- Poster presentation
- Open Access
Presence of late gadolinium enhancement in Duchenne muscular dystrophy patients is associated with age and global ventricular function
© Hor et al; licensee BioMed Central Ltd. 2012
- Published: 1 February 2012
- Ejection Fraction
- Cardiac Magnetic Resonance
- Late Gadolinium Enhancement
- Duchenne Muscular Dystrophy
- Cardiac Magnetic Resonance Imaging
The Duchenne muscular dystrophy (DMD) associated cardiac disease results in significant morbidity and mortality usually resulting in death by the second to third decade of life. End-stage cardiac pathology consists of alternating areas of myocyte hypertrophy, atrophy and fibrosis manifesting as late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) evaluation. There has been considerable interest in detecting the presence of LGE, and previous studies have associated LGE with global ventricular dysfunction as assessed by ejection fraction (EF). However, given the genetic origins and underlying pathogenesis of DMD-associated cardiac disease, we hypothesized that LGE is prevalent in patients without overt disease.
CMR studies performed between June 2006 and June 2011 were analyzed. Standard imaging sequences included cine images and LGE sequence of the left ventricle. Global ventricular function was assessed using QMASS® and LGE was assess qualitatively and reported as negative or positive. The presence of LGE was compared to age and EF. Statistical analysis was performed using SAS (version 9.2; SAS Institute Inc, Cary, NC) and reported as percentages and odds ratios (OR) with confidence interval (CI).
LGE Compared to Age and Ejection Fraction
Number of Pts
Age < 10 yrs
Age 10-15 yrs
3.4 (CI 1.8-6.7)
Age > 15 yrs
7.7 (CI 3.4-18.6)
EF > 55%
EF < 55%
18.8 (CI 6.2-57.1
This large series shows a high prevalence of LGE in DMD patients. LGE is strongly associated with age and global dysfunction. Thus it is most common in older patients with reduced EF. However, it is also detected in young DMD patients with normal EF. LGE may be a useful biomarker for detection of progression of DMD-associated cardiac disease. As such, it may have important implications to clinical care and research.
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.