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The incidence of thrombotic events following cardiopulmonary bypass (CPB) in patients receiving surgical repair or palliation of congenital heart defects (CHD) is as high as 16%. Protein C, an intrinsic anticoagulation protease which, when activated, breaks down factor Va of the coagulation system, aids in maintaining a normal procoagulant/anticoagulant balance. Resistance of factor Va to degradation by activated protein C occurs and predisposes to thrombotic events. The resistance of factor Va to such degradation is, in the majority of cases, due to a genetic mutation referred to as factor V Leiden (FVLeiden). The presence of FVLeiden can be diagnosed using a DNA based assay. The prevalence of FVLeiden in the with CHD has not been determined. The objective of this study was to determine the prevalence of FVLeiden in patients with CHD.

Prevalence of factor V Leiden in a population of patients with congenital heart disease