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  • Open Access

Myocardial fibrosis imaging based on T1-mapping and extracellular volume fraction (ECV) measurement in muscular dystrophy patients: proof of additional diagnostic value compared to conventional late gadolinium enhancement (LGE) imaging

  • 1,
  • 2,
  • 2,
  • 2,
  • 2 and
  • 1, 2
Journal of Cardiovascular Magnetic Resonance201416 (Suppl 1) :P296

https://doi.org/10.1186/1532-429X-16-S1-P296

  • Published:

Keywords

  • Left Ventricular Ejection Fraction
  • Cardiovascular Magnetic Resonance
  • Muscular Dystrophy
  • Late Gadolinium Enhancement
  • Myocardial Fibrosis

Background

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

Twenty-seven BMD patients (35 ± 12 yrs) and 28 matched healthy CONTROLS (33 ± 8 yrs) 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.

Results

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 to the CONTROL group (25 ± 3%, p = 0.005). 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 to BMD patients without cardiac involvement and to CONTROLS, respectively (24 ± 3% and 25 ± 3%, p = 0.01). 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).

Conclusions

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.

Funding

This work was financially supported by a grant from the German Society of Cardiology (DGK; grant-ID DGK12/yilmaz to A.Y.) and by the Robert-Bosch-Foundation (grant-ID KKF-11-14 to A.Y.).

Authors’ Affiliations

(1)
Cardiology and Angiology, University Hospital Münster, Münster, Germany
(2)
Cardiology, Robert-Bosch-Krankenhaus, Stuttgart, Germany

Copyright

© Florian et al.; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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