Children and Adolescents with Repaired Tetralogy of Fallot Report Quality of Life Similar to Healthy Peers

Objective. The study aims to evaluate and compare self-reported and parent proxy-reported quality of life (QOL) in pediatric patients with repaired tetralogy of Fallot (TOF) and determine relationships with residual disease. Design. QOL was prospectively evaluated in children/adolescents with repaired TOF and parents' proxy report using the Pediatric Quality of Life Inventory Generic Core and Cardiac Module scales. The scores were compared with published self and parent proxy-reported normative data for children considered healthy, chronically ill, and with congenital heart disease. Recent clinical data were reviewed for correlations between QOL and residual disease severity. Results. Twenty child-parent pairs were assessed at median age of 10.9 years (range 8.4-18.7 years). Self-report was higher than parent proxy report. Compared with peers, self-reported QOL was higher than for chronically ill children (overall QOL 85 vs. 77, P = 0.007) and similar to healthy children (85 vs. 83, P = 0.44), while proxy report by parent was similar to parents of chronically ill children (overall QOL 77 vs. 74, P = 0.035). Despite moderate pulmonary regurgitation (mean 35%) and right ventricular dilation (mean 114 mL/m(2)), 76% had a New York Heart Association class of 1, normal B-natriuretic peptide (24 pg/mL), reasonable exercise tolerance VO2 max% predicted (mean 77%), and preserved right ventricular ejection fraction (mean 58%, range 44-80%). Overall QOL positively correlated with child's VO2 max% predicted, when reported by child (r = 0.47, P < 0.05) and parent proxy (r = 0.63, P < 0.05). Conclusions. QOL in children/adolescents with repaired TOF is not proportional to the severity of their residual disease. Self-reported QOL appears similar to healthy peers while parent proxy reported lower QOL. For both children and parents, QOL positively correlated with the child's exercise capacity. Therefore, comprehensive follow-up should include cardiac rehabilitation and psychosocial evaluation to ensure an active lifestyle, improve health perception, and prevent later acquired heart disease.