Faculty Collaboration Database

Behavior and Quality of Life at 6 Years for Children With Hypoplastic Left Heart Syndrome. Pediatrics 2019 Nov;144(5)

Date

10/20/2019

Pubmed ID

31628208

Pubmed Central ID

PMC6856798

DOI

10.1542/peds.2019-1010

Scopus ID

2-s2.0-85074445715 (requires institutional sign-in at Scopus site)   24 Citations

Abstract

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 ± 17 vs 100 ± 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 (R ² = 0.32). Independent predictors of worse Behavior Assessment System for Children 2 (R ² = 0.07-0.20) and Pediatric Quality of Life Inventory 4.0 (R ² = 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.

Author List

Goldberg CS, Hu C, Brosig C, Gaynor JW, Mahle WT, Miller T, Mussatto KA, Sananes R, Uzark K, Trachtenberg F, Pizarro C, Pemberton VL, Lewis AB, Li JS, Jacobs JP, Cnota J, Atz AM, Lai WW, Bellinger D, Newburger JW, PHN INVESTIGATORS

Authors

Cheryl L. Brosig Soto PhD Chief, Professor in the Pediatrics department at Medical College of Wisconsin
Kathleen Mussatto Ph.D. Associate Professor in the School of Nursing department at Milwaukee School of Engineering

MESH terms used to index this publication - Major topics in bold

Child
Child Behavior
Child Behavior Disorders
Female
Humans
Hypoplastic Left Heart Syndrome
Male
Prospective Studies
Quality of Life
Surveys and Questionnaires