Management of severe factor <scp>XI</scp> deficiency in cardiac surgery: A case report and review of the literature

Azar, Sharl; Salomon, Ophira; Taylor, Jason

doi:10.1111/hae.13311

Cited by 9 publications

(12 citation statements)

References 13 publications

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“…10,14 Additionally, there are also large differences in the timetables that are used, for example, 2-to 4-hour dose intervals or continuous infusion for 6 to 7 days. 3,4,[14][15][16][17][18][19][20] The earlier findings of our group indicated that 90 µg/kg rFVIIa (the commonly used dose in hemophilia) caused a substantially higher TG in patients with severe FXI deficiency compared with plasma from severe hemophilia A or B patients, 10,21 and that 10 to 20 μg/kg rFVIIa was sufficient to normalize TG. 10,21 Indeed, the idea behind reducing the administrated dose of rFVIIa is related to the increased levels of FVIII following trauma or surgery in patients with FXI deficiency, in addition to increased tissue factor, both of which enable compensation for reduced thrombin activatable fibrinolysis inhibitor and higher levels of tissue factor pathway inhibitor (TFPI) related to reduced levels of FXI.…”

Section: Discussionmentioning

confidence: 59%

Single Low Dose of rFVIIa Combined with Antifibrinolytic Agent is a Simple and Safe Treatment for Factor XI-Deficient Patients undergoing Surgery

et al. 2019

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Background Factor XI (FXI) deficiency is a rare autosomal bleeding disorder. The rarity of spontaneous bleeding and absence of optimal tools to predict the bleeding risk in FXI-deficient patients hamper the standardization of prophylactic treatment enabling them to undergo major surgeries without blood products. Objectives We explored the effectiveness of a single and very low dose of recombinant factor VIIa (rFVIIa) along with tranexamic acid (TXA) as prophylactic treatment for FXI-deficient patients undergoing various types of surgery at various sites of injury. We studied the potential use of thrombin generation (TG) as a surrogate tool for predicting thrombogenicity. Patients and Methods Our cohort consisted of 10 patients with severe FXI deficiency undergoing 12 interventions. Patients received a single dose of 10 to 15 μg/kg rFVIIa at the end of surgery in addition to TXA initiated 2 hours before surgery at the dose of 4 g/day for 3 to 5 days. TG was tested before and 30 minutes after rFVIIa administration. Results All operations were uneventful and none of the patients bled excessively or required blood products. No thrombotic event was reported, and the postoperative hospitalization duration was comparable to that of patients without bleeding disorders. TG performed at the peak of rFVIIa was below the curve of healthy controls, thus confirming that the administered dose was not thrombogenic. Conclusion A single very low dose of rFVIIa along with TXA is a simple and safe treatment to control hemostasis in severe FXI-deficient patients undergoing diverse type of surgical procedure at various sites.

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Section: Discussionmentioning

confidence: 59%

Single Low Dose of rFVIIa Combined with Antifibrinolytic Agent is a Simple and Safe Treatment for Factor XI-Deficient Patients undergoing Surgery

et al. 2019

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“…If a FXI inhibitor is present, FXI suppletion is not beneficial and successful management in case of FXI inhibitor has been achieved with low dose recombinant FVIIa and antifibrinolytics. 4 To the authors' knowledge, this is the first case report of a serious complication during extracorporeal circulation after preoperative administration of FXI concentrate despite adequate heparin treatment. There are several reports of arterial thrombosis or venous thromboembolic complication after treatment with FXI concentrate, some of which were fatal, however not in cardiac surgery.…”

Section: Discussionmentioning

confidence: 81%

Clot Formation in Cardiopulmonary Bypass Circuit After Application of Factor XI Concentrate

Hulsman

Meulen

Talacua

et al. 2020

Journal of Cardiothoracic and Vascular Anesthesia

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“…Teruya et al 12 reported a female patient with severe FXI deficiency undergoing aortic valve replacement, who also underwent exchange transfusion and FFP infusion due to the presence of an inhibitor, however, massive bleeding and tamponade were observed in the postoperative period. However, Gay et al 13 used tranexamic acid infusion without plasma exchange throughout the procedure until the third postoperative day in a patient with a factor inhibitor. Fitzsimons et al 14 reported a case with severe FXI deficiency undergoing redo aortic valve replacement, in which the patient received perioperative FFP replacement and aminocaproic acid infusion which began intraoperatively and continued for 72 hours after the surgery to manage the bleeding.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2020

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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.

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Management of severe factor XI deficiency in cardiac surgery: A case report and review of the literature

Cited by 9 publications

References 13 publications

Single Low Dose of rFVIIa Combined with Antifibrinolytic Agent is a Simple and Safe Treatment for Factor XI-Deficient Patients undergoing Surgery

Single Low Dose of rFVIIa Combined with Antifibrinolytic Agent is a Simple and Safe Treatment for Factor XI-Deficient Patients undergoing Surgery

Clot Formation in Cardiopulmonary Bypass Circuit After Application of Factor XI Concentrate

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

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