Two center study to assess the functional relevance of myocardial fibrosis in muscular dystrophy patients with and without left ventricular systolic dysfunction
© Suttie et al; licensee BioMed Central Ltd. 2010
Published: 21 January 2010
Abnormalities of dystrophin expression cause Duchenne Muscular Dystrophy (D) or Becker Muscular Dystrophy (B). Since widespread use of non-invasive respiratory ventilation, many D patients who previously died in their early 20 s from respiratory failure are now surviving longer and developing cardiomyopathy. In B patients, disease progression is slower, however, development of progressive cardiomyopathy is highly frequent. One previously identified predictor of mortality in cardiomyopathy is myocardial fibrosis as detected by late gadolinium enhacement (LGE) cardiovascular magnetic resonance (CMR) imaging. Furthermore, a high percentage and typical inferolateral pattern of LGE has been previously reported in small numbers of patients with B/D, however, the relationship with left ventricular systolic dysfunction needs to be further evaluated.
Methods and Results
Myocardial fibrosis is present in most patients with B/D. There is a subtle association between the extent of myocardial fibrosis and impaired LVEF. Myocardial fibrosis in B/D with normal LVEF suggests that fibrosis is either an early disease manifestation or that other factors are required before progression to dilated cardiomyopathy.
This article is published under license to BioMed Central Ltd.