Brain maturation is delayed in infants with complex congenital heart defects

Divison of Neurology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
The Journal of thoracic and cardiovascular surgery (Impact Factor: 4.17). 04/2009; 137(3):529-36; discussion 536-7. DOI: 10.1016/j.jtcvs.2008.10.025
Source: PubMed


Small head circumferences and white matter injury in the form of periventricular leukomalacia have been observed in populations of infants with severe forms of congenital heart defects. This study tests the hypothesis that congenital heart defects delay in utero structural brain development.
Full-term infants with hypoplastic left heart syndrome or transposition of the great arteries were prospectively evaluated with preoperative brain magnetic resonance imaging. Patients with independent risk factors for abnormal brain development (shock, end-organ injury, or intrauterine growth retardation) were excluded. Outcome measures included head circumferences and the total maturation score on magnetic resonance imaging. Total maturation score is a previously validated semiquantitative anatomic scoring system used to assess whole brain maturity. The total maturation score evaluates 4 parameters of maturity: (1) myelination, (2) cortical infolding, (3) involution of glial cell migration bands, and (4) presence of germinal matrix tissue.
The study cohort included 29 neonates with hypoplastic left heart syndrome and 13 neonates with transposition of the great arteries at a mean gestational age of 38.9 +/- 1.1 weeks. Mean head circumference was 1 standard deviation below normal. The mean total maturation score for the cohort was 10.15 +/- 0.94, significantly lower than reported normative data in infants without congenital heart defects, corresponding to a delay of 1 month in structural brain development.
Before surgery, term infants with hypoplastic left heart syndrome and transposition of the great arteries have brains that are smaller and structurally less mature than expected. This delay in brain development may foster susceptibility to periventricular leukomalacia in the preoperative, intraoperative, and postoperative periods.

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    No preview · Article · Apr 2015 · Infant behavior & development
    • "Infants with CHD exhibit high rates of microcephaly, hypotonia, and atypical state regulation on clinical examination, and neuroimaging abnormalities such as ischemic infarcts and white matter injury (periventricular leukomalacia) are present in up to 59% before surgery (Owen, Shevell, Majnemer, & Limperopoulos, 2011). Relative to controls, the brains of full-term infants with TGA or HLHS are smaller and less mature structurally than those of typically developing infants (Licht et al., 2009), with reduced grey matter volumes particularly in the frontal lobe (Watanabe et al., 2009). Adolescents with corrected TGA (Bellinger et al., 2011) and those with TOF (Bellinger et al., 2014a) exhibit much higher rates of structural MRI abnormalities than controls. "
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    • "The imaging findings of their study are similar to those in premature newborns and may reflect abnormal brain development in utero. Licht et al. [4] showed that before surgery, term infants with hypoplastic left heart syndrome and transposition of the great arteries have brains that are smaller and structurally less mature than expected. Periventricular leukomalacia (PVL) is a type of brain injury that affects infants. "
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