Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Zurich, Switzerland; Children's Research Center, University Children's Hospital, Zurich, Switzerland.
Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Zurich, Switzerland; Children's Research Center, University Children's Hospital, Zurich, Switzerland; Diagnostic Imaging, MR-Center, University Children's Hospital, Zurich, Switzerland.
Int J Cardiol. 2019 Sep 15;291:42-44. doi: 10.1016/j.ijcard.2019.03.055. Epub 2019 Mar 27.
Brain growth in hypoplastic left heart syndrome (HLHS) is reduced before and after birth. Little is known about further brain growth until two years of age before Fontan procedure and the potential impact of type of surgery.
In a prospective, two-center study 29 patients with HLHS and variants were treated by Norwood (n = 5) or Hybrid procedure (n = 24). At two years of age a cerebral MRI was performed and brain volumes (total gray, deep gray, white matter) and cerebrospinal fluid volume were calculated using FreeSurfer image analysis suite and compared to a healthy control group (n = 8).
The total brain volumes in patients with HLHS were smaller compared to controls (HLHS: 893 ± 76 ml vs. controls: 1015 ± 148 ml, p = 0.005). This difference was found in all three brain compartments after Norwood procedure, whereas patients after Hybrid procedure had total and deep gray volumes comparable to controls. When comparing Norwood to Hybrid patients, deep gray matter volume reduction was more pronounced (Norwood: 38.4 ± 4.1 ml vs. Hybrid: 44.4 ± 3.9 ml, p = 0.005) than white matter reduction (Norwood: 255 ± 19 ml vs. Hybrid: 285 ± 31 ml, p = 0.032).
Smaller total and regional brain volumes were found two years after Norwood or Hybrid procedure in children with HLHS. The brain volume reduction was more distinct after Norwood than after Hybrid procedure. Longitudinal studies are needed to identify impact of early staged-surgeries on brain development and may become part of the decision-making process in individual patients.
在出生前后，左心发育不全综合征（HLHS）患儿的大脑生长发育会受到抑制。在 Fontan 手术之前，直到两岁，我们对进一步的大脑生长情况知之甚少，也不知道手术类型的潜在影响。
在一项前瞻性、双中心研究中，29 名 HLHS 及变异型患儿分别接受了 Norwood 手术（n=5）或 Hybrid 手术（n=24）治疗。在两岁时，对患儿进行脑部 MRI 检查，使用 FreeSurfer 图像分析套件计算脑容量（全脑灰质、深部灰质、白质）和脑脊液容量，并与健康对照组（n=8）进行比较。
HLHS 患儿的全脑容量明显小于对照组（HLHS：893±76ml 比对照组：1015±148ml，p=0.005）。在接受 Norwood 手术后，这种差异在所有三个脑区均存在，而接受 Hybrid 手术后，患儿的全脑灰质和深部灰质容量与对照组相似。与 Hybrid 患儿相比，接受 Norwood 手术的患儿深部灰质体积减少更为明显（Norwood：38.4±4.1ml 比 Hybrid：44.4±3.9ml，p=0.005），而白质体积减少则不明显（Norwood：255±19ml 比 Hybrid：285±31ml，p=0.032）。
在接受 Norwood 或 Hybrid 手术后两年，HLHS 患儿的全脑和区域性脑容量较小。与接受 Hybrid 手术相比，接受 Norwood 手术的患儿脑容量减少更为明显。需要进行纵向研究以确定早期分期手术对大脑发育的影响，并可能成为个体化患者决策过程的一部分。
