» Articles » PMID: 19258059

Brain Maturation is Delayed in Infants with Complex Congenital Heart Defects

Abstract

Objective: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Citing Articles

An exploratory fetal MRI study examining the impact of 22q11.2 microdeletion syndrome on early brain growth.

Cromb D, Finck T, Bonthrone A, Uus A, Van Poppel M, Steinweg J J Neurodev Disord. 2025; 17(1):7.

PMID: 39939911 PMC: 11817260. DOI: 10.1186/s11689-025-09594-9.


Cerebral Protection in Pediatric Cardiac Surgery.

Shams-Molkara S, Mendes V, Verdy F, Perez M, Di Bernardo S, Kirsch M Pediatr Cardiol. 2025; .

PMID: 39776194 DOI: 10.1007/s00246-024-03748-7.


Neonatal Seizures: New Evidence, Classification, and Guidelines.

Ziobro J, Pilon B, Wusthoff C, Benedetti G, Massey S, Yozawitz E Epilepsy Curr. 2024; :15357597241253382.

PMID: 39554267 PMC: 11562284. DOI: 10.1177/15357597241253382.


Individualized cortical gyrification in neonates with congenital heart disease.

Cromb D, Wilson S, Bonthrone A, Chew A, Kelly C, Kumar M Brain Commun. 2024; 6(5):fcae356.

PMID: 39429246 PMC: 11487749. DOI: 10.1093/braincomms/fcae356.


Effects of Prostaglandin E1 and Balloon Atrial Septostomy on Cerebral Blood Flow and Oxygenation in Newborns Diagnosed with Transposition of the Great Arteries.

Cucerea M, Ognean M, Pinzariu A, Simon M, Suciu L, Ghiga D Biomedicines. 2024; 12(9).

PMID: 39335532 PMC: 11428714. DOI: 10.3390/biomedicines12092018.


References
1.
de Ferranti S, Gauvreau K, Hickey P, Jonas R, Wypij D, du Plessis A . Intraoperative hyperglycemia during infant cardiac surgery is not associated with adverse neurodevelopmental outcomes at 1, 4, and 8 years. Anesthesiology. 2004; 100(6):1345-52. DOI: 10.1097/00000542-200406000-00005. View

2.
Freed D, Robertson C, Sauve R, Joffe A, Rebeyka I, Ross D . Intermediate-term outcomes of the arterial switch operation for transposition of great arteries in neonates: alive but well?. J Thorac Cardiovasc Surg. 2006; 132(4):845-52. DOI: 10.1016/j.jtcvs.2006.05.046. View

3.
Back S, Luo N, Borenstein N, Levine J, Volpe J, Kinney H . Late oligodendrocyte progenitors coincide with the developmental window of vulnerability for human perinatal white matter injury. J Neurosci. 2001; 21(4):1302-12. PMC: 6762224. View

4.
Bartlett J, Wypij D, Bellinger D, Rappaport L, Heffner L, Jonas R . Effect of prenatal diagnosis on outcomes in D-transposition of the great arteries. Pediatrics. 2004; 113(4):e335-40. DOI: 10.1542/peds.113.4.e335. View

5.
Mahle W, Tavani F, Zimmerman R, Nicolson S, Galli K, Gaynor J . An MRI study of neurological injury before and after congenital heart surgery. Circulation. 2002; 106(12 Suppl 1):I109-14. View