title: Importance of CMR within the Task Force Criteria for the diagnosis of ARVC in children and adolescents.
abstract: BACKGROUND
  Cardiac magnetic resonance (CMR) is a component of the revised Task Force Criteria (rTFC) for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC). However, its diagnostic value in a pediatric population is unknown.
  
  
  OBJECTIVE
  This study examined the contribution of CMR to diagnosing ARVC using the rTFC in a pediatric population.
  
  
  METHODS
  Clinical CMR studies of 142 pediatric patients evaluated for ARVC between 2005 and 2009 were reviewed. Patients were categorized into "definitive," "borderline," "possible," or "no" ARVC diagnostic groups based on the rTFC. The extent to which each element of the rTFC contributed to diagnosing ARVC was determined using a c-statistics model.
  
  
  RESULTS
  A total of 23 (16%), 32 (23%), 37 (26%), and 50 (35%) patients had definite, borderline, possible, and no ARVC, respectively, applying the rTFC. The prevalence of regional wall motion abnormalities in these groups was 83%, 53%, 22%, and 16%, respectively (p < 0.001). By CMR, right ventricular end-diastolic volumes were 118 ± 31 cc/m², 108 ± 22 cc/m², 94 ± 14 cc/m², and 92 ± 18 cc/m², respectively (p < 0.001). Right ventricular fatty infiltration and fibrosis were detected in only 1 and 3 patients, respectively, all of whom had definitive ARVC. Of all rTFC major criteria, CMR had the largest c-statistic decline (c = -0.163). Eleven of the 23 patients (48%) with definite ARVC would not have been in this group if CMR had not been performed.
  
  
  CONCLUSIONS
  CMR parameters are important contributors to a diagnosis of ARVC in children, using the rTFC. Fatty infiltration and myocardial fibrosis provide limited value in children and adolescents.
authors.affiliation: The Labatt Family Heart Centre, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Yousef
authors.initials: Y
authors.lastname: Etoom
authors.affiliation: The Labatt Family Heart Centre, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Sindu
authors.initials: S
authors.lastname: Govindapillai
authors.affiliation: The Labatt Family Heart Centre, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Robert
authors.initials: R
authors.lastname: Hamilton
authors.affiliation: The Labatt Family Heart Centre, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Cedric
authors.initials: C
authors.lastname: Manlhiot
authors.affiliation: The Labatt Family Heart Centre, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada; Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Shi-Joon
authors.initials: SJ
authors.lastname: Yoo
authors.affiliation: The Labatt Family Heart Centre, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Maryam
authors.initials: M
authors.lastname: Farhan
authors.affiliation: Department of Heart, Thoracic, Transplantation and Vascular Surgery, Hannover Medical School, Hannover, Germany.
authors.forename: Samir
authors.initials: S
authors.lastname: Sarikouch
authors.affiliation: Institute for Biometry and Medical Informatics, University of Magdeburg, Magdeburg, Germany.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Peters
authors.affiliation: The Labatt Family Heart Centre, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
authors.affiliation: The Labatt Family Heart Centre, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada; Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Canada. Electronic address: lars.grosse-wortmann@sickkids.ca.
authors.forename: Lars
authors.initials: L
authors.lastname: Grosse-Wortmann
mesh_terms: D000293:Adolescent; D026683:Advisory Committees; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000000981:diagnostic imaging; D002648:Child; D005260:Female; D006801:Humans; D016015:Logistic Models; D008279:Magnetic Resonance Imaging / Q000592:standards*; D008297:Male; D017410:Practice Guidelines as Topic; D011237:Predictive Value of Tests; D013318:Stroke Volume; D014463:Ultrasonography; D016278:Ventricular Function, Right
keywords: SD scores; cardiac magnetic resonance imaging; fibrosis; wall motion