An Unusual Cause of a Prolonged Activated Coagulation Time During CardiacSurgery: Congenital Hypofibrinogenemia

摘要

THE ACTIVATED COAGULATION TIME (ACT) is commonly used to monitor heparin anticoagulation during cardiac surgery with cardiopulmonary bypass (CPB). Prolongation of the ACT can also result from coagulation factor deficiencies, producing a clinical dilemma.Congenital hypofibrinogenemia is a rare inherited coagulation disorder with only about 250 reported cases. Patients with hypofibrinogenemia remain asymptomatic until fibrinogen levels are <60 mg/dL. The routine preoperative screening tests of coagulation are insufficient to detect subtle disorders of fibrinogen. The authors found no previous reports of the management of CPB in patients with congenital hypofibrinogenemia. Therefore, a case with prolonged ACTs during cardiac surgery caused by preoperatively undiagnosed hypofibrinogenemia is presented.