title: Early Marker of Myocardial Deformation in Children with Duchenne Muscular Dystrophy Assessed Using Echocardiographic Myocardial Strain Analysis.
abstract: OBJECTIVE
  As cardiomyopathy is more prevalent and currently the leading cause of death in Duchenne muscular dystrophy (DMD), early detection of myocardial involvement is important. The purpose of this study was to analyze myocardial strain in DMD children, for the possibility of early detection of myocardial dysfunction.
  
  
  METHODS
  We reviewed medical records of DMD patients who were >10 years of age (15.6±1.6 years, 12.5-18 years), from March 2013 to June 2014. Data of 24 DMD children who underwent echocardiography with three-layer specific myocardial strain were compared with 24 controls (age: 9.3±4.0 years, 5.5-17 years).
  
  
  RESULTS
  Epicardial longitudinal strain was lower in DMD (DMD: -9.3±3.8%; control: -12.3±4.3%; p=0.012). Radial strain (DMD: 24.1±11.1%; control: 37.3±25.9%; p=0.027) and strain rate (SR) (DMD: 1.68±0.91; control: 2.42±0.84; p=0.006) on parasternal short axis view were lower in DMD. Circumferential strains in the endocardium (DMD: -17.5±4.7%; control: -24.2±5.3%; p<0.001), myocardium (DMD: -12.7±3.8%; control: -18.0±4.0%; p<0.001), and epicardium (DMD: -8.4±4.0%; control: -12.2±5.0%; p=0.006) were significantly decreased in DMD. Circumferential SRs were lower in the endocardial (DMD: -1.46±0.38; control: -1.78±0.27; p=0.002) and myocardial layers (DMD: 1.02±0.27; control: -1.28±0.22; p=0.001).
  
  
  CONCLUSIONS
  In DMD patients, deteriorations in myocardial circumferential strain might be an indicator for predicting cardiomyopathy.
authors.affiliation: Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Won Ha
authors.initials: WH
authors.lastname: Jo
authors.affiliation: Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea. lucyeun@yuhs.ac.
authors.forename: Lucy Youngmin
authors.initials: LY
authors.lastname: Eun
authors.affiliation: Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Jo Won
authors.initials: JW
authors.lastname: Jung
authors.affiliation: Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Jae Young
authors.initials: JY
authors.lastname: Choi
authors.affiliation: Department of Rehabilitation Medicine, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Seung Woong
authors.initials: SW
authors.lastname: Gang
mesh_terms: D000293:Adolescent; D009202:Cardiomyopathies / Q000000981:diagnostic imaging* / Q000209:etiology*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D042241:Early Diagnosis; D004452:Echocardiography; D005260:Female; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000000981:diagnostic imaging*; D011237:Predictive Value of Tests
keywords: Duchenne muscular dystrophy; cardiomyopathies; children; myocardial strain