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

Authors :
Alžběta Slabá
Tomáš Holeček
Veronika Stará
Lenka Mrázová
Vladimír Kincl
Jana Haberlová
Martin Pešl
Věra Feitová
Jan Máchal
Pavel Vít
Roman Panovský
Source :
Orphanet Journal of Rare Diseases, Orphanet Journal of Rare Diseases, Vol 14, Iss 1, Pp 1-10 (2019)
Publication Year :
2018

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

Details

ISSN :
17501172
Volume :
14
Issue :
1
Database :
OpenAIRE
Journal :
Orphanet journal of rare diseases
Accession number :
edsair.doi.dedup.....305101ccf9d9880c0409ad3d2ffe112e