THE NORWOOD-SANO OPERATION: A COMPARISON OF TWO-YEAR CLINICAL AND NEURODEVELOPMENTAL OUTCOMES BETWEEN PATIENTS WITH CLASSICAL HYPOPLASTIC LEFT HEART SYNDROME AND THOSE WITH VARIANT ANATOMY

AbstractThe aim of this review is to describe the current state of knowledge related to neurodevelopmental outcomes and quality of life for children with hypoplastic left heart syndrome and to explore future questions to be answered for this group of children.

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Increasing cumulative exposure to volatile anesthetic agents is associated with poorer neurodevelopmental outcomes in children with hypoplastic left heart syndrome

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2016.03.095 ◽

2016 ◽

Vol 152 (2) ◽

pp. 482-489 ◽

Cited By ~ 27

Author(s):

Laura K. Diaz ◽

J. William Gaynor ◽

Shannon J. Koh ◽

Richard F. Ittenbach ◽

Marsha Gerdes ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Volatile Anesthetic ◽

Cumulative Exposure ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Neurodevelopmental Outcomes ◽

Anesthetic Agents ◽

Left Heart Syndrome

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Neurodevelopmental Outcomes for Children With Hypoplastic Left Heart Syndrome at the Age of 5 Years

Pediatric Cardiology ◽

10.1007/s00246-013-0679-3 ◽

2013 ◽

Vol 34 (7) ◽

pp. 1597-1604 ◽

Cited By ~ 30

Author(s):

Cheryl Brosig ◽

Kathleen Mussatto ◽

George Hoffman ◽

Raymond G. Hoffmann ◽

Mahua Dasgupta ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Neurodevelopmental Outcomes ◽

Outcomes For Children ◽

Left Heart Syndrome

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The neurodevelopmental implications of hypoplastic left heart syndrome in the fetus

Cardiology in the Young ◽

10.1017/s1047951116001645 ◽

2016 ◽

Vol 27 (2) ◽

pp. 217-223 ◽

Cited By ~ 5

Author(s):

David F. A. Lloyd ◽

Mary A. Rutherford ◽

John M. Simpson ◽

Reza Razavi

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Imaging Techniques ◽

Neurological Injury ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Neurodevelopmental Outcomes ◽

Detection Rates ◽

Number Of Patients ◽

Wide Range ◽

Left Heart Syndrome

AbstractAs 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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Neurodevelopmental Outcomes After Staged Palliation for Hypoplastic Left Heart Syndrome

PEDIATRICS ◽

10.1542/peds.2007-1282 ◽

2008 ◽

Vol 121 (3) ◽

pp. 476-483 ◽

Cited By ~ 116

Author(s):

S. Tabbutt ◽

A. S. Nord ◽

G. P. Jarvik ◽

J. Bernbaum ◽

G. Wernovsky ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Neurodevelopmental Outcomes ◽

Left Heart Syndrome

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Interstage somatic growth in children with hypoplastic left heart syndrome after initial palliation with the hybrid procedure

Cardiology in the Young ◽

10.1017/s104795111600024x ◽

2016 ◽

Vol 27 (1) ◽

pp. 131-138 ◽

Cited By ~ 4

Author(s):

Fiona T. S. Chan ◽

Hannah R. Bellsham-Revell ◽

Hannah Duggan ◽

John M. Simpson ◽

Tony Hulse ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Somatic Growth ◽

Hybrid Procedure ◽

Growth Patterns ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Neurodevelopmental Outcomes ◽

Second Stage ◽

Z Scores ◽

Left Heart Syndrome

AbstractIntroductionThe hybrid procedure is one mode of initial palliation for hypoplastic left heart syndrome. Subsequently, patients proceed with either the “three-stage” pathway – comprehensive second stage followed by Fontan completion – or the “four-stage” pathway – Norwood procedure, hemi-Fontan, or Fontan completion. In this study, we describe somatic growth patterns observed in the hybrid groups and a comparison primary Norwood group.MethodsA retrospective analysis of patients who have undergone hybrid procedure and Fontan completion was performed. Weight-for-age and height-for-age z-scores were recorded at each operation.ResultsWe identified 13 hybrid patients – eight in the three-stage pathway and five in the four-stage pathway – and 49 Norwood patients. Weight: three stage: weight decreased from hybrid procedure to comprehensive second stage (−0.4±1.3 versus −2.3±1.4, p<0.01) and then increased to Fontan completion (−0.4±1.5 versus −0.6±1.4, p<0.01); four stage: weight decreased from hybrid procedure to Norwood (−2.0±1.4 versus −3.3±0.9, p=0.06), then stabilised to hemi-Fontan. Weight increased from hemi-Fontan to Fontan completion (−2.7±0.6 versus −1.0±0.7, p=0.01); primary Norwood group: weight decreased from Norwood to hemi-Fontan (p<0.001) and then increased to Fontan completion (p<0.001). Height: height declined from hybrid procedure to Fontan completion in the three-stage group. In the four-stage group, height decreased from hybrid to hemi-Fontan, and then increased to Fontan completion. The Norwood group decreased in height from Norwood to hemi-Fontan, followed by an increase to Fontan completion.ConclusionIn this study, we show that patients undergoing the hybrid procedure have poor weight gain before superior cavopulmonary connection, before returning to baseline by Fontan completion. This study identifies key periods to target poor somatic growth, a risk factor of morbidity and worse neurodevelopmental outcomes.

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Aortic morphometry and microcephaly in hypoplastic left heart syndrome

Cardiology in the Young ◽

10.1017/s1047951107000248 ◽

2007 ◽

Vol 17 (2) ◽

pp. 189-195 ◽

Cited By ~ 79

Author(s):

Amanda J. Shillingford ◽

Richard F. Ittenbach ◽

Bradley S. Marino ◽

Jack Rychik ◽

Robert R. Clancy ◽

...

Keyword(s):

Birth Weight ◽

Aortic Arch ◽

Hypoplastic Left Heart Syndrome ◽

Ascending Aorta ◽

Full Term ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Neurodevelopmental Outcomes ◽

Term Infants ◽

Left Heart Syndrome

Microcephaly is a marker of abnormal fetal cerebral development, and a known risk factor for cognitive dysfunction. Patients with hypoplastic left heart syndrome have been found to have an increased incidence of abnormal neurodevelopmental outcomes. We hypothesized that reduced cerebral blood flow from the diminutive ascending aorta and transverse aortic arch in the setting of hypoplastic left heart syndrome may influence fetal growth of the brain. The purpose of our study, therefore, was to define the prevalence of microcephaly in full-term infants with hypoplastic left heart syndrome, and to investigate potential cardiac risk factors for microcephaly. We carried out a retrospective review of full-term neonates with hypoplastic left heart syndrome. Eligible patients had documented indexes of birth weight, and measurements of length, and head circumference, as well as adequate echocardiographic images for measurement of the diameters of the ascending aorta and transverse aortic arch. We used logistic regression for analysis of the data. A total of 129 neonates met the criterions for inclusion, with 15 (12%) proving to have microcephaly. The sizes of their heads were disproportionately smaller than their weights (p less than 0.001) and lengths (p less than 0.001) at birth. Microcephaly was associated with lower birth weight (p less than 0.001), lower birth length (p equal to 0.007), and a smaller diameter of the ascending aorta (p equal to 0.034), but not a smaller transverse aortic arch (p equal to 0.619), or aortic atresia (p equal to 0.969). We conclude that microcephaly was common in this cohort of neonates with hypoplastic left heart syndrome, with the size of the head being disproportionately smaller than weight and length at birth. Microcephaly was associated with a small ascending aorta, but not a small transverse aortic arch. Impairment of somatic growth may be an additional factor in the development of microcephaly in these neonates.

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