TY - JOUR
T1 - Behavior and quality of life at 6 years for children with hypoplastic left heart syndrome
AU - Goldberg, Caren S.
AU - Hu, Chenwei
AU - Brosig, Cheryl
AU - Gaynor, J. William
AU - Mahle, William T.
AU - Miller, Thomas
AU - Mussatto, Kathleen A.
AU - Sananes, Renee
AU - Uzark, Karen
AU - Trachtenberg, Felicia
AU - Pizarro, Christian
AU - Pemberton, Victoria L.
AU - Lewis, Alan B.
AU - Li, Jennifer S.
AU - Jacobs, Jeffrey P.
AU - Cnota, James
AU - Atz, Andrew M.
AU - Lai, Wyman W.
AU - Bellinger, David
AU - Newburger, Jane W.
N1 - Publisher Copyright:
© 2019 by the American Academy of Pediatrics.
PY - 2019/11/1
Y1 - 2019/11/1
N2 - OBJECTIVES:We measured behavioral, quality of life (QoL), and functional status outcomes for 6- year-old children with hypoplastic left heart syndrome enrolled in the Single Ventricle Reconstruction Trial. We sought to compare these outcomes with those in the normative population and to analyze risk factors for worse outcomes within the single-ventricle group. METHODS: Parent-response instruments included the Vineland Adaptive Behavior Scales, Second Edition (Vineland-II) (primary outcome), Behavior Assessment System for Children 2, Pediatric Quality of Life Inventory 4.0, and other measures of QoL and functional status. We compared subjects with those in the normative sample using 1-sample Wilcoxon rank tests and assessed outcome predictors using multivariable regression. RESULTS: Of 325 eligible patients, 250 (77%) participated. Compared with population norms, participants had lower scores on the Vineland-II motor skills domain (90 6 17 vs 100 6 15; P <001), with 11% scoring .2 SDs below the normative mean. On nearly all major domains, more study subjects (3.3%-19.7%) scored outside the normal range than anticipated for the general population. Independent risk factors for lower Vineland-II scores included perioperative extracorporeal membrane oxygenation, male sex, use of regional cerebral perfusion, catheterization after stage 2 operation, visual problems, seizure history, and more complications after 2 years (R2 = 0.32). Independent predictors of worse Behavior Assessment System for Children 2 (R2 = 0.07-0.20) and Pediatric Quality of Life Inventory 4.0 (R2 = 0.17-0.25) domain scores also included sociodemographic factors and measures of morbidity and/or greater course complexity. CONCLUSIONS: At 6 years, children with hypoplastic left heart syndrome had difficulty in areas of adaptive behavior, behavioral symptoms, QoL, and functional status. Principal risks for adverse outcomes include sociodemographic factors and measures of greater course complexity. However, models reveal less than one-third of outcome variance.
AB - OBJECTIVES:We measured behavioral, quality of life (QoL), and functional status outcomes for 6- year-old children with hypoplastic left heart syndrome enrolled in the Single Ventricle Reconstruction Trial. We sought to compare these outcomes with those in the normative population and to analyze risk factors for worse outcomes within the single-ventricle group. METHODS: Parent-response instruments included the Vineland Adaptive Behavior Scales, Second Edition (Vineland-II) (primary outcome), Behavior Assessment System for Children 2, Pediatric Quality of Life Inventory 4.0, and other measures of QoL and functional status. We compared subjects with those in the normative sample using 1-sample Wilcoxon rank tests and assessed outcome predictors using multivariable regression. RESULTS: Of 325 eligible patients, 250 (77%) participated. Compared with population norms, participants had lower scores on the Vineland-II motor skills domain (90 6 17 vs 100 6 15; P <001), with 11% scoring .2 SDs below the normative mean. On nearly all major domains, more study subjects (3.3%-19.7%) scored outside the normal range than anticipated for the general population. Independent risk factors for lower Vineland-II scores included perioperative extracorporeal membrane oxygenation, male sex, use of regional cerebral perfusion, catheterization after stage 2 operation, visual problems, seizure history, and more complications after 2 years (R2 = 0.32). Independent predictors of worse Behavior Assessment System for Children 2 (R2 = 0.07-0.20) and Pediatric Quality of Life Inventory 4.0 (R2 = 0.17-0.25) domain scores also included sociodemographic factors and measures of morbidity and/or greater course complexity. CONCLUSIONS: At 6 years, children with hypoplastic left heart syndrome had difficulty in areas of adaptive behavior, behavioral symptoms, QoL, and functional status. Principal risks for adverse outcomes include sociodemographic factors and measures of greater course complexity. However, models reveal less than one-third of outcome variance.
UR - https://www.scopus.com/pages/publications/85074445715
U2 - 10.1542/peds.2019-1010
DO - 10.1542/peds.2019-1010
M3 - Article
C2 - 31628208
AN - SCOPUS:85074445715
SN - 0031-4005
VL - 144
JO - Pediatrics
JF - Pediatrics
IS - 5
M1 - e20191010
ER -