Pediatric Cardiology

, Volume 36, Issue 3, pp 685–691 | Cite as

Early Results of Neurodevelopment Following Hybrid Stage I for Hypoplastic Left Heart Syndrome

  • Sharon L. Cheatham
  • Helen Carey
  • Joanne L. Chisolm
  • Jill C. Heathcock
  • Deborah Steward
Original Article


Motor skills and neurodevelopment in infants with hypoplastic left heart syndrome (HLHS) who have undergone Hybrid Stage I palliation is unknown. The purpose of this study is to assess early neurodevelopment in infants with HLHS after Hybrid Stage I palliation. Developmental assessment was performed in HLHS infants who underwent Hybrid Stage I palliation at 2 and 4 months of age using the Test of Infant Motor Performance, and at 6 months of age, prior to undergoing the second staged surgery, using the Bayley Scales of Infant and Toddler Development, 3rd edition (Bayley-III). Results were compared to healthy control subjects and norm-referenced data. The HLHS group scored between −1 and −2 standard deviations (SD) below the mean at 2 months of age (p = 0.002), and within −1 SD of the mean, at 4 months of age (p = 0.0019), on the TIMP. Compared to the control group, composite motor skills were significantly lower at 6 months of age on the Bayley-III in the HLHS group (p = 0.0489), however, not significant for cognitive (p = 0.29) or language (p = 0.68). Percentile rank motor scores were 17 ± 20 % in the HLHS group compared to 85 ± 12 % for the healthy age-matched control group. Infants with HLHS who undergo Hybrid Stage I palliation score lower on standardized motor skill tests compared to healthy age-matched controls and the norm-referenced population. This suggests that infants with HLHS have poorer motor skill performance than typically developing infants at 6 months of age.


Congenital heart disease Hypoplastic left heart syndrome Neurodevelopment Hybrid 



Congenital heart disease


Hypoplastic left heart syndrome


Mental development index


Psychomotor development index


Test of Infant Motor Performance


Bayley Scales of Infant and Toddler Development, 3rd edition


Standard deviation



This study was supported in part by the Cardiovascular Research Program in The Heart Center at Nationwide Children’s Hospital, as well as the National Institute of Health NRSA F31NR011253 by the National Institute of Nursing Research. The content is solely the responsibility of the author and does not necessarily represent the official views of the National Institute of Nursing Research or the National Institutes of Health.

Ethical standards

This study was approved by the Institutional Review Board and has therefore been performed in accordance with the ethical standards in the 1964 Declaration of Helsinki and its later amendments. Informed consent was obtained on all subjects prior to their inclusion in the study.


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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  • Sharon L. Cheatham
    • 1
    • 4
  • Helen Carey
    • 2
  • Joanne L. Chisolm
    • 1
  • Jill C. Heathcock
    • 3
    • 5
  • Deborah Steward
    • 3
    • 4
  1. 1.The Heart CenterNationwide Children’s HospitalColumbusUSA
  2. 2.Clinical Therapies DepartmentNationwide Children’s HospitalColumbusUSA
  3. 3.Center for Perinatal ResearchNationwide Children’s HospitalColumbusUSA
  4. 4.College of NursingThe Ohio State UniversityColumbusUSA
  5. 5.School of Health and Rehabilitation ServicesThe Ohio State UniversityColumbusUSA

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