Objective To examine the relative risk (RR) of congenital heart disease (CHD) in twins compared with singletons, according to chorionicity.

Methods Twins and singletons with CHD notified to the Northern Congenital Abnormality Survey between 1998 and 2010 were included in this population-based study. Information on chorionicity was obtained from the Northern Survey of Twins and Multiple Pregnancy. Prevalence was calculated as the number of cases occurring in live births, late miscarriages (20–23 weeks), stillbirths (≥24 weeks) and terminations of pregnancy for fetal anomaly, per 10 000 total births. The risk of CHD in twins compared with singletons was estimated using Poisson regression.

Results There were 399 414 singleton births of which 2984 (0.7%) had CHD. Among 11 871 twin births, 154 (1.3%) had CHD; one twin was affected by CHD in 2.5% of twin pregnancies. Of 8605 dichorionic (DC) births and 2317 monochorionic (MC) births, 96 (1.1%) and 47 (2.0%) were associated with CHD. Compared with singletons, twins were at significantly increased risk of CHD (RR=1.73, 95% CI 1.48 to 2.04; p<0.001). MC twins were at 82% significantly increased risk of CHD compared with DC twins (RR=1.82, 95% CI 1.29 to 2.57; p<0.001). The RR of severe and mild CHD was particularly high in MC twins compared with singletons (292% increased risk, RR=3.92, 95% CI 1.25 to 12.30, p=0.02 and 207% increased risk, RR=3.07, 95% CI 2.20 to 4.28; p<0.001).

Conclusions Compared with singletons, twins were at increased risk of CHD, the risk being substantially higher among MC twins. This information is important for health professionals when counselling women with twin pregnancies.