Prenatal Head Growth and White Matter Injury in Hypoplastic Left Heart Syndrome
Open accessAbstract
Children with hypoplastic left heart syndrome (HLHS) have an increased prevalence of central nervous system (CNS) abnormalities. The extent to which this problem is due to CNS maldevelopment, prenatal ischemia, postnatal chronic cyanosis and/or multiple exposures to cardiopulmonary bypass is unknown. To better understand the etiology of CNS abnormalities in HLHS, we evaluated 68 neonates with HLHS; in 28 cases, both fetal ultrasound and echocardiogram data were available to assess head size, head growth and aortic valve anatomy (atresia or stenosis). In addition, we evaluated neuropathology in 11 electively aborted HLHS fetuses. The mean head circumference percentile in HLHS neonates was significantly smaller than HLHS fetuses (22 +/- 2% versus 40 +/- 4%, p < 0.001). A significant decrease in head growth, defined as a 50% reduction in head circumference percentile, was observed in half (14/28) of HLHS fetuses and nearly a quarter (6/28) were already growth restricted (<or=10%) at the time of initial evaluation. Brains from HLHS fetuses demonstrated chronic diffuse white matter injury of varying severity. These patterns of prenatal head growth and brain histopathology identify a spectrum of abnormal CNS development and/or injury in HLHS fetuses.
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A high prevalence of anatomic and functional neurologic abnormalities has been documented before surgery in newborns with congenital heart disease, and such abnormalities are associated with childhood developmental status.
“Reports from a number of investigators have documented a high prevalence of anatomic and functional neurologic abnormalities prior to surgery in newborns with CHD [9-17], and an association of such abnormalities with childhood developmental status [11,13,18,19].”
From Brain Volume and Metabolism in Fetuses With Congenital Heart Disease: Evaluation With Quantitative Magnetic Resonance Imaging and Spectroscopy (2010) · cites this paper as [16]
Adverse neurodevelopmental outcomes in children with complex congenital heart disease are due not only to perioperative insult but also to inborn factors.
“However, there is increasing evidence that adverse neurodevelopmental outcomes in children with complex CHD are due not only perioperative insult, but to inborn factors as well.”
From Brain Volume and Metabolism in Fetuses With Congenital Heart Disease: Evaluation With Quantitative Magnetic Resonance Imaging and Spectroscopy (2010) · cites this paper as [16]
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- Authors
- Hinton, R. B., Andelfinger, G., Sekar, P., Hinton, A. C., Gendron, R. L., Michelfelder, E. C., Robitaille, Y., & Benson, D. W.
- Affiliations
- Cincinnati Children's Hospital Medical Center (US)
- Good Samaritan Hospital (US)
- Université de Montréal (CA)
- Venue
- Pediatric Research
- Published
- 2008-10
- Volume
- 64
- Issue
- 4
- Pages
- 364-369
- ISSN
- 0031-3998 (ISSN-L)
- 1530-0447
- PMC
- PMC2681225
- OpenAlex
- W2004723483
- Cited by
- 118
- References
- 47
- Citations by year
- 2026: 3 · 2025: 4 · 2024: 2 · 2023: 4 · 2022: 7 · 2021: 2 · 2020: 7 · 2019: 11 · 2018: 8 · 2017: 14 · 2016: 10 · 2015: 8 · 2014: 5 · 2013: 9 · 2012: 9
- FWCI
- 3.71
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Pediatric Research (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institutes of Health
- U.S. Public Health Service (GMHD79045)
- Canadian Institutes of Health Research
- National Heart, Lung, and Blood Institute (HL 085122, HL 069712, K23HL085122, K24HL069712)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Fetal and Pediatric Neurological Disorders
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Neonatal and fetal brain pathology
- Keywords
- hypoplastic left heart syndrome, white matter injury, fetal brain development, head circumference, fetal growth restriction, aortic valve anatomy, cardiopulmonary bypass, chronic cyanosis
- MeSH terms
- Aortic Valve, Cephalometry, Echocardiography, Head, Humans, Infant, Newborn, Ohio, Hypoplastic Left Heart Syndrome
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