Perioperative Management of a Patient with Factor XI Deficiency Undergoing Cardiac Surgery

Abstract

Background: Factor XI deficiency (FXID), is a rare disorder of the coagulation system and the incidence of FXID is estimated to be one in a million. It is claimed to be associated with prominent bleeding in case of trauma and surgery irrelevant to the FXI level. The treatment modality of FXID varies from Fresh frozen plasma (FFP) to the plasma-derived FXI concentrate depending on country and medical center. In this case report, we present a patient with FXID who underwent coronary artery bypass grafting (CABG), and his perioperative management.Case report: A 52-year-old, male patient was admitted to the cardiovascular surgery department and prepared for surgery. He was prepared in accordance with hematologist's recommendation and underwent CABG successfully. He was discharged from hospital on the seventh postoperative day without any complication. Conclusion:The diagnosis of FXID is mainly based upon suspicion in cases of elevated aPTT or unusual bleeding after or during the surgery or trauma, as well as family history like in our case. Suspicion and proper history taking warned us to take precautions and manage the case successfully. In case of cardiovascular surgery of FXID patients, a team-based approach including a cardiologist, surgeon, intensivist and a hematologist with an on-site coagulation laboratory is essential.