Division of Cardiac Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pa.
Division of Biostatistics, Cincinnati Children's Hospital, Cincinnati, Ohio.
J Thorac Cardiovasc Surg. 2014 Apr;147(4):1276-82; discussion 1282-1283.e5. doi: 10.1016/j.jtcvs.2013.12.019. Epub 2014 Jan 2.
The study objectives were to compare the neurodevelopmental outcomes of preschool survivors of the Fontan procedure with those of children with congenital heart disease undergoing biventricular repair and to investigate predictors of neurodevelopmental outcome for those with single ventricle congenital heart disease, including hypoplastic left heart syndrome.
Neurodevelopmental outcomes were assessed at 4 years of age, including cognition, visual-motor integration, behavior, social skills, and academic achievement. Unadjusted outcomes were compared between patients with biventricular circulation and patients with single ventricles. Predictors of neurodevelopmental outcome were assessed in the patients with single ventricles. Multiple covariate models were evaluated using patient-related, operative, and postoperative covariates.
Neurodevelopmental evaluation was performed in 365 children, 112 after the Fontan procedure (hypoplastic left heart syndrome, n = 91; other single ventricle, n = 21) and 253 after biventricular repair. Compared with patients with biventricular circulation, patients with single ventricles performed worse in terms of processing speed, inattention, and impulsivity. Otherwise, there were no significant differences between the groups for any domain. There was a trend toward lower performance for patients with single ventricles on visual motor integration. Outcomes for patients with hypoplastic left heart syndrome were not worse than for other forms of functional single ventricle. Patient factors were more important predictors of neurodevelopmental outcomes than were operative management variables.
In this cohort, unadjusted neurodevelopmental outcomes for preschool survivors of the Fontan procedure are similar to those for children with congenital heart disease undergoing biventricular repair for most domains. Among the patients undergoing the Fontan procedure, hypoplastic left heart syndrome was not associated with worse outcomes compared with other forms of single ventricle.
本研究旨在比较 Fontan 术后学龄前生存者与接受双心室修复术的先天性心脏病患儿的神经发育结局，并探讨单心室先天性心脏病（包括左心发育不全综合征）患儿神经发育结局的预测因素。
在 4 岁时评估神经发育结局，包括认知、视动整合、行为、社会技能和学业成绩。比较双心室循环患儿与单心室患儿的未调整结局。评估单心室患儿的神经发育结局预测因素。使用与患者相关的、手术和术后协变量评估多变量模型。
对 365 例儿童进行了神经发育评估，其中 112 例接受了 Fontan 手术（左心发育不全综合征，n=91；其他单心室，n=21），253 例接受了双心室修复术。与双心室循环患儿相比，单心室患儿在处理速度、注意力不集中和冲动方面表现较差。但在其他领域，两组之间没有显著差异。单心室患儿在视动整合方面的表现也有下降趋势。左心发育不全综合征患儿的结局并不比其他形式的功能性单心室差。患者因素是神经发育结局的更重要预测因素，而不是手术管理变量。
在本队列中，Fontan 术后学龄前生存者的未调整神经发育结局与接受双心室修复术的先天性心脏病患儿大多数领域相似。在接受 Fontan 手术的患者中，左心发育不全综合征与其他形式的单心室相比，结局并未恶化。
