title: 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.
authors.affiliation: Golden Jubilee National Hospital, Clydebank, United Kingdom.
authors.forename: Adarsh B
authors.initials: AB
authors.lastname: Lal
authors.forename: Ram
authors.initials: R
authors.lastname: Maruthavanan
authors.forename: Nicholas B
authors.initials: NB
authors.lastname: Scott
mesh_terms: D000293:Adolescent; D000347:Afibrinogenemia / Q000097:blood* / Q000150:complications; D001021:Aortic Valve / Q000601:surgery*; D007611:Aprotinin / Q000627:therapeutic use; D002315:Cardiopulmonary Bypass; D006801:Humans; D008297:Male
chemical_list: D007611:Aprotinin