title: Left ventricular noncompaction in Duchenne muscular dystrophy.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. Christopher.statile@cchmc.org
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Statile
authors.forename: Michael D
authors.initials: MD
authors.lastname: Taylor
authors.forename: Wojciech
authors.initials: W
authors.lastname: Mazur
authors.forename: Linda H
authors.initials: LH
authors.lastname: Cripe
authors.forename: Eileen
authors.initials: E
authors.lastname: King
authors.forename: Jesse
authors.initials: J
authors.lastname: Pratt
authors.forename: D Woodrow
authors.initials: DW
authors.lastname: Benson
authors.forename: Kan N
authors.initials: KN
authors.lastname: Hor
mesh_terms: D016009:Chi-Square Distribution; D002648:Child; D003287:Contrast Media; D005260:Female; D019786:Gadolinium DTPA; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D015995:Prevalence; D018709:Statistics, Nonparametric; D014672:Vectorcardiography
chemical_list: D003287:Contrast Media; D019786:Gadolinium DTPA
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