Neurodevelopmental outcome of children with univentricular heart

Hypoplastic left heart syndrome (HLHS) and other forms of functionally univentricular heart (UVH) are complex congenital heart defects (CHD) that during the past few decades have gained viability with staged palliative surgery. It results in the passive return of systemic venous blood to the lungs without passing through the heart. The aim of this study was to evaluate the neurodevelopmental outcome of children with HLHS and UVH in Finland with neurological, neuropsychological, and motor assessments and brain imaging, and to detect possible risk factors associated with an adverse outcome. Parental perceptions of child behavior and parenting stress were also assessed with parental question-naires. The first study cohort of patients born between 1995 and 1999, which included most of the first HLHS survivors in Finland, showed a significantly lower cognitive outcome compared to normative data among both patients with HLHS (n = 7) and with UVH (n = 19). Cerebral palsy was present in 14% of the patients with HLHS and in 10% of those with UVH, and milder difficulties in gross and fine motor functions were also commonly found. Patients with HLHS or UVH born between 2002 and 2005 were recruited in a prospective neurodevelopmental follow-up study as newborns. The neurodevelopmental evaluations at the ages of 12 months, 30 months, and 5 years revealed a significantly lower cognitive and motor outcome among the patients with HLHS (n = 23) compared to healthy age- and gender-matched controls (n = 40). The patients with UVH (n = 14) only demonstrated a statistically significant difference to controls in motor development during the earlier assessments, and at the age of 5 years also in cognitive development. A smaller sample size may have contributed to the lack of statistically significant difference in cognitive development in the earlier assessments. Major neurodevelopmental impairment was found in 26% of the patients with HLHS, and 23% of the patients with UVH at the age of 5 years. Brain magnetic resonance imaging revealed abnormalities in 82% of the patients with HLHS and 56% of those with UVH most commonly infarcts or ischemic findings of different degrees. Other than minimal ischemic findings were significantly associated with neurodevelopmental outcome. The level of parenting stress was significantly higher among the patients with HLHS compared to controls at the age of 18 months. The parents of the patients with UVH, however, did not report more stress than those of the controls. In conclusion, neurodevelopmental deficits remain a major concern among patients with UVH, and especially among those with HLHS. Neurodevelopmental follow-up is recommended for this seriously ill patient group and psychosocial support for their parents.

Grantor dc:publisher

Unigrafia Oy

Year dc:date.issued

2012

Author dc:creator

• Sarajuuri, Anne

Statement dc:rights

• Julkaisu on tekijänoikeussäännösten alainen. Teosta voi lukea ja tulostaa henkilökohtaista käyttöä varten. Käyttö kaupallisiin tarkoituksiin on kielletty.
• This publication is copyrighted. You may download, display and print it for Your own personal use. Commercial use is prohibited.
• Publikationen är skyddad av upphovsrätten. Den får läsas och skrivas ut för personligt bruk. Användning i kommersiellt syfte är förbjuden.

Language dc:language.iso

eng

Identifier URI

URN:ISBN:978-952-10-7933-7

OAI record oai:identifier

https://helda.helsinki.fi/server/oai/request?verb=GetRecord&metadataPrefix=dim&identifier=oai%3Ahelda.helsinki.fi%3A10138%2F32842