TY - JOUR
T1 - Importance of CMR within the task force criteria for the diagnosis of ARVC in children and adolescents
AU - Etoom, Yousef
AU - Govindapillai, Sindu
AU - Hamilton, Robert
AU - Manlhiot, Cedric
AU - Yoo, Shi Joon
AU - Farhan, Maryam
AU - Sarikouch, Samir
AU - Peters, Brigitte
AU - McCrindle, Brian W.
AU - Grosse-Wortmann, Lars
N1 - Funding Information:
The study was approved by the Research Ethics Board of the Hospital for Sick Children, Toronto, Ontario, Canada. The study was performed at the Hospital for Sick Children. This work was supported in part through the University of Toronto ‘Comprehensive Research Experience for Medical Students (CREMS)’ Program and by the German Competence Network for Congenital Heart Defects, funded by the German Federal Ministry of Education and Research (BMBF) ( FKZ 01G10210 , 01GI0601 ). All authors have reported that they have no relationships relevant to the contents of this paper to disclose. Drs. Etoom and Govindapillai contributed equally to this work.
Publisher Copyright:
© 2015 American College of Cardiology Foundation.
PY - 2015/3/17
Y1 - 2015/3/17
N2 - 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. Objectives 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.
AB - 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. Objectives 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.
KW - SD scores
KW - cardiac magnetic resonance imaging
KW - fibrosis
KW - wall motion
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U2 - 10.1016/j.jacc.2014.12.041
DO - 10.1016/j.jacc.2014.12.041
M3 - Article
C2 - 25766945
AN - SCOPUS:84924388837
SN - 0735-1097
VL - 65
SP - 987
EP - 995
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
IS - 10
ER -