Prenatal head growth and white matter injury in hypoplastic left heart syndrome.
Prenatal head growth and white matter injury in hypoplastic left heart syndrome.
复制标题
发育不全左心综合征的产前头部生长和白质损伤。
DOI：
10.1203/pdr.0b013e3181827bf4
复制
发表时间：
2008-10
影响因子：
3.6
通讯作者：
Benson DW
中科院分区：
文献类型：
--
作者：
Hinton RB;Andelfinger G;Sekar P;Hinton AC;Gendron RL;Michelfelder EC;Robitaille Y;Benson DW
患有左心发育不全综合征(HLHS)的儿童中枢神经系统(CNS)异常的发生率增加。这一问题在多大程度上是由于中枢神经系统发育不良、产前缺血、出生后慢性青紫和/或多次暴露于体外循环而引起的，目前尚不清楚。为了更好地了解HLHS中中枢神经系统异常的病因，我们评估了68例HLHS新生儿；在28例HLHS中，胎儿超声和超声心动图数据都可以评估头部大小、头部生长和主动脉瓣解剖(闭锁或狭窄)。此外，我们对11例选择性流产的HLHS胎儿进行了神经病理学评估。HLHS新生儿的平均头围百分位数显著小于HLHS胎儿(22±2%比40±4%，p<；0.001)。在初步评估时，有一半(14/28)的HLHS胎儿观察到头部生长显著下降，定义为头围减少50%，近四分之一(6/28)的胎儿已经生长受限(≤10%)。HLHS胎儿的大脑表现出不同程度的慢性弥漫性白质损伤。这些产前头部生长和脑组织病理学的模式确定了HLHS胎儿的一系列异常的中枢神经系统发育和/或损伤。
Children with hypoplastic left heart syndrome (HLHS) have an increased prevalence of central nervous system (CNS) abnormalities. The extent to which this problem is due to CNS maldevelopment, prenatal ischemia, postnatal chronic cyanosis and/or multiple exposures to cardiopulmonary bypass is unknown. To better understand the etiology of CNS abnormalities in HLHS, we evaluated 68 neonates with HLHS; in 28 cases, both fetal ultrasound and echocardiogram data were available to assess head size, head growth and aortic valve anatomy (atresia or stenosis). In addition, we evaluated neuropathology in 11 electively aborted HLHS fetuses. The mean head circumference percentile in HLHS neonates was significantly smaller than HLHS fetuses (22 ± 2% vs. 40 ± 4%, p<0.001). A significant decrease in head growth, defined as a 50% reduction in head circumference percentile, was observed in half (14/28) of HLHS fetuses and nearly a quarter (6/28) were already growth restricted (≤10%) at the time of initial evaluation. Brains from HLHS fetuses demonstrated chronic diffuse white matter injury of varying severity. These patterns of prenatal head growth and brain histopathology identify a spectrum of abnormal CNS development and/or injury in HLHS fetuses.