Coagulation factor abnormalities as possible thrombotic risk factors after Fontan operations

CT and MRI Evaluation of the Fontan Pathway: Pearls and Pitfalls

“…In this situation, Fontan circulation has an imbalance between procoagulant and anticoagulant factors [17]. High mortality from thromboembolic events is also related to arrhythmia as a result of increased atrial pressure and distention, particularly in atriopulmonary Fontan procedures.…”

Management of the Fontan circulation in the adult patient

“…The abnormalities in clotting factors occur due to the Fontan circuit formation (48)(49)(50) and although the incidence of thromboembolism is lower in TCPC Fontan patients, (21) it can nevertheless occur (51) and we advocate anticoagulation with warfarin for all Fontan patients regardless of type.…”

Increased P-Selectin Expression on Platelets and Decreased Plasma Thrombomodulin in Fontan Patients

“…The elevated TAT levels detected in the present study suggest the presence of coagulation abnormalities after the Fontan procedure, which is in agreement with the findings of previous studies. 1,9,10 Thrombosis in the Fontan procedure is often silent and it is not clear exactly when the thrombus forms. Nevertheless, the probability of freedom from thromboembolism decreases with the interval after the Fontan procedure.…”

“…Fontan circulation is associated with an increased risk of thromboembolism [1][2][3] and the prevalence of thromboembolic events has been reported to range from 3% to 30% among Fontan patients. [4][5][6][7][8] Various abnormalities in the coagulation system have been postulated as the mechanism of the increased incidence of thromboembolism in Fontan patients, including decreased levels of protein C, protein S, and antithrombin III.…”

“…[4][5][6][7][8] Various abnormalities in the coagulation system have been postulated as the mechanism of the increased incidence of thromboembolism in Fontan patients, including decreased levels of protein C, protein S, and antithrombin III. 1,9,10 Although platelets play an important role in thrombus formation, and collagen-induced and adenosine-induced platelet aggregation are elevated in patients after the Fontan procedure, 11 whether platelets are indeed activated after the Fontan procedure remains unknown. P-selectin is an adhesion molecule found in the secretory granules of platelets, and is mobilized to the membrane surface on activation.…”