Cardiology in the Young



Original Article

Aortic morphometry and microcephaly in hypoplastic left heart syndrome


Amanda J. Shillingford a1c1, Richard F. Ittenbach a4, Bradley S. Marino a1, Jack Rychik a1, Robert R. Clancy a3, Thomas L. Spray a2, J. William Gaynor a2 and Gil Wernovsky a1
a1 Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
a2 Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
a3 Division of Neurology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
a4 Division of Biostatistics and Data Management Core, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America

Article author query
shillingford aj   [PubMed][Google Scholar] 
ittenbach rf   [PubMed][Google Scholar] 
marino bs   [PubMed][Google Scholar] 
rychik j   [PubMed][Google Scholar] 
clancy rr   [PubMed][Google Scholar] 
spray tl   [PubMed][Google Scholar] 
gaynor jw   [PubMed][Google Scholar] 
wernovsky g   [PubMed][Google Scholar] 

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.

(Accepted April 7 2006)


Key Words: Hypoplasia of the left heart; aortic atresia; deep hypothermic circulatory arrest; central nervous system; cerebral blood flow.

Correspondence:
c1 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