Pediatric Cardiology and Congenital Heart Disease, German Heart Center, Munich, Germany.
Pediatric Heart Center, Pediatric Cardiology and Congential Heart Disease, University Hospital Giessen, Giessen, Germany.
Eur J Cardiothorac Surg. 2023 Aug 1;64(2). doi: 10.1093/ejcts/ezad267.
Children with univentricular congenital heart disease undergoing staged surgical palliation are at risk for impaired neurodevelopmental (ND) outcome. Little is known about the long-term effects on brain growth until school age.
In a prospective two-centre study, consecutive patients undergoing stage I (Hybrid or Norwood) to stage III (Fontan procedure) were evaluated by 2 serial cerebral magnetic resonance imaging examinations, somatic growth and ND testing before Fontan procedure at 2 years of age (Bayley-III) and after Fontan at 6-8 years of age (Wechsler Intelligence Scale for Children-third edition). Magnetic resonance imaging findings were compared with 8 healthy controls. Medical and sociodemographic characteristics were documented and related to cerebral and ND findings.
We examined 33 children (16 female) at a mean age of 2.3 (0.35) and 6.8 (± 0.7) years. The mean Bayley-III cognitive scales were 99.1 (9.9), language scales 98.4 (11.9) and motor scales 98.5 (13.8) at the first examination. Follow-up at school age showed a mean total IQ of 86.7 (13.6). The rate of structural brain lesions increased from 39% at 2 years to 58% at school age. Bayley-III language scale (P = 0.021) and mean Wechsler Intelligence Scale for Children-third edition (P = 0.019) were lower in children with pathological MR findings. Total brain volume (P < 0.001), total grey matter volume (P = 0.002), deep grey matter volume (P = 0.001) and white matter volume (P < 0.001) were smaller in patients compared to age- and gender-matched healthy controls.
Smaller brain volumes and structural brain lesions in complex congenital heart defect patients at school age are associated with impaired ND outcome. For the evaluation of predictive surgical or clinical factors, larger multicentre studies are needed.
接受分阶段手术姑息治疗的单心室先天性心脏病患儿存在神经发育（ND）受损的风险。直到学龄期，人们对其大脑生长的长期影响知之甚少。
在一项前瞻性的双中心研究中，连续接受 I 期（杂交或 Norwood）至 III 期（Fontan 手术）治疗的患者，在 Fontan 手术前 2 岁（Bayley-III）和 Fontan 手术后 6-8 岁（Wechsler 儿童智力量表-第三版）时，通过 2 次连续脑磁共振成像检查、体格生长和 ND 测试进行评估。将磁共振成像结果与 8 名健康对照者进行比较。记录患者的医疗和社会人口统计学特征，并将其与脑和 ND 结果相关联。
我们检查了 33 名儿童（16 名女性），平均年龄为 2.3（0.35）岁和 6.8（±0.7）岁。首次检查时，Bayley-III 认知量表的平均得分为 99.1（9.9），语言量表为 98.4（11.9），运动量表为 98.5（13.8）。在学龄期随访时，总智商的平均得分为 86.7（13.6）。2 岁时结构性脑损伤的发生率为 39%，学龄期为 58%。有病理 MR 发现的患儿 Bayley-III 语言量表（P=0.021）和 Wechsler 儿童智力量表-第三版（P=0.019）得分较低。与年龄和性别匹配的健康对照组相比，患者的总脑容量（P<0.001）、总灰质容量（P=0.002）、深部灰质容量（P=0.001）和白质容量（P<0.001）均较小。
学龄期复杂先天性心脏病患者的脑容量较小和结构性脑损伤与 ND 受损有关。为了评估预测性手术或临床因素，需要开展更大规模的多中心研究。
