Abstract
Myotonic dystrophy type 1 (MD), also known as Steinert's disease, is the most autosomal dominant muscular dystrophy in adults with a prevalence of 1 to 8000 individuals [ [1] Pelargonio G. Dello Russo A. Sanna T. De Martino G. Bellocci F. Myotonic dystrophy and the heart. Heart. Dec 2002; 88: 665-670 Crossref PubMed Scopus (154) Google Scholar ]. This disease is characterized by a multisystem disorder. MD is caused by a CTG expansion in the 3′-untranslated region of a protein kinase gene. This CTG trinucleotide is located in the chromosome region 19q13.3. In MD, RNA sequesters splicing regulators of genes. Cardiovascular disease is one of the most prevalent causes of death in MD patients [ [1] Pelargonio G. Dello Russo A. Sanna T. De Martino G. Bellocci F. Myotonic dystrophy and the heart. Heart. Dec 2002; 88: 665-670 Crossref PubMed Scopus (154) Google Scholar ]. The RV function is a determinant parameter of clinical outcome in heart failure. No data have been reported about RV function in Steinert's disease. We sought to assess RV cardiac function in MD patients using tissue Doppler imaging (TDI) and TAPSE measurement with echocardiography–Doppler.
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