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The neurodevelopmental implications of hypoplastic left heart syndrome in the fetus

  • David F. A. Lloyd (a1) (a2), Mary A. Rutherford (a2), John M. Simpson (a1) and Reza Razavi (a1) (a2)

Abstract

As survival after cardiac surgery continues to improve, an increasing number of patients with hypoplastic left heart syndrome are reaching school age and beyond, with growing recognition of the wide range of neurodevelopmental challenges many survivors face. Improvements in fetal detection rates, coupled with advances in fetal ultrasound and MRI imaging, are contributing to a growing body of evidence that abnormal brain architecture is in fact present before birth in hypoplastic left heart syndrome patients, rather than being solely attributable to postnatal factors. We present an overview of the contemporary data on neurodevelopmental outcomes in hypoplastic left heart syndrome, focussing on imaging techniques that are providing greater insight into the nature of disruptions to the fetal circulation, alterations in cerebral blood flow and substrate delivery, disordered brain development, and an increased potential for neurological injury. These susceptibilities are present before any intervention, and are almost certainly substantial contributors to adverse neurodevelopmental outcomes in later childhood. The task now is to determine which subgroups of patients with hypoplastic left heart syndrome are at particular risk of poor neurodevelopmental outcomes and how that risk might be modified. This will allow for more comprehensive counselling for carers, better-informed decision making before birth, and earlier, more tailored provision of neuroprotective strategies and developmental support in the postnatal period.

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Copyright

This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted re-use, distribution, and reproduction in any medium, provided the original work is properly cited.

Corresponding author

Correspondence to: Dr D. F. A. Lloyd, MBChB, MRCPCH, MPhil, Clinical Research Fellow (Paediatric & Fetal Cardiology), Division of Imaging Sciences and Biomedical Engineering, 4th Floor, Lambeth Wing, St. Thomas Hospital, Westminster Bridge Road, London, SE1 7TH, United Kingdom. Tel: +44 20 7188 7188; Fax: +44 20 7188 5442; E-mail: david.lloyd@kcl.ac.uk

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Keywords

The neurodevelopmental implications of hypoplastic left heart syndrome in the fetus

  • David F. A. Lloyd (a1) (a2), Mary A. Rutherford (a2), John M. Simpson (a1) and Reza Razavi (a1) (a2)

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