To identify regional cerebral blood flow (rCBF) alterations in children and adolescents with congenital heart disease (CHD) in relation to neurocognitive outcomes using a nonbiased data-driven approach. This is a prospective, observational study of children and adolescents with CHD without brain injury and healthy controls using pseudo-continuous arterial spin labeling (pCASL) MRI. Quantitative rCBF was compared between participants with CHD and healthy controls using a voxelwise data-driven method. Mediation analysis was then performed on a voxelwise basis, with the grouping variable as the independent variable, neurocognitive outcomes (from the NIH Toolbox Cognitive Battery) as the dependent variables, and rCBF as the mediator. After motion correction, a total of 80 studies were analyzable (27 for patients with CHD, 53 for controls). We found steeper age-related decline in rCBF among those with CHD compared to normal controls in the insula/ventromedial prefrontal regions (salience network) and the dorsal anterior cingulate and precuneus/posterior cingulate (default mode network), and posterior parietal/dorsolateral prefrontal (central executive network) (FWE-corrected P< 0.05). The reduced rCBF in the default mode/salience network was found to mediate poorer performance on an index of crystallized cognition from the NIH Toolbox Cognitive Battery in those with CHD compared to controls. In contrast, reduced rCBF in the central executive network/salience network mediated reduced deficits in fluid cognition among patients with CHD compared to controls. Regional cerebral blood flow alterations mediate domain-specific differences in cognitive performance in children and adolescents with CHD compared to healthy controls, independent of injury, and are likely related to brain and cognitive reserve mechanisms. Further research is needed to evaluate the potential of interventions in CHD targeting regional cerebral blood flow across lifespan.

使用无偏数据驱动方法确定先天性心脏病 (CHD) 儿童和青少年的局部脑血流 (rCBF) 改变与神经认知结果的关系。这是一项针对没有脑损伤的先心病儿童和青少年的前瞻性观察性研究，使用伪连续动脉自旋标记 (pCASL) MRI。使用体素数据驱动方法比较了冠心病参与者和健康对照者之间的定量 rCBF。然后在体素基础上进行中介分析，分组变量作为自变量，神经认知结果（来自 NIH 工具箱认知电池）作为因变量，rCBF 作为中介变量。运动校正后，共有 80 项研究可供分析（CHD 患者 27 项，对照组 53 项）。我们发现，在脑岛/腹内侧前额叶区域（显着网络）和背侧前扣带回和楔前叶/后扣带回（默认模式网络），以及后顶叶/背外侧前额叶区域，与正常对照相比，先心病患者的 rCBF 随年龄下降的幅度更大（中央执行网络）（FWE 校正P < 0.05）。与对照组相比，默认模式/显着性网络中减少的 rCBF 会介导 NIH 工具箱认知电池在结晶认知指数上的较差表现。相比之下，与对照组相比，中央执行网络/显着网络中 rCBF 的减少介导了 CHD 患者流体认知缺陷的减少。与健康对照组相比，区域性脑血流改变介导了患有先天性心脏病的儿童和青少年认知表现的特定领域差异，与损伤无关，并且可能与大脑和认知储备机制有关。需要进一步的研究来评估冠心病干预措施在整个生命周期中针对局部脑血流的潜力。