Abnormalities in liver function and coagulation profile following the Fontan procedure

Abstract

Objective-To investigate liver function and coagulation disorders in patients with a Fontan circulation at diVerent time intervals after surgery. Design-Retrospective analysis of clinical data and cross sectional study relating liver function and coagulation profile to time since surgery, in 28 surviving patients after the modified Fontan procedure. Patients-20 patients (71%) with atriopulmonary anastomosis, seven (25%) with atrioventricular anastomosis, and one (4%) with total cavopulmonary connection. Follow up ranged from 2.0 to 21.8 years (mean 11.1). Results-Abnormal liver function tests, mainly reflecting cholestasis, were present in 21 patients who had a significantly longer follow up (p < 0.01). Protein synthesis was normal in almost all patients. Coagulation profile showed abnormalities in 22 patients. "Procoagulant" abnormalitiesthat is, decreased plasminogen and protein C activity-were found in 11 and five patients, respectively. The extent of these abnormalities was less in patients with a longer follow up. Anticoagulant abnormalities were factor V deficiency in 16 patients and factor VII deficiency in 17, resulting in a prolonged prothrombin time in 19 patients. Thirteen patients had both pro-and anticoagulant abnormalities. A prethrombotic state was present in five patients, with a significantly longer mean time interval since surgery (p = 0.05). Thus, although the individual procoagulant indices decreased with increasing time intervals since surgery, a prethrombotic state was found particularly in patients with a long term follow up. Conclusions-Mild cholestasis was mainly present in Fontan patients with a long duration of follow up. Along with laboratory procoagulant abnormalities indicating a prethrombotic state, anticoagulant abnormalities were also present. The coagulation profile varied at diVerent time intervals after surgery. Thus detailed evaluation should be performed regularly, and the use of anticoagulants should be considered in every patient. Long term prospective studies are needed to evaluate the individual fluctuations of coagulation profile over time following a Fontan procedure. (Heart 1999;82:40-46)

Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease

“…Cyanotic CHD is more commonly reported to have known hemostatic abnormalities [136][137][138][139][140][141][142] compared with acyanotic CHD 143 and acquired heart disease (Kawasaki disease, cardiomyopathy).…”

“…• Coagulation proteins: II, V, VII, VIII, IX, and X, and fibrinogen: decreased; VIII: increased [136][137][138][139][140][141][142][143] • Inhibitors of coagulation: proteins C and S and antithrombin: decreased [136][137][138][139][140][141][142][143] • Fibrinolytic proteins: plasminogen: decreased 143 • Prothrombotic genetic polymorphisms identified: factor V Leiden, prothrombin gene 20120, plasminogen G4/ G4, methylene tetrahydrofolate reductase 677 144,145 Abnormalities in hemostatic function include the following:…”

“…[136][137][138][139][140][141][142][143] Depending on the abnormality or abnormalities present, these children can be at risk for bleeding or thrombosis. The reason for the decreased production and delayed normalization of coagulation protein levels (anticoagulant and procoagulant factors) is unknown in cyanotic and acyanotic heart disease.…”

“…138,139 Recent prospective studies using homogeneous patient cohorts demonstrate decreased protein C, antithrombin III (AT III), FII, FV, FVII, FX, and plasminogen before Fontan surgery and increased FVIII and decreased protein C after Fontan surgery. 136,[140][141][142]146 Increased FVIII levels have been demonstrated to be associated with an increased risk for thrombosis. 15 Nowak-Gottl et al 144 reported 44 neonates and infants with increased levels of plasminogen activator inhibitor-1, indicating that fibrinolysis may be decreased in some children with CHD.…”

Perioperative Management of a Patient With Fontan Physiology for Posterior Spinal Fusion

“…This may be exacerbated by the presence of abnormalities of coagulation function in patients with cyanotic CHD [4]. Preoperative coagulation function should be evaluated and treatment with vitamin K or blood products instituted as needed.…”