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Abstract #2001

Structural and functional myocardial impairments in Becker muscular dystrophy using quantitative cardiac magnetic resonance imaging

Benjamin Marty1,2, Raymond Gilles3, Karim Wahbi4, and Pierre Carlier1,5

1NMR Laboratory, Institute of Myology, NIC, Paris, France, 2NMR Laboratory, CEA, DRF, IBFJ, MIRCen, Paris, France, 3CHWAPI, Tournai, Belgium, 4Institute of Myology, Paris, France, 5NMR Laboratory, CEA, FRF, IBFJ, MIRCen, Paris, France

The management of cardiac involvement is central for Becker muscular dystrophy (BMD) patients since heart failure represents the most frequent cause of death in this population. We performed a comprehensive CMR evaluation of functional and structural myocardial alterations encountered in a cohort of 88 BMD patients. A total of 26% of the BMD patients had a reduced ejection fraction (EF). Globally, native T1, T2 and ECV values were significantly higher in BMD patients than in healthy volunteers, even in sub-clinical phenotypes and correlated with EF. Our results encourage a more systematic inclusion of CMR in the standard of care applied to BMD patients.

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