Heritable coagulopathies in pregnancy

Heritable coagulopathies are leading causes of maternal thromboembolism and are associated with an increased risk of maternal and perinatal morbidity and mortality. The most common of these disorders are antithrombin III deficiency, protein C deficiency, protein S deficiency, activated protein C resistance resulting from the factor V Leiden mutation, elevated prothrombin activity associated with a mutation in the prothrombin gene, and hyperhomocystinemia. The maternal risk of a thromboembolic episode is increased by a factor of eight in the presence of any of these heritable states. In addition, the relative risk for a stillbirth in the presence of one of these disorders is 3.6. These conditions are also associated with intrauterine growth retardation and preeclampsia. Proper management of heritable coagulopathies during pregnancy is essential to reduce the risk of these serious sequelae. Patients with newly diagnosed deep-vein thromboses or pulmonary emboli should be treated with therapeutic levels of unfractionated or low molecular weight heparin, followed by subsequent prophylactic heparin therapy. All patients with a history of thromboembolism before pregnancy or evidence of any of these coagulopathies may be offered prophylactic therapy with low molecular weight heparin. Patients with antithrombin III deficiency should receive full therapeutic heparin therapy for the entire pregnancy, irrespective of their thromboembolic history. Postpartum therapy with either heparin or warfarin is required in all cases.

Obstetricians & Gynecologists, Family Physicians

After completion of this article, the reader will be able to describe the various heritable coagulopathies that can complicate pregnancy, to state the potential adverse effects of heritable coagulopathies in pregnancy, and to explain the management of heritable coagulopathies during pregnancy.