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Cardiac profile of the Czech population of Duchenne muscular dystrophy patients: a cardiovascular magnetic resonance study with T1 mapping
- 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
- Subjects :
- 0301 basic medicine
musculoskeletal diseases
Male
medicine.medical_specialty
Adolescent
Cardiac magnetic resonance
Cardiomyopathy
Duchenne muscular dystrophy
Population
lcsh:Medicine
Gadolinium
030105 genetics & heredity
03 medical and health sciences
0302 clinical medicine
Fibrosis
Internal medicine
medicine
T1 mapping
extracellular volume
Humans
Pharmacology (medical)
Forty Nine
Muscular dystrophy
10. No inequality
education
Genetics (clinical)
Czech Republic
education.field_of_study
business.industry
Research
lcsh:R
General Medicine
medicine.disease
Magnetic Resonance Imaging
Muscular Dystrophy, Duchenne
Cardiology
Myocardial fibrosis
business
Cardiomyopathies
030217 neurology & neurosurgery
Duchene muscular dystrophy
Subjects
Details
- ISSN :
- 17501172
- Volume :
- 14
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Orphanet journal of rare diseases
- Accession number :
- edsair.doi.dedup.....305101ccf9d9880c0409ad3d2ffe112e