Role of CMR Imaging in Diagnostics and Evaluation of Cardiac Involvement in Muscle Dystrophies

Figures

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  Fig. 1. LV dilatation (EDV 201 ml) and severe systolic dysfunction (LVEF 32%) in a 21-year-old patient with DMD. Cine imaging in a long-axis four-chamber view (1a) and short axis (1b) and reduced global longitudinal strain (− 10.6%) (3a). Late Gadolinium Enhancement showing extensive focal non-ischemic enhancement in LV (arrows, 2a-b) corresponding with increased extracellular volume (ECV) in short axis (arrows 3b)

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  Fig. 2. LV dilatation (EDV 205 ml) and mild systolic dysfunction (LVEF 49%) in a 27-year-old patient with BMD. Cine imaging in a long-axis three-chamber view (1a) and corresponding image in late gadolinium enhancement with focal non-ischemic enhancement within inferolateral wall in long (arrows, 1b) and short axes (arrows 1d). Native T1 map with increased T1 native values corresponding to LGE in the short axis (arrows 1c). Green lines: slice location of the short axis

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  Fig. 3. Biatrial dilatation in a 43-year-old patient with EDMD. Cine imaging in a long-axis two- (1a) and four-chamber view (2a). Right atrium 45 cm2. Left atrium 30 cm2, 89 ml/m2

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  Fig. 4. A 45-year-old patient with LGMD2I and normal LV function (LVEF 57%). SSFP cine in a two-chamber view in end-diastole (1a) and end-systole (1b) with no wall motion abnormality, but with detectable myocardial structure abnormality (arrows). A 2-chamber view and a midventricular short axis. Fat/water imaging showing extensive epicardial fat with subepicardial and intramural fatty replacement of the myocardium (arrows 2a, b). Fibrosis imaging (LGE) showing a bright signal—indicating a scar, but the bright signal indicates fatty replacement as well (arrows 3a, b). Only the combination of LGE and fat imaging allows the differentiation of the tissue character

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  Fig. 5. A 57-year-old patient with DM2 and normal LV function (LVEF 58%). Detection of focal non-ischemic enhancement in the LV septum (arrows) in a four-chamber view (1a) and short axis (1b). Late gadolinium enhancement, corresponding with increased extracellular volume (ECV) in a four-chamber view and short axis accordingly (arrows 2a, b)

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  Fig. 6. Examples of free-breathing acquisition in a patient with atrial fibrillation using real-time cine stack acquired in 40 s (1a) and PSIR MOCO LGE SAX stack acquired in about 3 min (1b)