Congenital Heart Defects, Special Needs, and Developmental Vulnerability in Singleton Children at School Age: A Population-Based Data Linkage Study

BACKGROUND: We aimed to quantify the risk of developmental difficulties at school entry among children with congenital heart defects (CHD), considering CHD severity. Elevated risks are established for children with complex CHD, but little is known about other CHD categories.

METHODS: In this South Australian whole-of-population data linkage study, standardized assessments from the Australian Early Development Census were linked with perinatal records (2002-2014) and birth defects. Among singleton children, we compared the prevalence of developmental difficulties (special needs or developmental vulnerability) for subgroups with CHD (severe surgical, severe nonsurgical, non-severe), and for unaffected peers, and calculated adjusted relative risks. Children with diagnosed syndromes or chromosomal disorders were excluded.

RESULTS: Of 67 990 singleton children, 656 had a CHD diagnosis (9.6 per 1000 live births). Children with CHD had higher levels of special needs (13% [95% CI, 11-16]) or at least 1 developmental vulnerability (26% [95% CI, 23-29]) compared with children with no CHD (5.5% [95% CI, 5.3-5.7]; 21.6% [95% CI, 21.3-21.9]). The risk of developmental difficulties was elevated among children with complex CHD (ie, severe surgical, adjusted relative risk, 1.42 [95% CI, 1.20-1.68]) but also among severe nonsurgical CHD (adjusted relative risk, 1.52 [95% CI, 0.82-2.83]) and non-severe CHD (adjusted relative risk, 1.35 [95% CI, 1.18-1.55]), although for severe nonsurgical CHD the CI includes the null.

CONCLUSIONS: Developmental difficulties were prevalent among children with CHD irrespective of the need for early surgical intervention. The basis for this broad effect requires further study. Monitoring of development is warranted among all children with CHD.