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Hypoplastic Left Heart Syndrome is not Associated with Worse Clinical or Neurodevelopmental Outcomes Than Other Cardiac Pathologies After the Norwood–Sano Operation

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Abstract

There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood–Sano operation between 2005 and 2014 were included. Follow-up clinical, neurological, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Developmental outcomes were assessed at 2 years of age using the Bayley Scales of Infant and Toddler Development (Bayley-III). Survival was assessed using Kaplan–Meier analysis. Baseline characteristics, survival, and neurodevelopmental outcomes were compared between those with HLHS and those with non-HLHS anatomy (non-HLHS). The study comprised 126 infants (75 male), 87 of whom had HLHS. Five-year survival was the same for subjects with HLHS and those with non-HLHS (HLHS 71.8%, non-HLHS 76.9%; p = 0.592). Ninety-three patients underwent neurodevelopmental assessment including Bayley-III scores. The overall mean cognitive composite score was 91.5 (SD 14.6), language score was 86.6 (SD 16.7) and overall mean motor composite score was 85.8 (SD 14.5); being lower than the American normative population mean score of 100 (SD 15) for each (p-value for each comparison, <0.0001). None of the cognitive, language, or motor scores differed between those with HLHS and non-HLHS (all p > 0.05). In the generalized linear models, dominant right ventricle anatomy (present in 117 (93%) of patients) was predictive of lower language and motor scores. Comparative analysis of the HLHS and non-HLHS groups undergoing single ventricle palliation including a Norwood–Sano, during the same era, showed comparable 2-year survival and neurodevelopmental outcomes.

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Acknowledgements

We thank Irina Dinu for statistical support. The Complex Pediatric Therapies Follow-up Program has been supported over the years by Alberta Health, the Stollery Children’s Hospital, and the Glenrose Rehabilitation Hospital. The Women and Children’s Health Research Institute has funded registration and acute care data collection starting in 2014. These funding agencies had no role in the design and conduct of the study; analysis or interpretation of the data; preparation, review, or approval of the manuscript; or the decision to submit the manuscript for publication.

Funding

While the registry was funded as above, this study was not specifically funded.

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Authors and Affiliations

  1. Department of Surgery, University of Alberta, Edmonton, Canada

    Billie-Jean Martin, I. De Villiers Jonker, David B. Ross, Ivan M. Rebeyka & Joseph Atallah

  2. Department of Pediatrics, University of Alberta, Edmonton, Canada

    Ari R. Joffe, Charlene M. T. Robertson & Ivan M. Rebeyka

  3. Pediatric Rehabilitation, Glenrose Rehabilitation Hospital, Edmonton, Canada

    Gwen Y. Bond & Charlene M. T. Robertson

  4. Stollery Children’s Hospital, University of Alberta, Edmonton, Canada

    Gwen Y. Bond

  5. Royal University Hospital, University of Saskatchewan, Saskatoon, Canada

    Bryan V. Acton

Authors
  1. Billie-Jean Martin
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  2. I. De Villiers Jonker
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  3. Ari R. Joffe
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  4. Gwen Y. Bond
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  5. Bryan V. Acton
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  6. David B. Ross
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  7. Charlene M. T. Robertson
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  8. Ivan M. Rebeyka
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  9. Joseph Atallah
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Correspondence to Billie-Jean Martin.

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No authors have any conflicts of interest relevant to this study.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Martin, BJ., De Villiers Jonker, I., Joffe, A.R. et al. Hypoplastic Left Heart Syndrome is not Associated with Worse Clinical or Neurodevelopmental Outcomes Than Other Cardiac Pathologies After the Norwood–Sano Operation. Pediatr Cardiol 38, 922–931 (2017). https://doi.org/10.1007/s00246-017-1598-5

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  • DOI: https://doi.org/10.1007/s00246-017-1598-5

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