Cardiac profile of the Czech population of Duchenne muscular dystrophy patients: a cardiovascular magnetic resonance stu
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(2019) 14:10
RESEARCH
Open Access
Cardiac profile of the Czech population of Duchenne muscular dystrophy patients: a cardiovascular magnetic resonance study with T1 mapping Roman Panovský1,2* , Martin Pešl1,2,3, Tomáš Holeček1,4, Jan Máchal1,5, Věra Feitová1,4, Lenka Mrázová6, Jana Haberlová7, Alžběta Slabá7, Pavel Vít8, Veronika Stará9 and Vladimír Kincl1,2
Abstract Background: The progressive cardiomyopathy that develops in boys with Duchenne and Becker muscular dystrophy (DMD/BMD) is presumed to be a secondary consequence of the fibrosis within the myocardium. There are only limited data on using parametric imaging in these patients. The purpose of this study was to assess native T1 and extracellular volume (ECV) values in DMD patients. Methods: The Czech population of males with DMD/BMD was screened. All eligible patients fulfilling the inclusion criteria were included. Forty nine males underwent cardiac magnetic resonance (MR) examination including T1 native and post-contrast mapping measurements. One DMD patient and all BMD patients were excluded from statistical analysis. Three groups were compared – Group D1 - DMD patients without late gadolinium enhancement (LGE) (n = 23), Group D2 - DMD patients with LGE (n = 20), and Group C – gender matched controls (n = 13). Results: Compared to controls, both DMD groups had prolonged T1 native relaxation time. These results are concordant in all 6 segments as well as in global values (1041 ± 31 ms and 1043 ± 37 ms vs. 983 ± 15 ms, both p < 0.05). Group D2 had significantly increased global ECV (0.28 ± 0.044 vs. 0.243 ± 0.013, p < 0.05) and segmental ECV in inferolateral and anterolateral segments in comparison with controls. The results were also significant after adjustment for subjects’ age. Conclusion: DMD males had increased native T1 relaxation time independent of the presence or absence of myocardial fibrosis. Cardiac MR may provide clinically useful information even without contrast media administration. Keywords: Cardiac magnetic resonance, Duchene muscular dystrophy, T1 mapping; extracellular volume, Cardiomyopathy
Background Muscular dystrophies mostly affect the skeletal muscles, but in the case of dystrophinopathy such as Duchenne and Becker muscular dystrophy (DMD and BMD), the heart muscle can be also seriously affected and the dystrophin deficiency in the heart manifests as a cardiomyopathy. For a long time, the cardiac impairment was significantly * Correspondence: [email protected] 1 International Clinical Research Center, St. Anne’s University Hospital, Brno, Czech Republic 2 1st Department of Internal Medicine/Cardioangiology, St. Anne’s University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic Full list of author information is available at the end of the article
under-diagnosed for many reasons, even though patients suffer heart failure and also arrhythmic complications. In last years, the strong progress in cardiac management that includes first of all a regular cardiac assessment as well as early therapeutic recomme
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