CONTEXT Cognitive function and executive function (EF) impairments contribute to the long-term burden of congenital heart disease (CHD). However, the degree and profile of impairments are insufficiently described. OBJECTIVE To systematically review and meta-analyze the evidence on cognitive function and EF outcomes in school-aged children operated for CHD and identify the risk factors for an unfavorable outcome. DATA SOURCES Cochrane, Embase, Medline, and PsycINFO. STUDY SELECTION Original peer-reviewed studies reporting cognitive or EF outcome in 5- to 17-year old children with CHD after cardiopulmonary bypass surgery. DATA EXTRACTION Results of IQ and EF assessments were extracted, and estimates were transformed to means and SE. Standardized mean differences were calculated for comparison with healthy controls. RESULTS Among 74 studies (3645 children with CHD) reporting total IQ, the summary estimate was 96.03 (95% confidence interval: 94.91 to 97.14). Hypoplastic left heart syndrome and univentricular CHD cohorts performed significantly worse than atrial and ventricular septum defect cohorts (P = .0003; P = .027). An older age at assessment was associated with lower IQ scores in cohorts with transposition of the great arteries (P = .014). Among 13 studies (774 children with CHD) reporting EF compared with controls, the standardized mean difference was −0.56 (95% confidence interval: −0.65 to −0.46) with no predilection for a specific EF domain or age effect. LIMITATIONS Heterogeneity between studies was large. CONCLUSIONS Intellectual impairments in CHD are frequent, with severity and trajectory depending on the CHD subtype. EF performance is poorer in children with CHD without a specific EF profile. The heterogeneity in studied populations and applied assessments is large. A uniform testing guideline is urgently needed.

Background Over the past decades, survival rates of children born with congenital heart disease (CHD) have increased dramatically. Progress in prenatal diagnosis, less-invasive catheter techniques and perioperative intensive care as well as surgical techniques have led to an increased focus on extracardiac comorbidities, including potential neurodevelopmental sequelae associated with CHD. A growing body of literature reports impairments in early and school-age developmental outcome; however, there is a substantial variability in the spectrum of examined CHD types, assessment ages and applied test batteries. Furthermore, little information is available on executive function impairments in this population. Therefore, the aim of this systematic review is to determine the impact of CHD on intellectual outcome and executive functioning at school age and to determine risk factors for impaired outcomes by means of a systematic search. Methods A systematic review of literature that reports neurodevelopmental outcome in children with CHD undergoing cardiopulmonary bypass surgery. Intelligence quotient or executive function scores will be considered primary outcomes. Databases such as Cochrane, EMBASE, MEDLINE and PsycINFO will be searched. Discussion The results of this systematic review will summarize the current evidence on intellectual and executive function outcome after cardiopulmonary bypass surgery in school-age children with CHD. This review will thus be the basis for better patient and parental counselling and the establishment of tailored follow-up programmes and interventional trials. Systematic review registration In accordance with the guidelines, our systematic review protocol was registered with the International Prospective Register of Systematic Reviews (PROSPERO) on January 9, 2019 (CRD42018086568). PROSPERO CRD42019118736.