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Cognitive and Attentional Function in Children with Hypoplastic Left Heart Syndrome: A Pilot Study

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Abstract

While survival for children with hypoplastic left heart syndrome (HLHS) has improved, compromised cardiac output and oxygen delivery persist, and children show cognitive deficits. Most research has assessed young children on broad cognitive indices; less is known about specific indices in older youth. In this pilot study, cognitive function and attention in youth ages 8 to 16 years with HLHS (n = 20) was assessed with the Wechsler Intelligence Scale for Children – Fifth Edition (WISC-V) and NIH Toolbox Cognition Battery (NTCB); parents completed the Child Behavior Checklist. Children scored significantly lower than normative means on the WISC-V Full Scale IQ, Verbal Comprehension, Visual Spatial, Working Memory, and Processing Speed indices, and the NTCB Fluid Cognition Composite; effect sizes ranged from medium to large. Attention problems had a large significant effect. Child age corresponded to lower visual spatial scores. Findings highlight the importance of assessing multiple cognitive indices for targeted intervention and investigating age and disease factors as potential correlates in larger samples.

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References

  • Achenbach, T. M., & Rescorla, L. A. (2001). Manual for the ASEBA school-age forms and profiles.

  • Bauer, P. J., & Zelazo, P. D. (2013). NIH toolbox cognition battery (CB): Summary, conclusions, and implications for cognitive development. Monographs of the Society for Research in Child Development, 78, 133–146. https://doi.org/10.1111/mono.12039.

    Article  PubMed  Google Scholar 

  • Bauer, P. J., & Zelazo, P. D. (2014). The national institutes of health toolbox for the assessment of neurological and behavioral function: A tool for developmental science. Child Development Perspectives, 8, 119–124. https://doi.org/10.1111/cdep.12080.

    Article  Google Scholar 

  • Bellinger, D. C., Watson, C. G., Rivkin, M. J., Robertson, R. L., Roberts, A. E., Stopp, C., … Newburger, J. W. (2015). Neuropsychological status and structural brain imaging in adolescents with single ventricle who underwent the fontan procedure. Journal of the American Heart Association, 4, 1–17. https://doi.org/10.1161/JAHA.115.002302.

  • Benjamin, E. J., Virani, S. S., Callaway, C. W., Chamberlain, A. M., Chang, A. R., Cheng, S., … de Ferranti, S. D. (2018). Heart disease and stroke statistics—2018 update: A report from the American Heart Association. Circulation. https://doi.org/10.1161/CIR.0000000000000558.

  • Brosig, C. L., Mussatto, K. A., Kuhn, E. M., & Tweddell, J. S. (2007). Neurodevelopmental outcome in preschool survivors of complex congenital heart disease: Implications for clinical practice. Journal of Pediatric Health Care, 21, 3–12. https://doi.org/10.1016/j.pedhc.2006.03.008.

    Article  PubMed  Google Scholar 

  • Brosig, C., Mussatto, K., Hoffman, G., Hoffmann, R. G., Dasgupta, M., Tweddell, J., & Ghanayem, N. (2013). Neurodevelopmental outcomes for children with hypoplastic left heart syndrome at the age of 5 years. Pediatric Cardiology, 34, 1597–1604. https://doi.org/10.1007/s00246-013-0679-3.

    Article  PubMed  PubMed Central  Google Scholar 

  • Calderon, J., Bellinger, D. C., Hartigan, C., Lord, A., Stopp, C., Wypij, D., & Newburger, J. W. (2019). Improving neurodevelopmental outcomes in children with congenital heart disease: Protocol for a randomised controlled trial of working memory training. British Medical Journal Open, 9, 1–10. https://doi.org/10.1136/bmjopen-2018-023304.

    Article  CAS  Google Scholar 

  • Cassidy, A. R., Ilardi, D., Bowen, S. R., Hampton, L. E., Heinrich, K. P., Loman, M. M., … Wolfe, K. R. (2018). Congenital heart disease: A primer for the pediatric neuropsychologist. Child Neuropsychology, 24, 859–902. https://doi.org/10.1080/09297049.2017.1373758.

  • Cassidy, A. R., White, M. T., DeMaso, D. R., Newburger, J. W., & Bellinger, D. C. (2015). Executive function in children and adolescents with critical cyanotic congenital heart disease. Journal of the International Neuropsychological Society, 21, 34–49. https://doi.org/10.1017/S1355617714001027.

    Article  PubMed  Google Scholar 

  • Chang, L. Y., Wang, M. Y., & Tsai, P. S. (2016). Diagnostic accuracy of Rating Scales for attention-deficit/hyperactivity disorder: A meta-analysis. Pediatrics. https://doi.org/10.1542/peds.2015-2749.

    Article  PubMed  PubMed Central  Google Scholar 

  • Chang, R. K. R., Chen, A. Y., & Klitzner, T. S. (2002). Clinical management of infants with hypoplastic left heart syndrome in the United States, 1988–1997. Pediatrics, 110, 292–298. https://doi.org/10.1542/peds.110.2.292.

    Article  PubMed  Google Scholar 

  • Cleveland Clinic. (n.d.). Hypoplastic left heart syndrome. https://my.clevelandclinic.org/health/diseases/12214-hypoplastic-left-heart-syndrome

  • Cohen, J. (1988). Statistical power analysis for the behavioral sciences (2nd ed.). Hillsdale, NJ: Erlbaum.

    Google Scholar 

  • Compas, B. E., Jaser, S. S., Reeslund, K., Patel, N., & Yarboi, J. (2017). Neurocognitive deficits in children with chronic health conditions. American Psychologist, 72, 326–338. https://doi.org/10.1037/amp0000042.

    Article  Google Scholar 

  • DeMaso, D. R., Calderon, J., Taylor, G. A., Holland, J. E., Stopp, C., White, M. T., … Newburger, J. W. (2017). Psychiatric disorders in adolescents with single ventricle congenital heart disease. Pediatrics. https://doi.org/10.1542/peds.2016-2241.

  • Feinstein, J. A., Benson, D. W., Dubin, A. M., Cohen, M. S., Maxey, D. M., Mahle, W. T., … Johnson, B. A. (2012). Hypoplastic left heart syndrome: Current considerations and expectations. Journal of the American College of Cardiology, 59, S1–S42. https://doi.org/10.1016/j.jacc.2011.09.022.

  • Gaynor, J. W., Ittenbach, R. F., Gerdes, M., Bernbaum, J., Clancy, R. R., McDonald-Mcginn, D. M., … Spray, T. L. (2014). Neurodevelopmental outcomes in preschool survivors of the Fontan procedure. Journal of Thoracic and Cardiovascular Surgery, 147, 1276–1283. https://doi.org/10.1016/j.jtcvs.2013.12.019.

  • Gershon, R. C., Wagster, M. V., Hendrie, H. C., Fox, N. A., Cook, K. F., & Nowinski, C. J. (2013). NIH toolbox for assessment of neurological and behavioral function. Neurology, 80, 2–7. https://doi.org/10.1212/wnl.0b013e3182872e5f.

    Article  Google Scholar 

  • Gerstle, M., Beebe, D. W., Drotar, D., Cassedy, A., & Marino, B. S. (2016). Executive functioning and school performance among pediatric survivors of complex congenital heart disease. Journal of Pediatrics, 173, 154–159. https://doi.org/10.1016/j.jpeds.2016.01.028.

    Article  Google Scholar 

  • Javed, R., Cetta, F., Said, S. M., Olson, T. M., O’Leary, P. W., & Qureshi, M. Y. (2019). Hypoplastic leftheart syndrome: An overview for primary care providers. Pediatrics in Review, 40, 344–353. https://doi.org/10.1542/pir.2018-0005.

    Article  PubMed  Google Scholar 

  • Jordan, L. C., Siciliano, R. E., Cole, D. A., Lee, C. A., Patel, N. J., Murphy, L. K., & Compas, B. E. (2019). Cognitive training in children with hypoplastic left heart syndrome: A pilot randomized trial. Progress in Pediatric Cardiology. https://doi.org/10.1016/j.ppedcard.2019.101185.

  • Karsdorp, P. A., Everaerd, W., Kindt, M., & Mulder, B. J. M. (2007). Psychological and cognitive functioning in children and adolescents with congenital heart disease: A meta-analysis. Journal of Pediatric Psychology, 32, 527–541. https://doi.org/10.1093/jpepsy/jsl047.

    Article  PubMed  Google Scholar 

  • Kelleher, D. K., Laussen, P., Teixeira-Pinto, A., & Duggan, C. (2006). Growth and correlates of nutritional status among infants with hypoplastic left heart syndrome (HLHS) after stage 1 Norwood procedure. Nutrition, 22, 237–244. https://doi.org/10.1016/j.nut.2005.06.008.

    Article  CAS  PubMed  Google Scholar 

  • Knirsch, W., Liamlahi, R., Hug, M. I., Hoop, R., von Rhein, M., Prêtre, R., … Latal, B. (2012). Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures. European Journal of Cardio-Thoracic Surgery, 42, 33–39. https://doi.org/10.1093/ejcts/ezr286.

  • Mahle, W. T., Lu, M., Ohye, R. G., Gaynor, J. W., Goldberg, C. S., Sleeper, L. A., … Newburger, J. W. (2013). A predictive model for neurodevelopmental outcome following the norwood procedure. Pediatric Cardiology, 34, 327–333. https://doi.org/10.1007/s00246-012-0450-1.A.

  • Mahle, W. T., Visconti, K. J., Freier, M. C., Kanne, S. M., Hamilton, W. G., Sharkey, A. M., … Jenkins, P. C. (2006). Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome. Pediatrics. https://doi.org/10.1542/peds.2005-0575.

  • Marelli, A. J., Ionescu-Ittu, R., Mackie, A. S., Guo, L., Dendukuri, N., & Kaouache, M. (2014). Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation, 130, 749–756. https://doi.org/10.1161/CIRCULATIONAHA.113.008396.

    Article  PubMed  Google Scholar 

  • Marelli, A., Miller, S. P., Marino, B. S., Jefferson, A. L., & Newburger, J. W. (2016). Brain in congenital heart disease across the lifespan: The cumulative burden of injury. Circulation, 133, 1951–1962. https://doi.org/10.1161/CIRCULATIONAHA.115.019881.

    Article  PubMed  PubMed Central  Google Scholar 

  • Marino, B. S., Lipkin, P. H., Newburger, J. W., Peacock, G., Gerdes, M., Gaynor, J. W., … Mahle, W. T. (2012). Neurodevelopmental outcomes in children with congenital heart disease: Evaluation and management a scientific statement from the american heart association. Circulation, 126, 1143–1172. https://doi.org/10.1161/CIR.0b013e318265ee8a.

  • Mills, R., McCusker, C. G., Tennyson, C., & Hanna, D. (2018). Neuropsychological outcomes in CHD beyond childhood: A meta-analysis. Cardiology in the Young, 28, 421–431. https://doi.org/10.1017/S104795111700230X.

    Article  PubMed  Google Scholar 

  • Mosca, R. S., Kulik, T. J., Goldberg, C. S., Vermilion, R. P., Charpie, J. R., Crowley, D. C., & Bove, E. L. (2000). Early results of the Fontan procedure in one hundred consecutive patients with hypoplastic left heart syndrome. Journal of Thoracic and Cardiovascular Surgery, 119, 1110–1118. https://doi.org/10.1067/mtc.2000.106656.

    Article  CAS  Google Scholar 

  • Murphy, L. K., Compas, B. E., Reeslund, K. L., Gindville, M. C., Mah, M. L., Markham, L. W., & Jordan, L. C. (2017). Cognitive and attentional functioning in adolescents and young adults with Tetralogy of Fallot and d-transposition of the great arteries. Child Neuropsychology, 23, 99–110. https://doi.org/10.1080/09297049.2015.1087488.

    Article  PubMed  Google Scholar 

  • Mussatto, K. A., Hollenbeck-Pringle, D., Trachtenberg, F., Sood, E., Sananes, R., Pike, N. A., … Pemberton, V. L. (2018). Utilisation of early intervention services in young children with hypoplastic left heart syndrome. Cardiology in the Young, 28, 126–133. https://doi.org/10.1017/S104795111700169X.

  • Newburger, J. W., Sleeper, L. A., Bellinger, D. C., Goldberg, C. S., Tabbutt, S., Lu, M., … Pike, N. (2012). Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: The single ventricle reconstruction trial. Circulation, 125, 2081–2091. https://doi.org/10.1161/CIRCULATIONAHA.111.064113.

  • Oberhuber, R. D., Huemer, S., Mair, R., Sames-Dolzer, E., Kreuzer, M., & Tulzer, G. (2017). Cognitive development of school-age hypoplastic left heart syndrome survivors: A single center study. Pediatric Cardiology, 38, 1089–1096. https://doi.org/10.1007/s00246-017-1623-8.

    Article  PubMed  Google Scholar 

  • Sananes, R., Manlhiot, C., Kelly, E., Hornberger, L. K., Williams, W. G., MacGregor, D., … McCrindle, B. W. (2012). Neurodevelopmental outcomes after open heart operations before 3 months of age. Annals of Thoracic Surgery, 93, 1577–1583. https://doi.org/10.1016/j.athoracsur.2012.02.011.

  • Sanz, J. H., Wang, J., Berl, M. M., Armour, A. C., Cheng, Y. I., & Donofrio, M. T. (2018). Executive function and psychosocial quality of life in school age children with congenital heart disease. Journal of Pediatrics, 202, 63–69. https://doi.org/10.1016/j.jpeds.2018.07.018.

    Article  Google Scholar 

  • Sarajuuri, A., Jokinen, E., Puosi, R., Eronen, M., Mildh, L., Mattila, I., … Lönnqvist, T. (2007). Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: Related risk factor analysis. Journal of Thoracic and Cardiovascular Surgery, 133, 1524–1532. https://doi.org/10.1016/j.jtcvs.2006.12.022.

  • Shillingford, A. J., Glanzman, M. M., Ittenbach, R. F., Clancy, R. R., Gaynor, J. W., & Wernovsky, G. (2008). Inattention, hyperactivity, and school performance in a population of school-age children with complex congenital heart disease. Pediatrics. https://doi.org/10.1542/peds.2007-1066.

    Article  PubMed  Google Scholar 

  • Siciliano, R. E., Prussien, K. V., Lee, C. A., Patel, N. J., Murphy, L. K., Compas, B. E., & Jordan, L. C. (2019). Cognitive function in pediatric hypoplastic left heart syndrome: Systematic review and meta-analysis. Journal of Pediatric Psychology. https://doi.org/10.1093/jpepsy/jsz021.

    Article  PubMed  PubMed Central  Google Scholar 

  • Sterken, C., Lemiere, J., Vanhorebeek, I., Van den Berghe, G., & Mesotten, D. (2015). Neurocognition after paediatric heart surgery: A systematic review and meta-analysis. Open Heart, 2, e000255. https://doi.org/10.1136/openhrt-2015-000255.

    Article  PubMed  PubMed Central  Google Scholar 

  • Watson, C. G., Stopp, C., Wypij, D., Newburger, J. W., & Rivkin, M. J. (2017). Reduced cortical volume and thickness and their relationship to medical and operative features in post-Fontan children and adolescents. Pediatric Research, 81, 881–890. https://doi.org/10.1038/pr.2017.30.

    Article  PubMed  Google Scholar 

  • Wechsler, D. (2014). Wechsler intelligence scale for children, Fifth edition (WISC-V). New York: The Psychological Corporation.

    Google Scholar 

  • Weintraub, S., Dikman, S., Heaton, R. K., Tulsky, D. S., Zelazo, P. D., Bauer, P. J., … Gershon, R. C. (2013). Cognition assessment using the NIH Toolbox. Neurology, 80(11(Supplement 3)), S54–S64.

  • Wolfe, K. R., Liptzin, D. R., Brigham, D., Kelly, S. L., Rafferty, C., Albertz, M., … Di Maria, M. V. (2020). Relationships between physiologic and neuropsychologic functioning after fontan. Journal of Pediatrics. https://doi.org/10.1016/j.jpeds.2020.07.043.

  • Yoo, B. W. (2018). Epidemiology of congenital heart disease with emphasis on sex-related aspects. In P. Kerkof & V. Miller (Eds.), Sex-specific analysis of cardiovascular function (pp. 49–59). Cham: Springer.

    Chapter  Google Scholar 

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Funding

This work was supported by the Vanderbilt Department of Pediatrics via the Turner-Hazinski grant program, a training grant from the National Institute of Mental Health (T32-MH18921), a gift from Rhodes and Patricia Hart, and an anonymous donor.

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Contributions

Study Conceptualization: LCJ, LKM, BEC; Methodology: LCJ, LKM, BEC; Formal analysis and investigation: RES, LKM, KVP, LMH, NJP, CAL; CMM, BEC, LCJ; Writing: RES, LWM, BEC, LCJ; Funding acquisition: LCJ, BEC, KVP; Resources: LCJ; Supervision: LCJ, BEC.

Corresponding author

Correspondence to Lori C. Jordan.

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Conflict of interest

Rachel E. Siciliano, Lexa K. Murphy, Kemar V. Prussien, Lauren M. Henry, Kelly H. Watson, Niral J. Patel, Chelsea A. Lee, Colleen M. McNally, Larry W. Markham, Bruce E. Compas, and Lori C. Jordan declare that they have no conflict of interest.

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All procedures contributing to this work comply with the ethical standards of the relevant national guidelines on human experimentation and with the 1964 Helsinki Declaration and its later amendments and has been approved by the institutional review board of Vanderbilt University Medical Center.

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All parents and children completed informed consent and assent, respectively.

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Siciliano, R.E., Murphy, L.K., Prussien, K.V. et al. Cognitive and Attentional Function in Children with Hypoplastic Left Heart Syndrome: A Pilot Study. J Clin Psychol Med Settings 28, 619–626 (2021). https://doi.org/10.1007/s10880-020-09753-1

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