Wellbeing in Children and Adolescents with Fontan Physiology.

OBJECTIVE: To assess health-related quality of life (HRQOL) and global quality of life (QOL) in children and adolescents with Fontan physiology and identify key predictors influencing these outcomes. STUDY DESIGN: Cross-sectional analysis of 73 children and adolescents enrolled in the Australia and New Zealand Fontan Registry aged 6-17 years, at least 12 months post-Fontan operation. Assessments included the Pediatric Quality of Life Inventory (PedsQL) for HRQOL and a developmentally-tailored visual analogue scale (0-10) for global QOL, along with validated sociodemographic, clinical, psychological, relational, and parental measures. Clinical data were provided by the Australia and New Zealand Fontan Registry. RESULTS: Participants (mean age: 11.5 ± 2.6 years, 62% male) reported lower overall HRQOL (P < .001), and lower scores across all HRQOL domains (all P < .0001), compared with normative data. Median global QOL score was 7.0 (IQR 5.8-8.0), with most participants (79%) rating their global QOL ≥6. Anxiety and depressive symptoms requiring clinical assessment were reported by 21% and 26% of participants, respectively. Age, sex, and perceived seriousness of congenital heart disease explained 15% of the variation in HRQOL scores, while depressive symptoms and treatment-related anxiety explained an additional 37% (final model: 52% of variance explained). For global QOL, sociodemographic and clinical factors explained 13% of the variance in scores, while depressive symptoms explained a further 25% (final model: 38% of variance explained). Parental factors were not associated with child QOL outcomes. CONCLUSIONS: Children and adolescents with Fontan physiology experience lower HRQOL than community-based norms, despite reporting fair overall QOL. Psychological factors predominantly influenced QOL outcomes, indicating strategies to bolster psychological health could improve QOL in this population.

Need for Routine Screening of Health-Related Quality of Life in Families of Young Children with Complex Congenital Heart Disease.

OBJECTIVES: To assess health-related quality of life (HRQOL) in families of young children with complex congenital heart disease (CHD), and identify the demographic, clinical, and psychosocial factors that place these children and their mothers at greater risk of vulnerability. STUDY DESIGN: This cross-sectional study took place from June 2015 to October 2016 at The Sydney Children's Hospitals Network Cardiac Service, Australia. Mothers of a child aged 1-5 years with either single ventricle CHD or CHD requiring neonatal biventricular repair were invited to participate. Eighty-seven mothers completed a suite of validated measures, including the Pediatric Quality of Life Inventory, which assessed the outcomes of child and maternal HRQOL. RESULTS: Sixty percent of children with single ventricle CHD and 25% of children with biventricular repair had total Pediatric Quality of Life Inventory scores within the at-risk range. Lower child HRQOL was strongly associated with single ventricle CHD (ß = -0.38; P < .001), physical comorbidity (ß = -0.32; P = .001), feeding difficulties (ß = -0.26; P = .008), and greater maternal psychological stress (ß = -0.18; P = .045), accounting for 52% of the variance in child HRQOL. Lower maternal HRQOL was strongly associated with poorer family functioning (ß = 0.61; P < .001), greater maternal psychological stress (ß = -0.23; P = .004), child physical comorbidity (ß = -0.17; P = .01), and a 'difficult' child temperament (ß = -0.14; P = .01), accounting for 73% of the variance in maternal HRQOL. CONCLUSIONS: Lower HRQOL is common in young children with complex CHD, particularly single ventricle CHD. Several predictors of HRQOL are potentially modifiable, offering possible pathways for prevention and early intervention. Routine screening is a necessary first step toward developing models of care to improve HRQOL in this population.

Early developmental outcomes following major noncardiac and cardiac surgery in term infants: a population-based study.

OBJECTIVE: To ascertain developmental differences between term infants after major noncardiac surgery and cardiac surgery compared with healthy control infants in New South Wales, Australia. STUDY DESIGN: This prospective population-based cohort study enrolled infants between August 1, 2006, and December 31, 2008, who required major noncardiac surgery within the first 90 days of life. Developmental outcomes were compared in these children, cohorts of term infants requiring cardiac surgery, and healthy controls. Infants were assessed at 1 year of age using the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III). RESULTS: Of the 784 infants enrolled, 688 (90.2%) of infants alive at 1 year were assessed. Of these, 539 infants were term and were included in the present analysis. Compared with controls, the infants who underwent cardiac surgery had significantly lower (P < .001) mean scores in all 5 BSID-III subscales, and the infants who underwent noncardiac surgery had significantly lower (P < .05) mean scores in 4 of the 5 BSID-III subscales. The greatest difference was in the incidence of gross motor delay in both the cardiac surgery group (OR, 0.25; 95% CI, 0.16-0.41) and the noncardiac surgery group (OR, 0.41; 95% CI, 0.26-0.63). CONCLUSION: This unique population-based prospective study compared the developmental outcomes of infants who underwent major noncardiac surgery and cardiac surgery. Major surgery in infants was found to be significantly associated with developmental delay at 1 year of age compared with control infants. These data have important implications for interventions and clinical review in the first year of life.