title: Distribution of Hypertrophy and Late Gadolinium Enhancement in Children and Adolescents with Hypertrophic Cardiomyopathy.
abstract: BACKGROUND
  While well characterized in adult patients, the pattern of hypertrophy and the extent of myocardial scarring in hypertrophic cardiomyopathy (HCM) are insufficiently known. The aim of this study was to assess the hypertrophy patterns and the prevalence and clinical significance of scars in the hearts of young patients with HCM.
  
  
  RESULTS
  A retrospective analysis of the imaging findings of 38 children (aged 12.83 ± 2 years, 30 males) with HCM who underwent cardiac magnetic resonance imaging (CMR) was performed. In addition to left ventricular mass and volumes, the examinations were assessed for the pattern of hypertrophy and presence of late gadolinium enhancement (LGE). A myocardial signal intensity ≥6 standard deviations above the mean of normal myocardium defined positive LGE. Left ventricular mass index averaged 110 ± 34 g/m(2) . Nineteen children (50%) had diffuse septal, 13 (34%) diffuse concentric and 6 (16%) isolated basal hypertrophy. Seven children (18%) had LGE. Patients with LGE had a greater left ventricular mass index than those without (136 ± 34 g/m(2) vs. 104 ± 31 g/m(2) , P = .025). The only two patients who presented with an episode of aborted sudden cardiac death had LGE (P = .03).
  
  
  CONCLUSIONS
  The most common hypertrophy pattern in children with HCM was diffuse septal hypertrophy. The incidence of LGE observed is lower than that reported in adults. The presence of LGE appears to confer a risk for adverse events.
authors.affiliation: Department of Diagnostic Imaging, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.|Department of Paediatrics, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.
authors.forename: Jonathan D
authors.initials: JD
authors.lastname: Windram
authors.affiliation: Department of Paediatrics, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.
authors.forename: Lee N
authors.initials: LN
authors.lastname: Benson
authors.affiliation: Department of Paediatrics, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.
authors.forename: Andreea
authors.initials: A
authors.lastname: Dragelescu
authors.affiliation: Department of Diagnostic Imaging, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.|Department of Paediatrics, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.
authors.forename: Shi-Joon
authors.initials: SJ
authors.lastname: Yoo
authors.affiliation: Department of Paediatrics, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.
authors.forename: Luc
authors.initials: L
authors.lastname: Mertens
authors.affiliation: Department of Diagnostic Imaging, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.|Department of Paediatrics, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.
authors.forename: Derek
authors.initials: D
authors.lastname: Wong
authors.affiliation: Department of Diagnostic Imaging, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.|Department of Paediatrics, Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada.
authors.forename: Lars
authors.initials: L
authors.lastname: Grosse-Wortmann
mesh_terms: D000293:Adolescent; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D002648:Child; D003287:Contrast Media / Q000494:pharmacology; D005260:Female; D005500:Follow-Up Studies; D019786:Gadolinium DTPA / Q000494:pharmacology*; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D009206:Myocardium / Q000473:pathology*; D009864:Ontario / Q000453:epidemiology; D015995:Prevalence; D012189:Retrospective Studies; D014463:Ultrasonography; D016277:Ventricular Function, Left / Q000502:physiology*
chemical_list: D003287:Contrast Media; D019786:Gadolinium DTPA
keywords: Cardiac Magnetic Resonance Imaging; Echocardiography; Gadolinium Delayed Enhancement; Hypertrophic Cardiomyopathy