Dystrophinopathies are characterised by impaired cardiac metabolism, contractile dysfunction and fibrosis in patients with and without coxsackie B3 exposure

Inherited dystrophinopathies (Becker and Duchenne muscular dystrophy, and females with heterozygous mutations) have a high rate of myocardial disease with a variable clinical phenotype. We have previously demonstrated that dystrophinopathic patients have significantly impaired myocardial energetics and fibrosis even in the presence of normal left ventricular ejection fraction. Furthermore, Coxsackie B3 induced viral cardiomyopathy has been shown to be a form of acquired dystrophinopathy.