TY - JOUR
T1 - Health-Related Quality of Life in Children with Hypoplastic Left Heart Syndrome
T2 - Changes over Time and Associations with Neurodevelopmental and Clinical Factors
AU - Pediatric Heart Network
AU - Uzark, Karen
AU - Yu, Sunkyung
AU - Afton, Katherine
AU - Atz, Andrew
AU - Floh, Alejandro
AU - Kasparian, Nadine A.
AU - Lambert, Linda M.
AU - Morrison, Tonia
AU - Shah, Amee
AU - Sood, Erica
AU - Suthar, Divya
AU - Trachtenberg, Felicia
AU - Votava-Smith, Jodie K.
AU - Williams, Jason
AU - Newburger, Jane W.
AU - Goldberg, Caren S.
AU - Pearson, Gail
AU - Pemberton, Victoria
AU - Burns, Kristen
AU - Stylianou, Mario
AU - Freemon, D'Andrea
AU - Levine, Jamie
AU - Rhodes, Jonathan
AU - Dunbar-Masterson, Carolyn
AU - Pizarro, Christian
AU - Sood, Erica
AU - Prospero, Carol
AU - Madueme, Peace
N1 - Copyright © 2025 Elsevier Inc. All rights reserved.
PY - 2026/2
Y1 - 2026/2
N2 - 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.
AB - 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.
KW - congenital heart disease
KW - neurodevelopment
KW - PedsQL
KW - psychosocial
KW - quality of life
UR - https://www.scopus.com/pages/publications/105023855650
UR - https://nemours.elsevierpure.com/en/publications/2c5a36a9-adb2-416b-bd9a-843ee2b8443a
U2 - 10.1016/j.jpeds.2025.114868
DO - 10.1016/j.jpeds.2025.114868
M3 - Article
C2 - 41139009
AN - SCOPUS:105023855650
SN - 0022-3476
VL - 289
SP - 114868
JO - Journal of Pediatrics
JF - Journal of Pediatrics
M1 - 114868
ER -