Skip to main content
×
Home
    • Aa
    • Aa

Aortic morphometry and microcephaly in hypoplastic left heart syndrome

  • Amanda J. Shillingford (a1), Richard F. Ittenbach (a2), Bradley S. Marino (a1), Jack Rychik (a1), Robert R. Clancy (a3), Thomas L. Spray (a4), J. William Gaynor (a4) and Gil Wernovsky (a1)...
Abstract

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.

Copyright
Corresponding author
Correspondence to: Amanda J. Shillingford, MD, Division of Cardiology, The Children's Hospital of Philadelphia, 34th & Civic Center Boulevard, Philadelphia, Pennsylvania 19104, United States of America. Tel: 215 590 3548; Fax: 215 590 5825; E-mail: shillingford@email.chop.edu
Linked references
Hide All

This list contains references from the content that can be linked to their source. For a full set of references and notes please see the PDF or HTML where available.

FerenczC, RubinJD, McCarterRJ, et al. Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study.Am J Epidemiol1985; 121: 3136.

VerheijenPM, LisowskiLA, StoutenbeekP, et al. Prenatal diagnosis of congenital heart disease affects preoperative acidosis in the newborn patient.J Thorac Cardiovasc Surg2001; 121: 798803.

TworetzkyW, McElhinneyDB, ReddyVM, BrookMM, HanleyFL, SilvermanNH. Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome.Circulation2001; 103: 12691273.

IkleL, HaleK, FashawL, BoucekM, RosenbergAA. Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation.J Pediatr2003; 142: 2025.

MahleWT, ClancyRR, MossEM, GerdesM, JobesDR, WernovskyG. Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome.Pediatrics2000; 105: 10821089.

GoldbergCS, SchwartzEM, BrunbergJA, et al. Neurodevelopmental outcome of patients after the fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions.J Pediatr2000; 137: 646652.

EkeCC, GundrySR, BaumMF, ChinnockRE, RazzoukAJ, BaileyLL. Neurologic sequelae of deep hypothermic circulatory arrest in cardiac transplant infants.Ann Thorac Surg1996; 61: 783788.

WernovskyG, StilesKM, GauvreauK, et al. Cognitive development after the Fontan operation.Circulation2000; 102: 883889.

WoodsCG. Human microcephaly.Curr Opin Neurobiol2004; 14: 112117.

LimperopoulosC, MajnemerA, ShevellMI, RosenblattB, RohlicekC, TchervenkovC. Neurologic status of newborns with congenital heart defects before open heart surgery.Pediatrics1999; 103: 402408.

ClancyRR, McGaurnSA, GoinJE, et al. Allopurinol neurocardiac protection trial in infants undergoing heart surgery using deep hypothermic circulatory arrest.Pediatrics2001; 108: 6170.

LichtDJ, WangJ, SilvestreDW, et al. Preoperative cerebral blood flow is diminished in neonates with severe congenital heart defects.J Thorac Cardiovasc Surg2004; 128: 841849.

GaynorJW, GerdesM, ZackaiEH, et al. Apolipoprotein E genotype and neurodevelopmental sequelae of infant cardiac surgery. J Thorac Cardiovasc Surg2003; 126: 17361745.

ZhangJ, QuanH, NgJ, StepanavageME. Some statistical methods for multiple endpoints in clinical trials.Control Clin Trials1997; 18: 204221.

DonofrioMT, BremerYA, SchiekenRM, et al. Autoregulation of cerebral blood flow in fetuses with congenital heart disease: the brain sparing effect.Pediatr Cardiol2003; 24: 436443.

KaltmanJR, DiH, TianZ, RychikJ. Impact of congenital heart disease on cerebrovascular blood flow dynamics in the fetus.Ultrasound Obstet Gynecol2005; 25: 3236.

BokeschPM, AppachiE, CavagliaM, MossadE, MeeRB. A glial-derived protein, S100B, in neonates and infants with congenital heart disease: evidence for preexisting neurologic injury.Anesth Analg2002; 95: 889892.

CohnHE, SacksEJ, HeymannMA, RudolphAM. Cardiovascular responses to hypoxemia and acidemia in fetal lambs.Am J Obstet Gynecol1974; 120: 817824.

LumbersER, YuZY, GibsonKJ. The selfish brain and the barker hypothesis.Clin Exp Pharmacol Physiol2001; 28: 942947.

RosenthalGL. Patterns of prenatal growth among infants with cardiovascular malformations: possible fetal hemodynamic effects.Am J Epidemiol1996; 143: 505513.

BellingerDC, WypijD, KubanKC, et al. Developmental and neurological status of children at 4 years of age after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass.Circulation1999; 100: 526532.

BellingerDC, WypijD, duDuplessisAJ, et al. Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: the Boston Circulatory Arrest Trial.J Thorac Cardiovasc Surg2003; 126: 13851396.

DormanC. Microcephaly and intelligence.Dev Med Child Neurol1991; 33: 267269.

IvanovicDM, LeivaBP, PerezHT, et al. Head size and intelligence, learning, nutritional status and brain development. Head, IQ, learning, nutrition and brain.Neuropsychologia2004; 42: 11181131.

WatembergN, SilverS, HarelS, Lerman-SagieT. Significance of microcephaly among children with developmental disabilities.J Child Neurol2002; 17: 117122.

MilesJH, HaddenLL, TakahashiTN, HillmanRE. Head circumference is an independent clinical finding associated with autism.Am J Med Genet2000; 95: 339350.

LimperopoulosC, MajnemerA, ShevellMI, RosenblattB, RohlicekC, TchervenkovC. Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery.J Pediatr2000; 137: 638645.

LimperopoulosC, MajnemerA, ShevellMI, et al. Functional limitations in young children with congenital heart defects after cardiac surgery.Pediatrics2001; 108: 13251331.

LimperopoulosC, MajnemerA, ShevellMI, et al. Predictors of developmental disabilities after open heart surgery in young children with congenital heart defects.J Pediatr2002; 141: 5158.

MajnemerA, LimperopoulosC, ShevellM, RosenblattB, RohlicekC, TchervenkovC. Long-term neuromotor outcome at school entry of infants with congenital heart defects requiring open-heart surgery.J Pediatr2006; 148: 7277.

MillerG, VogelH. Structural evidence of injury or malformation in the brains of children with congenital heart disease.Semin Pediatr Neurol1999; 6: 2026.

Recommend this journal

Email your librarian or administrator to recommend adding this journal to your organisation's collection.

Cardiology in the Young
  • ISSN: 1047-9511
  • EISSN: 1467-1107
  • URL: /core/journals/cardiology-in-the-young
Please enter your name
Please enter a valid email address
Who would you like to send this to? *
×

Keywords:

Metrics

Altmetric attention score

Full text views

Total number of HTML views: 2
Total number of PDF views: 14 *
Loading metrics...

Abstract views

Total abstract views: 123 *
Loading metrics...

* Views captured on Cambridge Core between September 2016 - 28th May 2017. This data will be updated every 24 hours.