Division of Child Neurology, Helsinki University Central Hospital, Helsinki, Finland.
J Int Neuropsychol Soc. 2011 Nov;17(6):1094-103. doi: 10.1017/S135561771100110X. Epub 2011 Oct 13.
Recent advances in the treatment of children with severe congenital heart defects, such as hypoplastic left heart syndrome (HLHS) and other forms of univentricular heart (UVH), have significantly improved their survival rates. However, these children are at risk for various neurodevelopmental deficits. The aim of the present study was to assess cognitive development, expressive language, and behavior in 30-month-old children with univentricular heart. The participants were 22 children with HLHS, 14 with UVH, and 41 healthy control subjects. The Bayley Scales of Infant Development II, MacArthur Communicative Development Inventories, and Child Behavior Checklist were used for assessments. The results revealed that children with HLHS exhibited a significantly lower mean mental development index, more delays in expressive language functions, and more behavioral problems than did the control children. Two children with HLHS (9%) had mental development indexes below 50, indicating significantly delayed performance. The children with UVH differed from the control children with respect to their lower mean mental development index. These findings suggest that at the age of 30 months, neurodevelopmental deficits are especially prevalent in children with HLHS. Thus, early developmental screening, intervention, and neuropsychological follow-up until school age is recommended particularly for the children with HLHS.
最近在治疗严重先天性心脏缺陷儿童方面取得了进展，例如左心发育不全综合征（HLHS）和其他形式的单心室心脏（UVH），显著提高了这些儿童的生存率。然而，这些儿童存在各种神经发育缺陷的风险。本研究旨在评估 30 月龄患有单心室心脏的儿童的认知发育、表达性语言和行为。参与者包括 22 名 HLHS 患儿、14 名 UVH 患儿和 41 名健康对照儿童。采用贝利婴幼儿发展量表第二版、麦克阿瑟语言发展量表和儿童行为检查表进行评估。结果显示，HLHS 患儿的平均智力发育指数显著较低，表达性语言功能延迟更为明显，行为问题也比对照组儿童更多。两名 HLHS 患儿（9%）的智力发育指数低于 50，表明表现明显延迟。UVH 患儿的平均智力发育指数与对照组儿童存在差异。这些发现表明，在 30 月龄时，HLHS 患儿的神经发育缺陷尤其普遍。因此，建议对 HLHS 患儿进行早期发育筛查、干预和神经心理学随访至学龄期。
