Aortic morphometry and microcephaly in hypoplastic left heart syndrome

Amanda J. Shillingford, Richard F. Ittenbach, Bradley S. Marino, Jack Rychik, Robert R. Clancy, Thomas L. Spray, J. William Gaynor, Gil Wernovsky
2007 Cardiology in the Young  
when the systemic or left ventricle fails to develop adequately. It is associated with varying degrees of hypoplasia of the aorta, aortic valve, mitral valve, and left ventricle. It is the second most common critical cardiac defect to present in the first week of life, and is fatal in infancy if palliative surgery is not performed. 1 Genetic syndromes and non-cardiac malformations have been reported in up to three-tenths of neonates with the syndrome. 2 Early survival has now improved, due to
more » ... ompt diagnosis and stabilization in the neonatal period, as well as advances in surgical techniques and perioperative management. 3,4 Studies to date, however, have indicated that survivors of both reconstructive surgery and transplantation have a greater likelihood of decreased intelligence, delays in speech and language, attentional and behavioural difficulties, and an increased need for special services in school. [5] [6] [7] [8] [9] Accordingly, greater attention has been directed toward understanding factors that may influence undesirable outcomes. 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.
doi:10.1017/s1047951107000248 pmid:17338838 fatcat:quwnj5w5bnbuxhduhd7c3vinkq