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Neurodevelopment in children with hypoplastic left heart syndrome. 2010

Anne Sarajuuri, and Eero Jokinen, and Riina Puosi, and Leena Mildh, and Ilkka Mattila, and Aulikki Lano, and Tuula Lönnqvist
Division of Child Neurology, Department of Gynecology and Pediatrics, Helsinki University Central Hospital, Helsinki, Finland. Anne.Sarajuuri@hus.fi

OBJECTIVE To assess neurodevelopment in children with hypoplastic left heart syndrome (HLHS) or other types of functionally single ventricle (univentricular heart, UVH) and to estimate the effect of possible clinical predictors on outcome. METHODS A total of 22 patients with HLHS and 14 with UVH, surviving after palliative surgery performed in the same center, and 42 healthy control subjects were examined at a median age of 30.2 months neurologically and according to the Bayley Scales of Infant Development in a population based prospective neurodevelopmental follow-up study. RESULTS The mean Mental Developmental Index was significantly lower (89.9) in patients with HLHS than in control subjects (105.5, P<.001), whereas there was no difference between patients with UVH (98.5) and control subjects. The mean Psychomotor Developmental Index in patients with HLHS (80.7, P<.001) as well as in those with UVH (94.5, P=.016) was significantly inferior to that in control subjects (105.3). CONCLUSIONS In patients with HLHS, mean Mental Developmental Index lags behind control subjects. Psychomotor Developmental Index is impaired in patients with both HLHS and UVH. Routine neurodevelopmental follow-up is recommended for this seriously ill patient group.

UI MeSH Term Description Entries
D008297 Male Males
D009420 Nervous System The entire nerve apparatus, composed of a central part, the brain and spinal cord, and a peripheral part, the cranial and spinal nerves, autonomic ganglia, and plexuses. (Stedman, 26th ed) Nervous Systems,System, Nervous,Systems, Nervous
D009422 Nervous System Diseases Diseases of the central and peripheral nervous system. This includes disorders of the brain, spinal cord, cranial nerves, peripheral nerves, nerve roots, autonomic nervous system, neuromuscular junction, and muscle. Neurologic Disorders,Nervous System Disorders,Neurological Disorders,Disease, Nervous System,Diseases, Nervous System,Disorder, Nervous System,Disorder, Neurologic,Disorder, Neurological,Disorders, Nervous System,Disorders, Neurologic,Disorders, Neurological,Nervous System Disease,Nervous System Disorder,Neurologic Disorder,Neurological Disorder
D011446 Prospective Studies Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group. Prospective Study,Studies, Prospective,Study, Prospective
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D018636 Hypoplastic Left Heart Syndrome A condition caused by underdevelopment of the whole left half of the heart. It is characterized by hypoplasia of the left cardiac chambers (HEART ATRIUM; HEART VENTRICLE), the AORTA, the AORTIC VALVE, and the MITRAL VALVE. Severe symptoms appear in early infancy when DUCTUS ARTERIOSUS closes. Left Heart Syndrome, Hypoplastic,Left Heart Hypoplasia Syndrome

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