Health-Related Quality of Life in Children with Hypoplastic Left Heart Syndrome: Changes over Time and Associations with Neurodevelopmental and Clinical Factors.

OBJECTIVES: To evaluate changes in health-related quality of life (HRQOL) over time as perceived by parents and to examine associations between neurodevelopmental and clinical factors and patient-reported HRQOL in a large multicenter cohort of children with hypoplastic left heart syndrome. STUDY DESIGN: Longitudinal/cohort. The Pediatric Quality of Life Inventory (PedsQL) was administered to 163 parents/165 Fontan survivors enrolled in the Single Ventricle Reconstruction (SVR) trial follow-up studies. In addition to examination of changes in parent-reported HRQOL over time, univariate associations between clinical and neurodevelopmental measures and concurrent self-reported PedsQL scores were evaluated. RESULTS: Despite no significant change in reported heart problems/cardiac symptoms, PedsQL scores reported by parents decreased between SVR II (median age: 6.1 years, interquartile range: 6.0-6.2) and SVR III (median age: 10.9 years, interquartile range: 10.4-11.4) across all domains: total P < .001, physical P = .02, psychosocial P < .001, emotional P < .001, social P ≤ .001, and school P < .001. There was a corresponding increase in proportions with "at risk" impaired HRQOL: total 28% to 39%, physical 27% to 39%, psychosocial 25% to 40%, emotional 20% to 34%, social 22% to 31%, and school 21% to 38%. While there were no significant correlations between medical variables and self-reported PedsQL scores, neurodevelopmental dysfunction across multiple measures was significantly correlated with worse self-reported HRQOL. CONCLUSIONS: By parent report, HRQOL in children with hypoplastic left heart syndrome decreases over time unrelated to their cardiac symptoms and complications. Neurodevelopmental dysfunction is associated with worse patient-reported HRQOL. Routine assessment of neurodevelopmental function and HRQOL is essential to inform interventions to improve outcomes.

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