Division of Child Neurology, Department of Gynecology and Pediatrics, Helsinki University Central Hospital, Helsinki, Finland.
J Pediatr. 2010 Sep;157(3):414-20, 420.e1-4. doi: 10.1016/j.jpeds.2010.04.027. Epub 2010 Jun 8.
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.
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.
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).
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.
评估左心发育不全综合征（HLHS）或其他类型功能性单心室（单心室心脏，UVH）患儿的神经发育情况，并评估可能的临床预测因素对结果的影响。
对在同一中心接受姑息性手术治疗后存活的 22 例 HLHS 患儿和 14 例 UVH 患儿以及 42 例健康对照者进行了研究，这些患儿在中位年龄 30.2 个月时接受了神经学检查，并根据贝利婴幼儿发育量表进行了基于人群的前瞻性神经发育随访研究。
HLHS 患儿的平均智力发育指数明显低于对照组（89.9 对 105.5，P<.001），而 UVH 患儿与对照组之间无差异。HLHS 患儿（80.7，P<.001）和 UVH 患儿（94.5，P=.016）的平均精神运动发育指数明显低于对照组（105.3）。
HLHS 患儿的平均智力发育指数落后于对照组。HLHS 和 UVH 患儿的精神运动发育指数均受损。建议对这一重病患儿群体进行常规神经发育随访。
