Background and objective Children with congenital heart disease (CHD) may experience difficulties in academic and daily life functioning. Intelligence and neurocognitive skills have been associated with these difficulties. The aim of this systematic review and meta-analysis is to assess intelligence in comparison with neurocognitive skills, like memory, attention and executive functions, in children with CHD.

Methods A comprehensive search of electronic databases PubMed, Embase and Cochrane was conducted for studies measuring intelligence, memory, attention and executive functions in children with CHD. Standardised mean differences (SMD’s) between the CHD-group and a healthy control group were calculated for these neurocognitive functions.

Results 28 studies were retrieved, of which 10 tested a healthy control group. Children with CHD (median age 7.4 years; median sample size 20) scored worse than healthy control children for all investigated neurocognitive functions. A medium effect size was found for intelligence (SMD = -0.53 [95% CI: -0.71 to -0.35] p < 0.00001). Effect sizes, ranging from -0.41 to -0.53, were similar for memory, global executive function and non-reaction time measures of attention and executive functions. Executive function reaction time had the largest effect size (SMD = 0.76 [95% CI: 0.48 to 1.05]; p < 0.00001).

Conclusions Children with CHD risk lower performance on intelligence and neurocognitive skills. Only executive function reaction time showed a large effect size. A high diversity in research practices and small sample sizes were also ascertained. Large, more standardised, long-term follow-up studies of neurocognitive skills are required for a better understanding of these deficits and their impact on daily life functioning.