title: Early regional myocardial dysfunction in young patients with Duchenne muscular dystrophy.
abstract: BACKGROUND
  In young patients (aged <12 years) with Duchenne muscular dystrophy (DMD), cardiac systolic function is generally described to be within the normal range. Recent studies have suggested the presence of subclinical dysfunction in these young patients using cardiac magnetic resonance imaging, tissue Doppler measurements, and myocardial velocity gradients. The aim of this study was to further assess regional myocardial function in a young group of patients with DMD using myocardial velocity and deformation imaging.
  
  
  METHODS
  Thirty-two patients with DMD (mean age, 7.9 years; range, 3-12 years) and 29 age-matched normal controls were studied with echocardiography. Standard echocardiographic measurements of left ventricular (LV) systolic and diastolic function were performed. Myocardial velocity and deformation data, including peak systolic and early and late diastolic myocardial velocities, peak systolic strain rate (SR), and peak systolic strain (epsilon), were calculated in the radial direction in the inferolateral LV wall and in the longitudinal direction in the interventricular septum, the LV anterolateral wall, and the right ventricular (RV) free wall.
  
  
  RESULTS
  Higher heart rates and increased LV end-systolic dimensions were seen in patients with DMD compared with controls. Significant decreases in radial and longitudinal peak systolic SR, peak systolic epsilon, and peak systolic and early diastolic myocardial velocities were found in the LV inferolateral and anterolateral walls in patients with DMD. No significant differences in longitudinal function could be found in the interventricular septum or in the RV free wall.
  
  
  CONCLUSIONS
  In young patients with DMD who have global normal systolic function, reductions in systolic deformation parameters as well as reduced early diastolic myocardial velocities can be detected in the anterolateral and inferolateral LV walls. The prognostic significance of these findings warrants further longitudinal follow-up.
authors.affiliation: Department of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Luc
authors.initials: L
authors.lastname: Mertens
authors.forename: Javier
authors.initials: J
authors.lastname: Ganame
authors.forename: Piet
authors.initials: P
authors.lastname: Claus
authors.forename: Nathalie
authors.initials: N
authors.lastname: Goemans
authors.forename: Daisy
authors.initials: D
authors.lastname: Thijs
authors.forename: Bénédicte
authors.initials: B
authors.lastname: Eyskens
authors.forename: David
authors.initials: D
authors.lastname: Van Laere
authors.forename: Bart
authors.initials: B
authors.lastname: Bijnens
authors.forename: Jan
authors.initials: J
authors.lastname: D'hooge
authors.forename: George R
authors.initials: GR
authors.lastname: Sutherland
authors.forename: Gunnar
authors.initials: G
authors.lastname: Buyse
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000000981:diagnostic imaging*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000209:etiology*