients living with Duchenne or Becker.