Acta radiologica (Stockholm, Sweden : 1987) · 2022
Patterns of cardiac involvement in different muscular dystrophies assessed by magnetic resonance imaging.
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Goebel Juliane, Schult Karolin, Schara Ulrike, Neudorf Ulrich, Forsting Michael, Schlosser Thomas, Nassenstein Kai
Аннотация
BACKGROUND: In muscular dystrophies, it is not only skeletal muscles that can be affected, but also the myocardium. This cardiac involvement can represent a major cause of morbidity and mortality.
PURPOSE: To investigate cardiac involvement in Duchenne (DMD), Becker (BMD), and limb girdle muscular dystrophy (LGMD) patients, and carriers of DMD/BMD by cardiac magnetic resonance (CMR) imaging and to search for differences in the pattern of cardiac involvement.
MATERIAL AND METHODS: All patients with genetically or histologically proven DMD, BMD, and LGMD, or confirmed carriers of DMD/BMD who had undergone CMR at our clinic between January 2008 and November 2018 were retrospectively included and re-evaluated for regional and global left ventricular function, increased trabecularization, and late enhancement.
RESULTS: A total of 26 DMD, 10 BMD, 11 LGMD, and seven DMD/BMD carriers were included. Only one carrier of DMD presented with normal CMR results; all other participants showed cardiac abnormalities. Regional wall motion abnormalities (RWMA; prevalence in LGMD patients: 55%) and late enhancement (prevalence in LGMD patients: 82%) were frequent. RWMA were accentuated basal inferolateral in DMD/BMD carriers, while in LGMD they were accentuated apical. In all groups late enhancement was located mainly subepicardial/midmyocardial with a basal inferolateral accentuation. Apart from the different RWMA distribution, no further group-specific differences were found.
CONCLUSION: We found a high rate of cardiac involvement not only in DMD/BMD, but also in LGMD and DMD/BMD carriers with a different RWMA accentuation (apical in LGMD and basal inferolateral in DMD/BMD) as a single group-specific difference.
PURPOSE: To investigate cardiac involvement in Duchenne (DMD), Becker (BMD), and limb girdle muscular dystrophy (LGMD) patients, and carriers of DMD/BMD by cardiac magnetic resonance (CMR) imaging and to search for differences in the pattern of cardiac involvement.
MATERIAL AND METHODS: All patients with genetically or histologically proven DMD, BMD, and LGMD, or confirmed carriers of DMD/BMD who had undergone CMR at our clinic between January 2008 and November 2018 were retrospectively included and re-evaluated for regional and global left ventricular function, increased trabecularization, and late enhancement.
RESULTS: A total of 26 DMD, 10 BMD, 11 LGMD, and seven DMD/BMD carriers were included. Only one carrier of DMD presented with normal CMR results; all other participants showed cardiac abnormalities. Regional wall motion abnormalities (RWMA; prevalence in LGMD patients: 55%) and late enhancement (prevalence in LGMD patients: 82%) were frequent. RWMA were accentuated basal inferolateral in DMD/BMD carriers, while in LGMD they were accentuated apical. In all groups late enhancement was located mainly subepicardial/midmyocardial with a basal inferolateral accentuation. Apart from the different RWMA distribution, no further group-specific differences were found.
CONCLUSION: We found a high rate of cardiac involvement not only in DMD/BMD, but also in LGMD and DMD/BMD carriers with a different RWMA accentuation (apical in LGMD and basal inferolateral in DMD/BMD) as a single group-specific difference.