Aortic valve repair in a patient with congenital afibrinogenemia.

Abstract

We describe the management of cardiopulmonary bypass (CPB) in a patient with congenital afibrinogenemia (AF). Although this state is akin to the state after hirudin administration, where CPB has been successfully performed, heparinization is still essential to preserve other clotting factors and prevent excessive bleeding. Aprotinin further helps in preservation of clotting factors. There are case reports of thrombosis associated with AF, but aprotinin can be safely used because it is, by definition, an anticoagulant. Cryoprecipitate effectively restores the fibrinogen and produces normal clotting.