USE OF ULTRASOUND TISSUE CHARACTERIZATION TO DETECT PRECLINICAL MYOCARDIAL STRUCTURAL CHANGES IN PATIENTS AFFECTED BY DUCHENNE AND BECKER MUSCULAR DYSTROPHY

Diagnosis of subclinical cardiomyopathy and his correlation with deletion of o specific region of the dystrophin gene will be of advantage in performing an early and adeguate pharmacological treatment, also in the pediatric age and conseguently modifying, the evolution of the cardiomyopathy. Although some potential correlation between genotype and phenotype have emerged, the precise relationship between dystrophin mutations and cardiomyopathy is still not entirely clear.The main objectives of our project may be resumes as follow;a) to define the early heart involvement and the evolution of myocardial fibrosis, in patients with Duchenne and Becker muscular dystrophy;b) to correlate the cardiac phenotype (regard the onset and evolution of cardiomyopathy) with type of deletion of dystrophin gene, particularly in patients affected by Becker Muscular Dystrophy.Ultrasound Tissue Characterisation analysis will be perform in children adolescents and adults patients with Duchenne and Becker muscular dystrophy with normal cardiac function, for to evaluate during three years of follow-up, if are present early signs of cardiac involvement and if there are correlation of UTC data with type of deletion and mutation of dystrophin gene.