Knirsch Walter, Mayer Kristina Nadine, Scheer Ianina, Tuura Ruth, Schranz Dietmar, Hahn Andreas, Wetterling Kristina, Beck Ingrid, Latal Beatrice, Reich Bettina
Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital Zurich, Switzerland.
Children's Research Center, University Children's Hospital Zurich, Switzerland.
Eur J Cardiothorac Surg. 2017 Apr 1;51(4):740-746. doi: 10.1093/ejcts/ezw399.
Neonates with single ventricle congenital heart disease are at risk for structural cerebral abnormalities. Little is known about the further evolution of cerebral abnormalities until Fontan procedure.
Between August 2012 and July 2015, we conducted a prospective cross-sectional two centre study using cerebral magnetic resonance imaging (MRI) and neuro-developmental outcome assessed by the Bayley-III. Forty-seven children (31 male) were evaluated at a mean age of 25.9 ± 3.4 months with hypoplastic left heart syndrome (25) or other single ventricle (22).
Cerebral MRI was abnormal in 17 patients (36.2%) including liquor space enlargements (10), small grey (9) and minimal white (5) matter injuries. Eight of 17 individuals had combined lesions. Median (range) cognitive composite score (CCS) (100, 65-120) and motor composite score (MCS) (97, 55-124) were comparable to the reference data, while language composite score (LCS) (97, 68-124) was significantly lower ( P = 0.040). Liquor space enlargement was associated with poorer performance on all Bayley-III subscores (CCS: P = 0.02; LCS: P = 0.002; MCS: P = 0.013). The number of re-operations [odds ratio (OR) 2.2, 95% confidence interval (CI) 1.1-4.3] ( P = 0.03) and re-interventions (OR 2.1, 95% CI 1.1-3.8) ( P = 0.03) was associated with a higher rate of overall MRI abnormalities.
Cerebral MRI abnormalities occur in more than one third of children with single ventricle, while the neuro-developmental status is less severely affected before Fontan procedure. Liquor space enlargement is the predominant MRI finding associated with poorer neuro-developmental status, warranting further studies to determine aetiology and further evolution until school-age.
患有单心室先天性心脏病的新生儿有发生结构性脑异常的风险。在进行Fontan手术之前，对于脑异常的进一步演变了解甚少。
在2012年8月至2015年7月期间，我们进行了一项前瞻性横断面双中心研究，使用脑磁共振成像（MRI）以及贝利婴幼儿发展量表第三版（Bayley-III）评估神经发育结局。对47名儿童（31名男性）进行了评估，这些儿童平均年龄为25.9±3.4个月，患有左心发育不全综合征（25例）或其他单心室疾病（22例）。
17例患者（36.2%）的脑MRI异常，包括脑室间隙扩大（10例）、轻度灰质损伤（9例）和轻度白质损伤（5例）。17例中有8例合并多种病变。认知综合评分（CCS）[中位数（范围）为100，65 - 120]和运动综合评分（MCS）（97，55 - 124）与参考数据相当，而语言综合评分（LCS）（97，68 - 124）显著更低（P = 0.040）。脑室间隙扩大与贝利婴幼儿发展量表第三版所有子评分的较差表现相关（CCS：P = 0.02；LCS：P = 0.002；MCS：P = 0.013）。再次手术次数[比值比（OR）2.2，95%置信区间（CI）1.1 - 4.3]（P = 0.03）和再次干预次数（OR 2.1，95% CI 1.1 - 3.8）（P = 0.03）与总体MRI异常的发生率较高相关。
超过三分之一的单心室儿童存在脑MRI异常，而在进行Fontan手术之前神经发育状况受影响较轻。脑室间隙扩大是与较差神经发育状况相关的主要MRI表现，需要进一步研究以确定病因以及直至学龄期的进一步演变情况。
