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PEDIATRIC ANESTHESIA

Aortic Valve Repair in a Patient with Congenital Afibrinogenemia

Golden Jubilee National Hospital, Clydebank, United Kingdom

Address correspondence and reprint requests to Adarsh B. Lal, Consultant Anesthetist, Golden Jubilee National Hospital, Beardmore St., Clydebank G81 4HX, United Kingdom. Address e-mail to adarsh.lal{at}gjnh.scot.nhs.uk.

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.

This article has been cited by other articles:

				L. H.A. Sanders, B. J. Anderson, J. Shehatha, M. Clarson, and J. A. Mundy Aortic valve replacement and coronary artery bypass grafting in a rare case of congenital hypofibrinogenemia. Ann. Thorac. Surg., October 1, 2009; 88(4): 1329 - 1331. [Abstract] [Full Text] [PDF]