Hemophilia: A Review of Perioperative Management for Cardiac Surgery

Abstract

Hemophilia is an X-linked, recessive bleeding disorder. Improvements in factor replacement therapy and overall approach to hemophilia management have increased the lifespan and quality of life for patients with hemophilia. Thus, they are presenting for cardiac surgery related to age-related atherosclerosis, vascular disorders, and degenerative valvular disease. Although challenging, cardiac surgery can be performed safely with appropriate planning, using a multidisciplinary approach. This article provides a narrative review and framework for the perioperative management of patients with hemophilia A and B undergoing cardiac surgery.

Keywords: cardiac surgery; cardiopulmonary bypass; factor IX; factor VIII; hemophilia A; hemophilia B; thromboelastography; thromboelastometry.