Perioperative anesthetic management in a patient with factor XI deficiency undergoing coronary artery bypass graft surgery

Abstract

Spontaneous bleeding is rare in patients with factor XI deficiency and significant bleeding usually occurs after a trauma or a surgical procedure. It is difficult to maintain hemostatic balance in these patients. In the present case report, a 68‐year‐old male patient with no chronic disease was scheduled for elective cardiopulmonary bypass surgery. Eight units of fresh‐frozen plasma (FFP) were slowly infused and the operation was initiated with the activated partial thromboplastin time (aPTT) of 34.5, which was 108.7 in the preoperative period. Tranexamic acid bolus was administered before the skin incision and continued throughout the operation. Intraoperative aPTT was measured intermittently and a total of six units of FFP were administered. After 76 minutes of cross‐clamp time, the patient was separated from cardiopulmonary bypass without any problem. There is no consensus regarding the management of bleeding during cardiac surgery in patients with factor XI deficiency. The common approach includes normalizing the factor levels via FFP infusion or factor concentrates in the preoperative period, proceeding with surgery following the replacement, and close monitoring of perioperative factor levels and aPTT values.

Perioperative Management of a Patient with Hemophilia C and Allergy to Fresh Frozen Plasma

“…Ultimately, we chose to optimize the aPTT levels using a combination of pretreatment with diphenhydramine, acetaminophen and hydrocortisone, as well as slow administration of FFP over the course of hours. Through this technique, our team was able to successfully address our patient's coagulopathy through the normalization of aPTT, which has been demonstrated to correlate more closely to the risk of clinically significant bleeding than fXI levels [ 3 , 7 ].…”

“…Treatment of patients with hemophilia C undergoing surgery has been achieved with the use of FFP until target laboratory parameters are achieved, both preoperatively and with intraoperative assessments [3,11]. Other reported treatment modalities for cardiac surgery cases, include purifed fXI replacement, activated recombinant factor VIIa, desamino-8-D-arginine vasopressin, and antifbrinolytic agents such as tranexamic acid [6,[12][13][14][15][16][17][18][19].…”

“…Affected individuals are generally asymptomatic, although significant bleeding may occur with trauma or surgery [ 2 ]. The utilization of systemic heparinization, sternotomy, extracorporeal circulation, and hypothermia during coronary artery bypass grafting (CABG) procedures results in significant trauma and disrupted hemostatic balance [ 3 , 4 ]. Factor XI levels can be normalized prior to surgery with either fresh frozen plasma (FFP) or fXI concentrate.…”

Factor XI deficiency and delayed hemorrhages after resection of choroid plexus papilloma: illustrative case

“… 15 Unexplained lengthening of APTT or history of bleeding, particularly in populations at risk, 2 , 3 might justify specific screening for the disease. Prompt diagnosis might call for protective measures such as infusion of fresh frozen plasma, tranexamic acid, or FXI concentrate 16 , 17 in preparation for elective surgical procedures. Unfortunately, most patients, including the reported case, have normal coagulation parameters, and the disease can only be suspected on a clinical basis.…”