Six-Year Neurodevelopmental Outcomes for Children With Single-Ventricle Physiology

Author:

Sananes Renee1,Goldberg Caren S.2,Newburger Jane W.3,Hu Chenwei4,Trachtenberg Felicia4,Gaynor J. William5,Mahle William T.6,Miller Thomas78,Uzark Karen2,Mussatto Kathleen A.9,Pizarro Christian10,Jacobs Jeffrey P.11,Cnota James12,Atz Andrew M.13,Lai Wyman W.14,Burns Kristin M.15,Milazzo Angelo161718,Votava-Smith Jodie19,Brosig Cheryl L.20,

Affiliation:

1. Department of Pediatrics and Labatt Family Heart Centre, Hospital for Sick Children, University of Toronto, Toronto, Canada;

2. Department of Pediatrics, Michigan Medicine, Medical School, University of Michigan, Ann Arbor, Michigan;

3. Department of Cardiology, Boston Children’s Hospital and Department of Pediatrics, Harvard Medical School, Harvard University, Boston, Massachusetts;

4. New England Research Institutes, Watertown, Massachusetts;

5. Division of Pediatric Cardiothoracic Surgery, Children’s Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania;

6. Department of Pediatrics, Children’s Healthcare of Atlanta, Emory University, Atlanta, Georgia;

7. Department of Pediatrics, Primary Children’s Hospital, University of Utah, Salt Lake City, Utah;

8. Division of Pediatric Cardiology, Maine Medical Center, Portland, Maine;

9. Departments of Surgery and

10. Department of Surgery, Nemours Cardiac Center, Alfred I du Pont Hospital for Children, Wilmington, Delaware;

11. The Heart Institute of Florida, St Petersburg, Florida;

12. Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio;

13. Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina;

14. Children’s Heart Institute, Children’s Hospital of Orange County, Orange, California;

15. National Heart, Lung, and Blood Institute, Bethesda, Maryland;

16. Department of Pediatrics, School of Medicine, Duke University, Durham, North Carolina;

17. Department of Pediatrics, East Carolina University, Greenville, North Carolina;

18. Department of Pediatrics, Wake Forest University, Winston-Salem, North Carolina; and

19. Department of Pediatrics, Children’s Hospital Los Angeles, Los Angeles, California

20. Pediatrics, Herma Heart Institute, Children’s Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin;

Abstract

OBJECTIVES: To determine if neurodevelopmental deficits in children with single-ventricle physiology change with age and early developmental scores predict 6-year outcomes. METHODS: In the Single Ventricle Reconstruction Trial, Bayley Scales of Infant Development, Second Edition, were administered at 14 months of age, and parents completed the Behavior Assessment System for Children, Second Edition (BASC-2) annually from the ages of 2 to 6 years. Scores were classified as average, at risk, or impaired. We calculated sensitivities, specificities, and positive and negative predictive values of earlier tests on 6-year outcomes. RESULTS: Of 291 eligible participants, 244 (84%) completed the BASC-2 at 6 years; more Single Ventricle Reconstruction participants than expected on the basis of normative data scored at risk or impaired on the BASC-2 Adaptive Skills Index at that evaluation (28.7% vs 15.9%; P < .001). Children with Adaptive Skills Composite scores <2 SD below the mean at the age of 6 were more likely to have had delayed development at 14 months, particularly on the Psychomotor Development Index (sensitivity of 79%). However, the positive predictive value of the 14-month Mental Development Index and Psychomotor Development Index for 6-year BASC-2 Adaptive Scores was low (44% and 36%, respectively). Adaptive Skills Composite score impairments at the age of 6 were poorly predicted by using earlier BASC-2 assessments, with low sensitivities at the ages of 3 (37%), 4 (48%), and 5 years (55%). CONCLUSIONS: Many children with hypoplastic left heart syndrome who have low adaptive skills at the age of 6 years will not be identified by screening at earlier ages. With our findings, we highlight the importance of serial evaluations for children with critical congenital heart disease throughout development.

Publisher

American Academy of Pediatrics (AAP)

Subject

Pediatrics, Perinatology and Child Health

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