Background and aims: Maternal hyperglycaemia and hyperhomocysteinaemia are risk factors for congenital heart disease (CHD). These metabolic derangements and deranged lipid levels are associated with adult cardiovascular disease. We examined whether maternal lipid levels are associated with the risk of CHD offspring. Methods and Results: From 2003 onwards, a case-control study was conducted. Participants were mothers of children with (n = 261) and without (n = 325) CHD. At around 16 months after the index-pregnancy, maternal lipid levels were determined. Maternal characteristics and lipid levels were compared by Student's t-test. In a multivariable logistic regression model, risk estimates were calculated for associations between CHD and lipid levels. Adjustments were made for maternal age, diabetes, ethnici Case mothers showed higher cholesterol (4.9 vs. 4.7 mmol l(-1), P < 0.05), low-density lipo-protein (LDL)-cholesterol (3.2 vs. 3.0 mmol l(-1), P < 0.05), apolipoprotein B (84.0 vs. 80.0 mg dl (-1), P < 0.01) and homocysteine (10.8 vs. 10.2 mu mol l(-1), P < 0.05) than controls. LDL-cholesterol above 3.3 mmol l(-1) (OR 1.6 (95% CI, 1.1-2.3)) and apolipoprotein B above 85.0 mg dl(-1) were associated with an almost twofold increased CHD risk (OR 1.8 (95% CI, 1.2-2.6)). This was supported by elevate Conclusion: A mildly deranged maternal lipid profile is associated with an increased risk of CHD offspring. (C) 2010 Elsevier B.V. All rights reserved.