Family history of cardiovascular events and endothelial dysfunction in children with familial hypercholesterolemia

Objectives: in patients with familial hypercholesterolemia (FH), the propensity towards atherosclerosis may vary considerably. In the general population, a positive family history is associated with an increased risk for cardiovascular events. Since endothelial dysfunction is predictive for future cardiovascular events, we evaluated whether FH-children with a positive family history of premature cardiovascular disease have more pronounced endothelial dysfunction compared to children with a negative family history. Study design: 50 FH children, 10–18 years, participated in this study. Thirty-one children had a positive family history for cardiovascular events (fh+) and 19 children had no events in the family (fh−). Nineteen matched siblings participated as controls. Endothelial function was assessed by testing the flow mediated dilatation (FMD) of the brachial artery. Results: baseline characteristics were comparable for fh+, fh− and controls. Lipid levels were significantly higher in FH children. In FH, FMD was impaired compared to controls (11.7±4.4 vs. 15.6±6.8%, P<0.03). In addition, FMD was significantly lower in fh+ compared to fh− (10.7±9.9 vs. 13.3±4.6%, P<0.05). Conclusion: In FH-children, endothelial function is impaired compared to matched controls. This impairment is most pronounced in FH children with a positive family history of premature cardiovascular disease.