Bellinger David C, Rivkin Michael J, DeMaso David, Robertson Richard L, Stopp Christian, Dunbar-Masterson Carolyn, Wypij David, Newburger Jane W
1Department of Neurology,Boston Children's Hospital,Harvard Medical School,Boston,MA,United States of America.
2Department of Psychiatry,Boston Children's Hospital,Harvard Medical School,Boston,MA,USA.
Cardiol Young. 2015 Feb;25(2):338-47. doi: 10.1017/S1047951114000031. Epub 2014 Feb 11.
Few data are available on the neuropsychological, behavioural, or structural brain imaging outcomes in adolescents who underwent corrective surgery in infancy for tetralogy of Fallot.
In this single-centre cross-sectional study, we enrolled 91 adolescents (13-16 years old) with tetralogy of Fallot and 87 referent subjects. Assessments included tests of academic achievement, memory, executive functions, visual-spatial skills, attention, and social cognition, as well as brain magnetic resonance imaging.
Genetic abnormalities or syndromes were present in 25% of tetralogy of Fallot patients, who had markedly greater neuropsychological morbidities than did patients without a syndrome. However, even patients without a syndrome performed significantly worse than the referent group or population norms in all of the neuropsychological domains assessed. In multivariable regression in those without a genetic/phenotypic syndrome, the strongest predictors of adverse late neurodevelopmental outcomes included a greater number of complications at the first operation, more total surgical complications across all operations, and occurrence of post-operative seizures. The presence of at least one abnormality on structural magnetic resonance imaging was more frequent in tetralogy of Fallot patients than the referent group (42% versus 8%).
Adolescents with tetralogy of Fallot are at increased neurodevelopmental risk and would benefit from ongoing surveillance and educational supports even after childhood.
关于婴儿期接受法洛四联症矫正手术的青少年的神经心理学、行为或脑结构成像结果的数据很少。
在这项单中心横断面研究中，我们纳入了91名法洛四联症青少年（13 - 16岁）和87名对照受试者。评估包括学业成绩、记忆、执行功能、视觉空间技能、注意力和社会认知测试，以及脑磁共振成像。
25%的法洛四联症患者存在基因异常或综合征，这些患者的神经心理学发病率明显高于无综合征的患者。然而，即使是无综合征的患者，在所有评估的神经心理学领域的表现也显著低于对照组或人群常模。在无基因/表型综合征的患者的多变量回归分析中，不良晚期神经发育结果的最强预测因素包括首次手术时更多的并发症、所有手术中更多的总手术并发症以及术后癫痫发作。法洛四联症患者结构磁共振成像至少存在一种异常的情况比对照组更常见（42%对8%）。
法洛四联症青少年的神经发育风险增加，即使在儿童期后，持续的监测和教育支持也会使他们受益。
