Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pa; Division of Cardiology, Department of Pediatrics, Loma Linda University Children's Hospital, Loma Linda, Calif.
Division of Biometrics Research, Merck & Co, Inc, North Wales, Pa.
J Thorac Cardiovasc Surg. 2014 Apr;147(4):1312-8. doi: 10.1016/j.jtcvs.2013.06.021. Epub 2013 Jul 21.
Preoperative brain injury is common in neonates with complex congenital heart disease. Increasing evidence suggests a complex interaction of prenatal and postnatal risk factors for development of brain white matter injury, called periventricular leukomalacia (PVL), in neonates with complex congenital heart disease. To date, there remains a limited understanding of the risk factors contributing to preoperative PVL in hypoplastic left heart syndrome (HLHS).
Neonates with HLHS or HLHS variants from 3 prospective magnetic resonance imaging studies (2003-2010) were selected for this cohort. Preoperative brain magnetic resonance imaging was performed the morning of the surgery. Stepwise multilogistic regression of patient characteristics, mode of delivery (cesarean section vs vaginal), time of diagnosis (prenatal vs postnatal), HLHS subtypes, brain total maturation score, time to surgery, individual averaged daily preoperative blood gases, and complete blood cell count values was used to determine significant associations.
A total of 57 neonates with HLHS were born at 38.7 ± 2.3 weeks; 86% (49/57) had a prenatal diagnosis, with 31% (18/57) delivered by cesarean section. HLHS with aortic atresia (AA) was common in this cohort, 71% (41/57). Preoperative PVL was identified in 19% (11/57). Male patients with AA (P = .004) were at higher risk for PVL. Lower total brain maturation score was also identified as a strong predictor for preoperative PVL (P = .005).
In neonates with HLHS, nonmodifiable patient-related factors, including male sex with AA (lack of antegrade blood flow) and lower total brain maturation score, placed neonates at the greatest risk for preoperative white matter injury.
患有复杂先天性心脏病的新生儿常伴有术前脑损伤。越来越多的证据表明，产前和产后的多种危险因素会导致患有复杂先天性心脏病的新生儿脑白质损伤，即脑室周围白质软化症（PVL）。迄今为止，对于左心发育不全综合征（HLHS）患儿术前发生 PVL 的危险因素，人们的了解仍十分有限。
从 3 项前瞻性磁共振成像研究（2003-2010 年）中选择患有 HLHS 或 HLHS 变异型的新生儿作为本队列研究对象。在手术当天上午进行术前脑部磁共振成像。采用逐步多因素逻辑回归分析患儿特征、分娩方式（剖宫产与阴道分娩）、诊断时间（产前与产后）、HLHS 亚型、脑总成熟评分、手术时间、平均每日术前血气值和全血细胞计数值，以确定显著相关性。
共有 57 例 HLHS 新生儿在 38.7 ± 2.3 周时出生；86%（49/57）患儿存在产前诊断，31%（18/57）为剖宫产分娩。本队列中，HLHS 合并主动脉瓣闭锁（AA）较为常见，占 71%（41/57）。术前发现 19%（11/57）患儿存在 PVL。AA 男性患儿（P = 0.004）发生 PVL 的风险更高。总脑成熟评分较低也被认为是术前发生 PVL 的一个强有力预测因子（P = 0.005）。
在 HLHS 患儿中，非可改变的患者相关因素，包括 AA（缺乏前向血流）的男性患儿和总脑成熟评分较低，使患儿面临更大的术前脑白质损伤风险。
