Two center study to assess the functional relevance of myocardial fibrosis in muscular dystrophy patients with and witho
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BioMed Central
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Two center study to assess the functional relevance of myocardial fibrosis in muscular dystrophy patients with and without left ventricular systolic dysfunction Joseph J Suttie*1, Ali Yilmaz2, Sairia Dass1, Theodoros D Karamitsos1, Jane M Francis1, Colin Forfar1, David Hilton-Jones1, Hugh Watkins1, Stefan Neubauer1, Steffen E Petersen1 and Udo Sechtem2 Address: 1Oxford Centre for Clinical Magnetic Resonance Research, Department of Cardiovascular Medicine, Oxford University, Oxford, UK and 2Division of Cardiology of the Robert-Bosch-Krankenhaus, Stuttgart, Germany * Corresponding author
from 13th Annual SCMR Scientific Sessions Phoenix, AZ, USA. 21-24 January 2010 Published: 21 January 2010 Journal of Cardiovascular Magnetic Resonance 2010, 12(Suppl 1):P260
doi:10.1186/1532-429X-12-S1-P260
Abstracts of the 13th Annual SCMR Scientific Sessions - 2010
Meeting abstracts - A single PDF containing all abstracts in this Supplement is available here. http://www.biomedcentral.com/content/files/pdf/1532-429X-11-S1-infoThis abstract is available from: http://jcmr-online.com/content/12/S1/P260 © 2010 Suttie et al; licensee BioMed Central Ltd.
Background 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 In this two center study, 25 patients (median age 40 yrs, range 11 to 60) with skeletal muscle biopsy and/or genetically detected dystrophin abnormalities were prospectively recruited from the Division of Cardiology of the Robert-Bosch-Krankenhaus, Stuttgart, Germany and the John Radcliffe Hospital, Oxford, UK. CMR was performed using either a Siemens Magnetom Sonata 1.5-T (Erlangen,
walls axis Short Figure 1 slice showing LGE in the lateral and inferolateral Short axis slice showing LGE in the lateral and inferolateral walls. The unaffected reion in the septum provided the reference pont for LGE quantitative analysis.
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Journal of Cardiovascular Magnetic Resonance 2010, 12(Suppl 1):P260
http://jcmr-online.com/content/12/S1/P260
Figurein2patients with reduced LVEF (< 60%) %LGE %LGE in patients with reduced LVEF (< 60%).
Germany) or 3-T Siemens Tim Trio (Erlangen, Germany). Anatomical, functional and
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