LAUSR

Abstract Congenital afibrinogenemia is a rare autosomal recessive hematologic disorder that results in coagulopathy due to the complete lack of fibrinogen production. While the common manifestation of the disease entails a bleeding diathesis, these patients are also at high risk of thromboembolic event due to abnormal coagulation cascade. We present a case in which a patient with congenital afibrinogenemia complicated by chronic thromboembolic pulmonary hypertension undergoes pulmonary thromboendarterectomy requiring cardiopulmonary bypass and deep hypothermic circulatory arrest. We discuss the clinical management technique with the use of human fibrinogen concentrate. We also discuss the potential safety implications and recent literature involving the use of human fibrinogen concentrate during cardiac surgery involving cardiopulmonary bypass and deep hypothermic circulatory arrest.