PMID- 24686257 OWN - NLM STAT- MEDLINE DCOM- 20150511 LR - 20160511 IS - 2047-2412 (Electronic) IS - 2047-2404 (Linking) VI - 15 IP - 9 DP - 2014 Sep TI - Myocardial fibrosis imaging based on T1-mapping and extracellular volume fraction (ECV) measurement in muscular dystrophy patients: diagnostic value compared with conventional late gadolinium enhancement (LGE) imaging. PG - 1004-12 LID - 10.1093/ehjci/jeu050 [doi] AB - AIM: Cardiac involvement with progressive myocardial fibrosis leading to dilated cardiomyopathy is a major cause of death in muscular dystrophy patients. Extracellular volume fraction (ECV) measurement based on T1-mapping pre- and post-contrast promises the detection of early 'diffuse' myocardial fibrosis that cannot be depicted by conventional contrast-imaging based on late gadolinium enhancement (LGE). With this study, we evaluated the presence of diffuse myocardial fibrosis in regions of 'normal' (LGE-negative) and 'diseased' (LGE-positive) appearing myocardium as well as its relation to the extent of left ventricular (LV) dysfunction and the occurrence of arrhythmias in Becker muscular dystrophy (BMD) patients. METHODS AND RESULTS: Twenty-seven BMD patients (35 +/- 12 years) and 17 matched male healthy controls (33 +/- 8 years) underwent cardiovascular magnetic resonance (CMR) studies including ECV measurement and LGE-imaging. Ambulatory monitoring of arrhythmic events was performed by means of an external event loop recorder. Twenty BMD patients (74%) demonstrated cardiac involvement as detected by typical inferolateral presence of LGE. Twelve patients (44%) had an impaired LV ejection fraction-all being LGE-positive. Global myocardial ECV was significantly higher in the BMD group (29 +/- 6%) compared with the control group (24 +/- 2%, P = 0.001). Patients with cardiac involvement demonstrated higher global ECV (31 +/- 6%) as well as significantly increased regional ECV not only in LGE-positive segments (34 +/- 6%), but also in LGE-negative segments (28 +/- 6%) compared with BMD patients without cardiac involvement and to controls, respectively (24 +/- 3 and 24 +/- 2%, P = 0.005). Global ECV in patients with cardiac involvement substantially correlated to LV ejection fraction (r = -0.629, P = 0.003) and to the number of LGE-positive segments (r = 0.783, P < 0.001). On univariable analysis, global ECV-but not the categorical presence of LGE per se--was significantly associated with arrhythmic events (OR: 1.97, CI: 32.22-1.21, P = 0.032). CONCLUSION: ECV measurement by CMR is a useful tool in assessing the total extent of myocardial fibrosis as well as in depicting subtle diffuse fibrosis in areas of normal appearing myocardium on LGE-images. Thus, myocardial ECV is a potential additional quantitative tool for accurate detection of cardiac involvement and risk stratification in muscular dystrophy patients. CI - Published on behalf of the European Society of Cardiology. All rights reserved. (c) The Author 2014. For permissions please email: journals.permissions@oup.com. FAU - Florian, Anca AU - Florian A AD - Department of Cardiology and Angiology, University Hospital Munster, Albert-Schweitzer-Campus 1, building A1, 48149 Munster, Germany. FAU - Ludwig, Anna AU - Ludwig A AD - Division of Cardiology, Robert-Bosch-Krankenhaus, Stuttgart, Germany. FAU - Rosch, Sabine AU - Rosch S AD - Division of Cardiology, Robert-Bosch-Krankenhaus, Stuttgart, Germany. FAU - Yildiz, Handan AU - Yildiz H AD - Division of Cardiology, Robert-Bosch-Krankenhaus, Stuttgart, Germany. FAU - Sechtem, Udo AU - Sechtem U AD - Division of Cardiology, Robert-Bosch-Krankenhaus, Stuttgart, Germany. FAU - Yilmaz, Ali AU - Yilmaz A AD - Department of Cardiology and Angiology, University Hospital Munster, Albert-Schweitzer-Campus 1, building A1, 48149 Munster, Germany ali.yilmaz@ukmuenster.de. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140330 PL - England TA - Eur Heart J Cardiovasc Imaging JT - European heart journal. Cardiovascular Imaging JID - 101573788 RN - 0 (Contrast Media) SB - IM MH - Adult MH - Cardiac-Gated Imaging Techniques MH - Cardiomyopathies/*diagnosis/etiology MH - Contrast Media MH - Female MH - Humans MH - *Magnetic Resonance Imaging, Cine MH - Male MH - Muscular Dystrophy, Duchenne/*complications MH - Prospective Studies MH - Stroke Volume MH - Ventricular Dysfunction, Left/*diagnosis/etiology OTO - NOTNLM OT - CMR OT - Cardiomyopathy OT - LGE OT - Mapping OT - Muscular dystrophy EDAT- 2014/04/02 06:00 MHDA- 2015/05/12 06:00 CRDT- 2014/04/02 06:00 PHST- 2014/04/02 06:00 [entrez] PHST- 2014/04/02 06:00 [pubmed] PHST- 2015/05/12 06:00 [medline] AID - jeu050 [pii] AID - 10.1093/ehjci/jeu050 [doi] PST - ppublish SO - Eur Heart J Cardiovasc Imaging. 2014 Sep;15(9):1004-12. doi: 10.1093/ehjci/jeu050. Epub 2014 Mar 30.