Perioperative management of rare coagulation factor deficiency states in cardiac surgery

Abstract

Rare bleeding disorders (RBDs) include the hereditary deficiency of fibrinogen, factor (F)II, FV, FV + FVIII, FVII, FX, FXI or FXIII. RBDs do not confer a protective effect against atheromatous plaque formation, and thus the need for cardiovascular (CV) surgery in RBD patients is expected to increase with improved healthcare access (diagnosis and management) and longevity of the population. Clinical data regarding the management of RBDs in this setting are sparse, but the perioperative care team is obliged to gain a better understanding on available biological and pharmacological hemostatic agents. Perioperative management of RBDs in CV surgery is further complicated by heparin anticoagulation, haemodilution, and consumption of procoagulant and anticoagulant proteins associated with cardiopulmonary bypass (CPB). The aims of this review are to summarize pathophysiology of RBDs and laboratory monitoring pertinent to CV surgery, available factor replacement agents, and to provide the framework for perioperative coagulation management of RBD patients.

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

“…8,9 The presence of coagulation factor deficiency in patients undergoing cardiac surgery represents a challenge to the whole perioperative care team, necessitating comprehensive and individualized plans for surgery and possible factor replacement strategy. 10 This case highlights the challenges in the management of patients with FXI deficiency undergoing cardiac surgery and the need for a multidisciplinary approach. Caution is warranted when using FXI concentrate in this patient population.…”

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

“…There are several challenges and approaches in the efficacious management of patients with FXI deficiency undergoing cardiac surgery. The use of FFP, factor concentrates, and antifibrinolytic agents are among the recommended treatment options to achieve this 4 . Thrombosis may occur in 10% of patients receiving factor concentrate due to the procoagulant and antifibrinolytic effects of FXI 5 .…”

“…The use of FFP, factor concentrates, and antifibrinolytic agents are among the recommended treatment options to achieve this. 4 Thrombosis may occur in 10% of patients receiving factor concentrate due to the procoagulant and antifibrinolytic effects of FXI. 5 In 1984, Brunken et al, 6 were the first to report the successful management of perioperative bleeding during CABG surgery using FFP in two patients with FXI deficiency.…”

Perioperative Management of Hereditary Factor V Deficiency: Timing of Plasma Administration is Critical in Maximizing Hemostatic Potency of Transfused Factor V

“…While the authors concisely described the limitations of FFP transfusion in FV replacement, we felt it worthwhile to provide additional comments and views on the management of this rare hereditary bleeding disorder. 3 Firstly, the authors initially estimated that 4 units of FFP (14.3 ml/kg) would increase the FV level by about 30%, but they only observed a 15% increase. This is not surprising because an incremental change in the FV level is expected to be about 3% per unit of FFP without diuresis.…”

“…Low plasma tissue factor pathway inhibitor (TFPI) activity and residual platelet derived-FV seem important in supporting thrombin generation for hemostatic competence 6 and might explain mild bleeding tendency in FV-deficient patients. Also, given the potentially low thrombin generation and susceptibility to fibrinolysis, 3 it is 4 prudent to consider the use of tranexamic acid or -aminocaproic acid if bleeding persists after FV replacement (Fig. 1).…”