Patterns of cardiac involvement in different muscular dystrophies assessed by magnetic resonance imaging. (February 2023)

• Record Type: Journal Article
• Authors: Goebel, Juliane; Schult, Karolin; Schara, Ulrike; Neudorf, Ulrich; Forsting, Michael; Schlosser, Thomas; Nassenstein, Kai
• Is Part Of: Acta radiologica. Volume 64:Number 2(2023)
• Page Start: 605
• Page End: 611
• Publication Date: 2023-02
• Subjects: Muscular dystrophy -- Duchenne -- Becker -- limb girdle -- magnetic resonance imaging -- heart; Radiology, Medical -- Periodicals; Radiography, Medical -- Periodicals; Radiotherapy -- Periodicals; 616.0757
• DOI: 10.1177/02841851221077402
• Languages: English
• ISSNs: 0284-1851

Abstract

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.