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A Florian, M Bietenbeck, C Meier, S Roesch, U Sechtem, A Yilmaz, P3168
Progression of cardiomyopathy in patients with muscular dystrophy - a CMR-based extensive follow-up study, European Heart Journal, Volume 39, Issue suppl_1, August 2018, ehy563.P3168, https://doi.org/10.1093/eurheartj/ehy563.P3168Close - Share Icon Share
Extract
Background: Becker (BMD) and Duchenne (DMD) muscular dystrophies (MD) are X-linked progressive neuromuscular disorders. Cardiac involvement with a non-ischemic, myocarditis-like pattern of left ventricular (LV) myocardial fibrosis leading to dilated cardiomyopathy is a major cause of morbidity and mortality. Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) is increasingly used for (initial) diagnosis as well as for subsequent surveillance of cardiac disease in MD patients. However, CMR-based follow-up data are still limited in MD patients.
Methods: Between 2007 and 2016, n=112 DMD/BMD males underwent comprehensive CMR studies including (amongst others) cine- and LGE-imaging. In n=83 of these patients (31±14 years; 22% DMD and 78% BMD) at least one follow-up (FU) CMR study was performed.
Results: CMR parameters at baseline were as follow: LV-EDVi = 81±25 ml/m2, LV-EF = 55±11%, LV-mass = 58±16 g/m2, and n=54 (65%) MD patients were LGE-positive. After a median FU period of 6 years (range 1 to 10 years), there was an increase in LV-EDV to 87±25 ml/m2 (p=0.004 vs. baseline), a decrease in LV-EF to 52±11% (p<0.001 vs. baseline) and in LV-mass to 55±14 g/m2 (p=0.024 vs. baseline). The proportion of MD patients with an impaired LV function (LV-EF <55%) increased from 45 to 49% (p=NS) and those with a LV-EF ≤35% from 6 to 8% (p=NS). At FU, the prevalence of LGE-positive MD patients significantly increased to 78% (p=0.08 vs. baseline). In the n=54 LGE-positive MD patients at baseline, median LGE extent (%LV-mass) increased from 9% (1 to 44%) to 11% (1 to 40%) (p<0.001) and the number of LGE-positive segments increased from 4 (1 to 13) to 7 (1 to 16) (p<0.001). The prevalence of a transmural pattern of LGE increased from 28% to 46% in these patients (p=0.023). Those n=11 initially LGE-negative MD that developed LGE at follow-up showed a median LGE extent of 2% (0.5 to 15) and 3 (1 to 9) segments, whereas none of them presented a transmural LGE pattern.
