J 2019

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

PANOVSKÝ, Roman, Martin PEŠL, Tomáš HOLEČEK, Jan MÁCHAL, Věra FEITOVÁ et. al.

Basic information

Original name

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

Authors

PANOVSKÝ, Roman (203 Czech Republic, guarantor, belonging to the institution), Martin PEŠL (203 Czech Republic, belonging to the institution), Tomáš HOLEČEK (203 Czech Republic), Jan MÁCHAL (203 Czech Republic, belonging to the institution), Věra FEITOVÁ (203 Czech Republic), Lenka MRÁZOVÁ, Jana HABERLOVÁ (203 Czech Republic), Alžběta SLABÁ (203 Czech Republic), Pavel VÍT (203 Czech Republic), Veronika STARÁ (203 Czech Republic) and Vladimír KINCL (203 Czech Republic, belonging to the institution)

Edition

ORPHANET JOURNAL OF RARE DISEASES, LONDON, BMC, 2019, 1750-1172

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30201 Cardiac and Cardiovascular systems

Country of publisher

United Kingdom of Great Britain and Northern Ireland

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

Impact factor

Impact factor: 3.523

RIV identification code

RIV/00216224:14110/19:00109165

Organization unit

Faculty of Medicine

DOI

UT WoS

000455361900001

Keywords in English

Cardiac magnetic resonance Duchene muscular dystrophy T1 mapping; extracellular volume Cardiomyopathy

Tags

Tags

International impact, Reviewed
Změněno: 15/4/2019 13:51, Soňa Böhmová

Abstract

V originále

BackgroundThe 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.MethodsThe 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).ResultsCompared 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 (104131ms and 1043 +/- 37ms vs. 983 +/- 15ms, 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.