Management Considerations for a Heterozygous Protein C Deficient Patient Undergoing Open Heart Surgery with Cardiopulmonary Bypass

Abstract

The protein C system is an important anti thrombotic pathway that regulates the coagulation system by inactivating procoagulant factors Va and VIlla and enhancing clot lysis by inhibiting the activity of plasminogen activator inhibitor. Protein C deficiency, therefore, is a hypercoaguable state characterized by increased risk of thromboembolic events such as stroke, pulmonary embolism, and venous thrombosis. Extra caution would be warranted in patients with protein C deficiency who undergo cardiopulmonary bypass and are naturally predisposed to hypercoaguability. To our knowledge, however, the management considerations of a patient with protein C deficiency undergoing cardiopulmonary bypass have not been published. This case report presents a pediatric patient with heterozygous protein C deficiency and a prior history of venous thrombosis after open heart surgery who underwent repeat open heart surgery with cardiopulmonary bypass. Initiation of anticoagulation therapy upon admission to reduce procoagulant levels, the use of plasma replacement therapy to increase protein C levels prior to cardiopulmonary bypass, and the careful reinstatement of anticoagulation therapy once postoperative bleeding is controlled, are preliminary suggestions to decrease the risk of thrombotic events in this unusual patient population.