Neurodevelopmental Burden at Age 5 Years in Patients With Univentricular Heart
Neurodevelopmental Burden at Age 5 Years in Patients With Univentricular Heart
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DOI：
10.1542/peds.2012-0486
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发表时间：
2012-12-01
期刊：
影响因子：
8
通讯作者：
Lonnqvist, Tuula
中科院分区：
文献类型：
--
作者：
Sarajuuri, Anne;Jokinen, Eero;Lonnqvist, Tuula
背景技术背景：尽管存活率增加，但患有发育不良性左心综合征（HLHS）和其他形式的功能性单心室心脏缺陷（UVH）的患者仍然存在长期神经发育缺陷的风险增加。一个全国范围内的23例HLHS患者，13例UVH患者和40例对照者的样本被前瞻性地随访，直到5岁，当神经学，神经心理学，结果：HLHS患者（97，P <0.001）和UVH患者（112，P = 0.024）的中位全面智商显著低于对照组（121）。HLHS和UVH患者中分别有26%和23%出现严重神经发育障碍，43%和46%出现轻微神经功能障碍。MRI显示异常，主要是不同程度的缺血性变化，在82%的HLHS患者和56%的UVH患者。显著的变化与神经发育结果和父母的适应行为报告显着相关。在线性回归分析中，预后不良的显著危险因素是与初次手术相关的临床癫痫发作史、新生儿升主动脉直径较低以及与初次和双向Glenn手术相关的几个术前、术中和术后因素。虽然中位认知能力在正常范围内，但在大多数UVH患者中发现了神经发育和脑MRI异常，尤其是在学龄前的HLHS患者中。狭窄的升主动脉和手术相关因素促成了这些结果。儿科2012;130：e1636-e1646
BACKGROUND: Despite increasing survival, patients with hypoplastic left heart syndrome (HLHS) and other forms of functionally univentricular heart defects (UVHs) remain at increased risk of long-term neurodevelopmental deficits.METHODS: A nationwide sample of 23 patients with HLHS, 13 with UVH, and 40 controls were followed prospectively until the age of 5 years, when neurologic, neuropsychological, and motor examinations and brain MRI were performed.RESULTS: The median full-scale IQ was significantly lower in patients with HLHS (97, P < .001) and patients with UVH (112, P = .024) compared with controls (121). Major neurodevelopmental impairment was found in 26% of the patients with HLHS and 23% of those with UVH, and minor neurologic dysfunction was found in 43% and 46%, respectively. MRI revealed abnormalities, mostly ischemic changes of different degrees, in 82% of the patients with HLHS and in 56% of those with UVH. Prominent changes were significantly associated with neurodevelopmental findings and parental reports of adaptive behavior. In linear regression, significant risk factors for a worse outcome were a history of clinical seizures in connection with the primary operation, a lower diameter of the neonatal ascending aorta, and several pre-, peri-, and postoperative factors related to the primary and bidirectional Glenn operations.CONCLUSIONS: Although median cognitive performance was within the normal range, neurodevelopmental and brain MRI abnormalities were found in the majority of the patients with UVH, and especially in those with HLHS, at preschool age. Both a narrowed ascending aorta and operation-related factors contributed to these findings. Pediatrics 2012;130:e1636-e1646