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Pediatrics. 2019 Nov;144(5). pii: e20191010. doi: 10.1542/peds.2019-1010. Epub 2019 Oct 18.

Behavior and Quality of Life at 6 Years for Children With Hypoplastic Left Heart Syndrome.

Author information

1
Department of Pediatrics, Medical School, University of Michigan and Michigan Medicine, Ann Arbor, Michigan; cgoldber@med.umich.edu.
2
New England Research Institutes, Watertown, Massachusetts.
3
Department of Pediatrics, Medical College of Wisconsin and Children's Hospital of Wisconsin, Milwaukee, Wisconsin.
4
Pediatric Cardiothoracic Surgery, School of Medicine, University of Pennsylvania and Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
5
Department of Pediatrics, Emory University and Children's Healthcare of Atlanta, Atlanta, Georgia.
6
Department of Pediatrics, and The University of Utah and Primary Children's Hospital, Salt Lake City, Utah.
7
Labatt Family Heart Center, Hospital for Sick Children, Toronto, Canada.
8
Department of Pediatrics, Medical School, University of Michigan and Michigan Medicine, Ann Arbor, Michigan.
9
Department of Surgery, Nemours Cardiac Center, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware.
10
National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland.
11
Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, California.
12
Department of Pediatrics, Duke University, Durham, North Carolina.
13
Department of Pediatrics, East Carolina University, Greenville, North Carolina.
14
Department of Pediatrics, Wake Forest University, Winston-Salem, North Carolina.
15
Congenital Heart Institute of Florida, St Petersburg, Florida.
16
Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
17
Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina.
18
Department of Pediatrics, NewYork-Presbyterian Morgan Stanley Children's Hospital, New York, New York.
19
Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
20
Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts; and.

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.

PMID:
31628208
PMCID:
PMC6856798
[Available on 2020-11-01]
DOI:
10.1542/peds.2019-1010

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