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.