Left ventricular noncompaction in Duchenne muscular dystrophy. J Cardiovasc Magn Reson 2013 Aug 01;15(1):67
Date
08/07/2013Pubmed ID
23914774Pubmed Central ID
PMC3750745DOI
10.1186/1532-429X-15-67Scopus ID
2-s2.0-84881121835 (requires institutional sign-in at Scopus site) 36 CitationsAbstract
BACKGROUND: Left ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function.
METHODS: Cardiac magnetic resonance (CMR) was used to assess ventricular morphology and function in 151 subjects: DMD with ejection fraction (EF) > 55% (n = 66), DMD with EF < 55% (n = 30), primary LVNC (n = 15) and normal controls (n = 40). The non-compacted to compacted (NC/C) ratio was measured in each of the 16 standard myocardial segments. LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.
RESULTS: LVNC criteria were met by 27/96 DMD patients (prevalence of 28%): 11 had an EF > 55% (prevalence of 16.7%), and 16 had an EF < 55% (prevalence of 53.3%). The median maximum NC/C ratio was 1.8 for DMD with EF > 55%, 2.46 for DMD with EF < 55%, 1.54 for the normal subjects, and 3.69 for primary LVNC patients. Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.
CONCLUSION: The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.
Author List
Statile CJ, Taylor MD, Mazur W, Cripe LH, King E, Pratt J, Benson DW, Hor KNMESH terms used to index this publication - Major topics in bold
Chi-Square DistributionChild
Contrast Media
Female
Gadolinium DTPA
Humans
Isolated Noncompaction of the Ventricular Myocardium
Magnetic Resonance Imaging
Male
Muscular Dystrophy, Duchenne
Prevalence
Statistics, Nonparametric
Vectorcardiography