PMID- 30626423 OWN - NLM STAT- MEDLINE DCOM- 20190506 LR - 20200309 IS - 1750-1172 (Electronic) IS - 1750-1172 (Linking) VI - 14 IP - 1 DP - 2019 Jan 9 TI - Cardiac profile of the Czech population of Duchenne muscular dystrophy patients: a cardiovascular magnetic resonance study with T1 mapping. PG - 10 LID - 10.1186/s13023-018-0986-0 [doi] LID - 10 AB - 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. FAU - Panovsky, Roman AU - Panovsky R AUID- ORCID: 0000-0001-8489-132X AD - International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. panovsky@fnusa.cz. AD - 1st Department of Internal Medicine/Cardioangiology, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic. panovsky@fnusa.cz. FAU - Pesl, Martin AU - Pesl M AD - International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. AD - 1st Department of Internal Medicine/Cardioangiology, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic. AD - Department of Biology, Faculty of Medicine, Masaryk University, Brno, Czech Republic. FAU - Holecek, Tomas AU - Holecek T AD - International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. AD - Department of Medical Imaging, St. Anne's University Hospital, Brno, Czech Republic. FAU - Machal, Jan AU - Machal J AD - International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. AD - Department of Pathophysiology, Faculty of Medicine, Masaryk University, Brno, Czech Republic. FAU - Feitova, Vera AU - Feitova V AD - International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. AD - Department of Medical Imaging, St. Anne's University Hospital, Brno, Czech Republic. FAU - Mrazova, Lenka AU - Mrazova L AD - Department of Pediatric Neurology, University Hospital Brno, Brno, Czech Republic. FAU - Haberlova, Jana AU - Haberlova J AD - Department of Pediatric Neurology, University Hospital Motol, Second Faculty of Medicine, Charles University, Prague, Czech Republic. FAU - Slaba, Alzbeta AU - Slaba A AD - Department of Pediatric Neurology, University Hospital Motol, Second Faculty of Medicine, Charles University, Prague, Czech Republic. FAU - Vit, Pavel AU - Vit P AD - Pediatric Clinic, University Hospital Brno, Brno, Czech Republic. FAU - Stara, Veronika AU - Stara V AD - Department of Pediatrics, University Hospital Motol, Second Faculty of Medicine, Charles University, Prague, Czech Republic. FAU - Kincl, Vladimir AU - Kincl V AD - International Clinical Research Center, St. Anne's University Hospital, Brno, Czech Republic. AD - 1st Department of Internal Medicine/Cardioangiology, St. Anne's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic. LA - eng GR - LQ1605/Ministerstvo Skolstvi, Mladeze a Telovychovy/International PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20190109 PL - England TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - AU0V1LM3JT (Gadolinium) SB - IM MH - Adolescent MH - Cardiomyopathies/*diagnostic imaging MH - Czech Republic MH - Gadolinium/analysis MH - Humans MH - Magnetic Resonance Imaging/*methods MH - Male MH - Muscular Dystrophy, Duchenne/*diagnostic imaging PMC - PMC6327529 OTO - NOTNLM OT - Cardiac magnetic resonance OT - Cardiomyopathy OT - Duchene muscular dystrophy OT - T1 mapping; extracellular volume COIS- AUTHORS' INFORMATION: Not applicable. ETHICS APPROVAL AND CONSENT TO PARTICIPATE: The study was performed in accordance with the Declaration of Helsinki (2000) of the World Medical Association, and was approved by the institutional ethics committee (University Hospital Brno, reference number 20130410-03). Written informed consent was obtained from the subjects and/or their legally authorized representative. CONSENT FOR PUBLICATION: No person's personal data are published. COMPETING INTERESTS: The authors declare that they have no competing interests. PUBLISHER'S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2019/01/11 06:00 MHDA- 2019/05/07 06:00 PMCR- 2019/01/09 CRDT- 2019/01/11 06:00 PHST- 2018/10/08 00:00 [received] PHST- 2018/12/21 00:00 [accepted] PHST- 2019/01/11 06:00 [entrez] PHST- 2019/01/11 06:00 [pubmed] PHST- 2019/05/07 06:00 [medline] PHST- 2019/01/09 00:00 [pmc-release] AID - 10.1186/s13023-018-0986-0 [pii] AID - 986 [pii] AID - 10.1186/s13023-018-0986-0 [doi] PST - epublish SO - Orphanet J Rare Dis. 2019 Jan 9;14(1):10. doi: 10.1186/s13023-018-0986-0.