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Cardiac profile of the Czech population of Duchenne muscular dystrophy patients: a cardiovascular magnetic resonance study with T1 mapping

R. Panovský, M. Pešl, T. Holeček, J. Máchal, V. Feitová, L. Mrázová, J. Haberlová, A. Slabá, P. Vít, V. Stará, V. Kincl,

. 2019 ; 14 (1) : 10. [pub] 20190109

Jazyk angličtina Země Anglie, Velká Británie

Typ dokumentu časopisecké články, práce podpořená grantem

Perzistentní odkaz   https://www.medvik.cz/link/bmc19027921

Grantová podpora
LQ1605 Ministerstvo Školství, Mládeže a Tělovýchovy - International

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.

Citace poskytuje Crossref.org

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$a BACKGROUND: The progressive cardiomyopathy that develops in boys with Duchenne and Becker muscular dystrophy (DMD/BMD) is presumed to be a secondary consequence of the fibrosis within the myocardium. There are only limited data on using parametric imaging in these patients. The purpose of this study was to assess native T1 and extracellular volume (ECV) values in DMD patients. METHODS: The Czech population of males with DMD/BMD was screened. All eligible patients fulfilling the inclusion criteria were included. Forty nine males underwent cardiac magnetic resonance (MR) examination including T1 native and post-contrast mapping measurements. One DMD patient and all BMD patients were excluded from statistical analysis. Three groups were compared - Group D1 - DMD patients without late gadolinium enhancement (LGE) (n = 23), Group D2 - DMD patients with LGE (n = 20), and Group C - gender matched controls (n = 13). RESULTS: Compared to controls, both DMD groups had prolonged T1 native relaxation time. These results are concordant in all 6 segments as well as in global values (1041 ± 31 ms and 1043 ± 37 ms vs. 983 ± 15 ms, both p < 0.05). Group D2 had significantly increased global ECV (0.28 ± 0.044 vs. 0.243 ± 0.013, p < 0.05) and segmental ECV in inferolateral and anterolateral segments in comparison with controls. The results were also significant after adjustment for subjects' age. CONCLUSION: DMD males had increased native T1 relaxation time independent of the presence or absence of myocardial fibrosis. Cardiac MR may provide clinically useful information even without contrast media administration.
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$a Pešl, Martin $u International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. 1st Department of Internal Medicine/Cardioangiology, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic. Department of Biology, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
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$a Holeček, Tomáš $u International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. Department of Medical Imaging, St. Anne's University Hospital, Brno, Czech Republic.
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$a Máchal, Jan $u International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. Department of Pathophysiology, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
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$a Feitová, Věra $u International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. Department of Medical Imaging, St. Anne's University Hospital, Brno, Czech Republic.
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$a Mrázová, Lenka $u Department of Pediatric Neurology, University Hospital Brno, Brno, Czech Republic.
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$a Haberlová, Jana $u Department of Pediatric Neurology, University Hospital Motol, Second Faculty of Medicine, Charles University, Prague, Czech Republic.
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$a Vít, Pavel $u Pediatric Clinic, University Hospital Brno, Brno, Czech Republic.
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$a Stará, Veronika $u Department of Pediatrics, University Hospital Motol, Second Faculty of Medicine, Charles University, Prague, Czech Republic.
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$a Kincl, Vladimír $u International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. 1st Department of Internal Medicine/Cardioangiology, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
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