Management of cardiac involvement in muscular dystrophies: paediatric versus adult forms.

ABSTRACT: Muscular dystrophies are a group of genetic disorders characterized by muscle degeneration and consequent substitution by fat and fibrous tissue. Cardiac involvement is an almost constant feature in a great part of these diseases, as both primary myocardial involvement and secondary involvement due to respiratory insufficiency, pulmonary hypertension or reduced mobility. Primary myocardial involvement usually begins more precociously compared to the secondary involvement. In fact the first signs of cardiomyopathy can be observed in the first decade of life in muscular dystrophies with childhood onset and later in adult form of muscular dystrophies as myotonic dystrophy type 1. At least an annual cardiac follow-up is recommended in these patients including clinical and instrumental examinat

SUBMITTER: Palladino A 

PROVIDER: S-EPMC5416740 | biostudies-literature | 2016 Dec

REPOSITORIES: biostudies-literature