title: Left Ventricular Function in Children and Adolescents With Arrhythmogenic Right Ventricular Cardiomyopathy.
abstract: The aim of this study was to determine if left ventricular (LV) contractility is reduced in children with arrhythmogenic right ventricular cardiomyopathy (ARVC). For this retrospective study, children and adolescents undergoing a workup for ARVC were characterized according to the revised Task Force Criteria (rTFC). LV strain, rotation, and torsion were measured by feature-tracking cardiovascular magnetic resonance imaging (CMR). Of 142 pediatric patients, 41% had no, 23% possible, 20% borderline, and 16% definite ARVC. LV ejection fraction (EF) did not differ between rTFC categories. Patients in higher rTFC categories had lower right ventricular (RV) EF z-scores (Z-), higher Z-RV end-diastolic volumes (EDVs) and larger Z-LVEDVs (p <0.001, p = 0.002 and 0.013, respectively). LV global circumferential strain was lower in higher rTFC categories (p = 0.018). Z-LVEDV correlated with Z-RVEDV, and Z-LVEF correlated with Z-RVEF (r = 0.69 and r = 0.55, both p <0.001). Z-LVEF and Z-RVEF correlated with LV global circumferential strain (r = 0.48 and r = 0.46, both p <0.001). Forty-eight patients (34%) underwent follow-up CMR investigations after a mean of 3.2 ± 1.9 (0.4 to 8.4) years. A decrease of Z-LVEF over time correlated with that of Z-RVEF (r = 0.35), and Z-LVEDV increase correlated with Z-RVEDV increase (r = 0.57). In conclusion, LV myocardial dysfunction is present in young patients with suspected ARVC. Progressive LV dysfunction assessed by conventional CMR and feature-tracking and enlargement over time parallel adverse remodeling of the RV.
authors.affiliation: Department of Paediatrics, Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Paweena
authors.initials: P
authors.lastname: Chungsomprasong
authors.affiliation: Department of Paediatrics, Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Robert
authors.initials: R
authors.lastname: Hamilton
authors.affiliation: Department of Paediatrics, Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Wietske
authors.initials: W
authors.lastname: Luining
authors.affiliation: Department of Paediatrics, Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Meena
authors.initials: M
authors.lastname: Fatah
authors.affiliation: Department of Paediatrics, Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Shi-Joon
authors.initials: SJ
authors.lastname: Yoo
authors.affiliation: Department of Paediatrics, Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: lars.grosse-wortmann@sickkids.ca.
authors.forename: Lars
authors.initials: L
authors.lastname: Grosse-Wortmann
mesh_terms: D000293:Adolescent; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000000981:diagnostic imaging* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D012189:Retrospective Studies; D013318:Stroke Volume; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000503:physiopathology; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging* / Q000503:physiopathology; D016277:Ventricular Function, Left