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Cardiology in the Young Cardiology in the Young

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Aortic morphometry and microcephaly in hypoplastic left heart syndrome

Published online by Cambridge University Press:  05 March 2007

Amanda J. Shillingford ,
Richard F. Ittenbach ,
Bradley S. Marino ,
Jack Rychik ,
Robert R. Clancy ,
Thomas L. Spray ,
J. William Gaynor  and
Gil Wernovsky
Amanda J. Shillingford
Affiliation:
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Richard F. Ittenbach
Affiliation:
Division of Biostatistics and Data Management Core, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Bradley S. Marino
Affiliation:
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Jack Rychik
Affiliation:
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Robert R. Clancy
Affiliation:
Division of Neurology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Thomas L. Spray
Affiliation:
Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
J. William Gaynor
Affiliation:
Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Gil Wernovsky
Affiliation:
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
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Abstract

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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.

Keywords

Hypoplasia of the left heartaortic atresiadeep hypothermic circulatory arrestcentral nervous systemcerebral blood flow
Type
Original Article
Information
Cardiology in the Young , Volume 17 , Issue 2 , April 2007 , pp. 189 - 195
Copyright
© 2007 Cambridge University Press

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