Lianne M. Geerdink

Background: Due to the improved survival rates of children and adolescents with congenital heart disease (CHD), more attention is now being directed towards their health-related quality of life (HRQoL), emotional and behavioral problems. Ebstein anomaly (EA) is a rare CHD with a broad clinical spectrum. The aim of the current study is to evaluate self- and proxy-reported HRQoL and emotional and behavioral problems in children and adolescents with EA. Methods: In this cross-sectional, multicenter study, we included EA patients (aged 8–17 years), who underwent routine clinical assessments in Dutch university hospitals between May 2017 and March 2019. The Generic Pediatric Quality of Life Inventory 4.0 (for ages 8–12/13–17 years) was used to assess HRQoL. The Child Behavior Checklist, Youth Self-Report and Teacher’s Report Form were completed to measure emotional and behavioral problems. Results: Questionnaires for thirty-eight patients (median age: 13 years; 48% male) were completed by patients themselves, parents and teachers. Compared to normative data, self-reported HRQoL was not significantly impaired, except for social functioning in children aged 8–12 years. Gender, severity of tricuspid regurgitation or previous surgery did not predict HRQoL. Parents reported significantly more internalizing (anxiety/depression), attention and thought problems in children with EA. These problems, however, were not reported by adolescents themselves. School teachers reported significantly fewer externalizing problems compared to the norm. Conclusions: Overall, despite satisfactory HRQoL, children and adolescents show emotional and behavioral problems. We recommend routine HRQoL and psychological assessment to screen for less apparent internalizing and psychosocial problems.