And arrhythmias has become an important cause of morbidity and mortality [1,4,8-10]. In the last years, cardiovascular magnetic resonance (CMR) has been increasingly used for diagnosis as well as for follow-up of cardiac involvement in DMD/BMD patients [11-14]. CMR does not only offer an accurate and reproducible tool for LV systolic function assessment, but also the possibility of noninvasive myocardial tissue characterization and early fibrosis detection based on late gadolinium enhancement (LGE) imaging. Nevertheless, published data on the risk stratification as well as on the prognostic role of CMR in DMD/BMD-related purchase MS023 cardiomyopathy are scarce [8]. With this study, we aimed at elucidating the relationship between the phenotype of cardiac involvement (assessed by CMR) and the occurrence of death and adverse cardiac events in DMD/BMD patients. MethodsStudy populationpatients (20 DMD and 68 BMD) underwent a complete CMR study at study inclusion and represented the final study group. The study protocol complies with the Declaration of Helsinki and was approved by the local ethics committee. Informed consent was obtained from the patients prior to study inclusion.CMR data acquisitionECG-gated CMR studies were performed on 1.5-T scanners (Aera, Siemens Medical Solutions, Erlangen, PubMed ID: Germany and Achieva, Philips, Best, The Netherlands) using commercially available cardiac software, electrocardiographic triggering, and cardiac-dedicated surface coils. Cine-imaging was performed using a steady-statefree-precession (SSFP) sequence in three long-axis slices (four-, three- and two-chamber) and a stack of shortaxis slices completely covering the LV. LGE-imaging was performed using a T1-weighted inversion recovery gradient-echo sequence 10-15 min after intravenous contrast administration (0.15 mmol/kg Magnevist? in the same imaging planes as the cine-images.CMR data analysisNinety male patients with known muscular dystrophy (MD) were prospectively enrolled in two German centres (Robert-Bosch-Krankenhaus, Stuttgart and Universit sklinikum Muenster, Muenster) between 2006 and 2013. A diagnosis of muscular dystrophy was previously made in a specialized neurology centre based on skeletal muscle pathology with immunohistochemical dystrophin analyses and/or genetic testing [2]. Eighty-eight of theseCMR analysis was performed off-line by two experienced readers (AF and AY) blinded to outcomes and clinical characteristics. Ventricular volumes, ejection fraction and LV mass were derived by contouring endo- and epicardial borders on the short-axis cine images and indexed to body surface area. LGE presence and pattern were first visually assessed on the short-and long-axis images by using a model dividing each short xis slice into 12 sectors and each sector into 3 equal circumferential segments (subendocardial, midmyocardial, subepicardial; in total 36 segments per slice). A LV ejection fraction (EF) less than 55 and/or presence of LGE in at least one myocardial segment were considered signs of cardiac involvement. LGE pattern was globally assessed by two readers (AF and AY) and classified according to its location as: subepicardial, intramural, mixed (subepicardial and intramural). In addition to the three patterns, a fourth transmural pattern was considered whenever all three segments in at least one sector were LGE positive. Eventual discrepancies were resolved by common agreement. Second, LGE extent was planimetered on the short-axis contrast images with.