Prospective Becker-Heart-Study

Prospective Cohort Study of Patients With Mutations in the Dystrophin Gene (X Linked Dilated Cardiomyopathy and Becker Muscular Dystrophy)

The purpose of this study is to determine whether electrocardiogram, echocardiography, cardiac MRI, sera biomarkers can improve early detection of myocardial involvement and clinical outcome.

A cohort of 100 patients with mutations in the dystrophin gene associated with Becker muscular dystrophy and/or dilated cardiomyopathy will be included (patients with Duchenne muscular dystrophy are excluded).

Patients with undergo at baseline the following workups: electrocardiogram, echocardiography, cardiac MRI, sera biomarkers measurement.

At 3 years and 5 years, patients will be investigated according to the same protocol and occurrence of cardiac adverse events in the meanwhile will be recorded.

Statistical analysis will assess correlations between cardiac phenotype and DMD mutations and prognostic value of cardiac investigations.

Inclusion Criteria:

• mutation in the DMD gene
• Becker muscular dystrophy and/or dilated cardiomyopathy
• age>18 years
• affiliation to the French medical insurance

Exclusion Criteria:

• Duchenne muscular dystrophy
• Any other chronic disease that may be associated with heart disease