Of 50 during a median follow-up of five years (comprising nine deaths and eight

Of 50 during a median follow-up of five years (comprising nine deaths and eight heart transplantations) [8]. Obviously, the patients in the study of Diegoli et al. suffered from more advanced cardiomyopathy with established DCM and a mean LV-EF of 30 ?11 only compared to a mean LV-EF of 53 ?14 in our study group. In contrast, Schram et al. studied a relatively young DMD patient group (9 ?4 yrs) with normal or only mildly impaired LVEF at baseline and evaluated the role of prophylactic steroid therapy on mortality and cardiovascular outcomes. The authors reported no deaths in the first five years of follow-up. Moreover, 96 of their patients demonstrated a preserved LV-EF of 45 at 5-year follow-up [21]. Taken together, these data clearly illustrate the (age-dependent) association between the degree of LV systolic dysfunction and the occurrence of adverse cardiac events in MD patients.A new prognostic indicator: presence of “transmural LGE”Whereas the association of a reduced LV-EF with the occurrence of adverse cardiac events was shown for ischemic as well as different non-ischemic cardiomyopathies, the unique finding of the present study was obtained when LGE characteristics (including LGE presence, extent and pattern) were carefully looked at: A second independent predictor for adverse cardiac events was the presence of a transmural pattern of LGE (assessed as a dichotomous parameter) – but not the categorical presence of LGE per se PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/27385778 or the extent of LGE. At first view, this finding seems to be in disagreement with previously established data in other non-ischemic cardiomyopathies, e.g. DCM, myocarditis or hypertrophic cardiomyopathy; in these forms of cardiac disease the qualitative presence of any LGE (without further quantification or classification) was suggested to be of prognostic value [22-25]. However, a possible explanation for our different finding may be due to the different timing of focal myocardial fibrosis occurrence (depicted by LGE-CMR in the disease course) in different forms of non-ischemic cardiomyopathies. While in our DMD/BMD study group presence of LGE was depicted in 64 of cases and preceded LV-EF decline, e.g. Gulati et al. showed presence of intramural LGE in (only) 30 of DCM patients [26]. Moreover, those LGE-positive DCM patients showed significantly more severe LV adverse remodelling than DCM patients without LGE suggesting that LV dysfunction supposedly precedes occurrence of LGE in (idiopathic) DCM [26]. Furthermore, when looking at biopsy proven viral myocarditis, patients frequently show subepicardial or intramural patterns of LGE (similar to DMD/BMD ones) and presence of LGE was suggested to be the best independentpredictor of cardiac mortality [22,27,28]. However, in case of (viral) myocarditis, a subepicardial pattern of LGE is frequently observed in the LV free wall in the acute phase with a shrinkage of LGE (or sometimes even a complete disappearance) in the further disease course that is explained by resolution of myocardial inflammation. Hence, timing of CMR is critical in order to depict the total extent of myocardial inflammation/damage in the acute phase. Moreover, LGE in DMD/BMD patients is caused by progressive myocardial fibrosis as a consequence of ongoing cardiomyocyte cell death due to dystrophindeficiency. Consequently, naturally occurring LGE does not disappear in DMD/BMD patients and is rather CEP-37440 side effects extending in the further disease course with a continuous shift in the pattern from sub.