Licht Daniel J, Shera David M, Clancy Robert R, Wernovsky Gil, Montenegro Lisa M, Nicolson Susan C, Zimmerman Robert A, Spray Thomas L, Gaynor J William, Vossough Arastoo
Divison of Neurology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
J Thorac Cardiovasc Surg. 2009 Mar;137(3):529-36; discussion 536-7. doi: 10.1016/j.jtcvs.2008.10.025.
Small head circumferences and white matter injury in the form of periventricular leukomalacia have been observed in populations of infants with severe forms of congenital heart defects. This study tests the hypothesis that congenital heart defects delay in utero structural brain development.
Full-term infants with hypoplastic left heart syndrome or transposition of the great arteries were prospectively evaluated with preoperative brain magnetic resonance imaging. Patients with independent risk factors for abnormal brain development (shock, end-organ injury, or intrauterine growth retardation) were excluded. Outcome measures included head circumferences and the total maturation score on magnetic resonance imaging. Total maturation score is a previously validated semiquantitative anatomic scoring system used to assess whole brain maturity. The total maturation score evaluates 4 parameters of maturity: (1) myelination, (2) cortical infolding, (3) involution of glial cell migration bands, and (4) presence of germinal matrix tissue.
The study cohort included 29 neonates with hypoplastic left heart syndrome and 13 neonates with transposition of the great arteries at a mean gestational age of 38.9 +/- 1.1 weeks. Mean head circumference was 1 standard deviation below normal. The mean total maturation score for the cohort was 10.15 +/- 0.94, significantly lower than reported normative data in infants without congenital heart defects, corresponding to a delay of 1 month in structural brain development.
Before surgery, term infants with hypoplastic left heart syndrome and transposition of the great arteries have brains that are smaller and structurally less mature than expected. This delay in brain development may foster susceptibility to periventricular leukomalacia in the preoperative, intraoperative, and postoperative periods.
在患有严重先天性心脏病的婴儿群体中，观察到小头围以及脑室周围白质软化形式的白质损伤。本研究检验先天性心脏病会延迟子宫内脑结构发育这一假说。
对患有左心发育不全综合征或大动脉转位的足月儿进行术前脑磁共振成像的前瞻性评估。排除具有脑发育异常独立危险因素（休克、终末器官损伤或宫内生长迟缓）的患者。观察指标包括头围和磁共振成像上的总成熟度评分。总成熟度评分是一种先前已验证的半定量解剖评分系统，用于评估全脑成熟度。总成熟度评分评估成熟度的4个参数：（1）髓鞘形成，（2）皮质折叠，（3）神经胶质细胞迁移带的退化，以及（4）生发基质组织的存在。
研究队列包括29例患有左心发育不全综合征的新生儿和13例患有大动脉转位的新生儿，平均胎龄为38.9±1.1周。平均头围比正常低1个标准差。该队列的平均总成熟度评分为10.15±0.94，显著低于无先天性心脏病婴儿的报告规范数据，相当于脑结构发育延迟1个月。
在手术前，患有左心发育不全综合征和大动脉转位的足月儿的大脑比预期的更小且结构上更不成熟。这种脑发育延迟可能会增加术前、术中和术后发生脑室周围白质软化的易感性。
