Dystrophinopathies are characterised by impaired cardiac metabolism, contractile dysfunction and fibrosis in patients with and without coxsackie B3 exposure
© Suttie et al; licensee BioMed Central Ltd. 2011
Published: 2 February 2011
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.
We therefore acquired 31-Phosphorus magnetic resonance spectroscopy (MRS), proton MRS and tagging to determine the relationship between energetics, lipidosis, fibrosis and regional systolic myocardial strain in patients with inherited dystrophinopathies with and without prior exposure to Coxsackie B3.
Abnormal myocardial energetics is strongly associated with impaired regional contractility in dystrophinopathies. Myocardial fibrosis, on the other hand, is increased with prior Coxsackie B3 exposure. This provides compelling evidence for a two-hit interaction between inherited and acquired mechanisms of myocardial damage in dystrophinopathies.
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