Coagulation profile and liver function in 102 patients after total cavopulmonary connection at mid term follow up

Abstract

Objective: To examine coagulation factors and liver function test abnormalities in patients after total cavopulmonary connection (TCPC). Design: Cross sectional study comprising clinical and echocardiographic evaluation, and biochemical and coagulation profile screening. Setting: Tertiary referral centre. Methods: 102 patients aged 4-24 years (median 10 years) at one to eight years (median five years) after TCPC were examined. All patients were maintained on a low dose of aspirin. 96% of patients were in a good clinical condition (New York Heart Association class I or II). No intracardiac thrombi were detected on echocardiography and ventricular function was good in 91% of patients. Results: Total bilirubin was increased in 27% and c glutamyltransferase in 54% of patients. Serum total protein, albumin, and prealbumin were normal in almost in all patients. Compared with the control group, patients after TCPC had significantly lower fibrinogen, factor V, factor VII, and protein C concentrations, prolonged international normalised ratio, and increased antithrombin III concentration. Factor V concentration was abnormally decreased in 35%, factor VII in 16%, and protein C in 28% of patients. Antithrombin III was increased in 23% of patients. Factor VII, factor V, protein C, and antithrombin III correlated significantly with serum prealbumin. There was also a significant correlation between procoagulant factor VII and both anticoagulant protein C and antithrombin III. Conclusions: Almost half of patients after TCPC had laboratory signs of mild cholestasis. Decreased liver synthesis of procoagulant and anticoagulant factors was observed but overall coagulation homeostasis appeared to be in balance in this selected group of patients with a good clinical outcome.

Protein Losing Enteropathy after Fontan Surgery – Clinical and Diagnostical Aspects

“…Also mesenteric hypoperfusion due to the increased mesenteric vascular resistance could serve as a possible trigger for the PLE development [17]. The results of Chaloupecky et al [18] support the hypothesis that the abnormalities in the balance of coagulation factors observed in patients after Fontan operation are related to the coagulation factor production in the liver. The passive lymph loss caused by high central venous pressure does not explain the selective loss of CD4+ lymphocytes.…”

Congenital Heart Disease in the Older Adult

“…195,196 Serum protein and albumin and prealbumin levels are usually normal in most patients unless they have protein-losing enteropathy. [194][195][196] Some biochemical markers may be used as surrogates for liver biopsy to estimate the degree of liver fibrosis. There are composite blood tests that combine the quantitative results of 6 serum biochemical markers to provide a numerical quantitative estimate of liver fibrosis that corresponds to the Metavir scoring system.…”

“…184,194 At midterm follow-up (median of 10-12 years after initial Fontan operation), the most common laboratory abnormalities are elevated levels of γ-glutamyltranspeptidase, alkaline phosphatase, and total bilirubin, consistent with cholestasis. [194][195][196] Abnormalities in coagulation are commonly seen that involve both procoagulant factors and anticoagulant factors. 195,196 Serum protein and albumin and prealbumin levels are usually normal in most patients unless they have protein-losing enteropathy.…”

“…[194][195][196] Abnormalities in coagulation are commonly seen that involve both procoagulant factors and anticoagulant factors. 195,196 Serum protein and albumin and prealbumin levels are usually normal in most patients unless they have protein-losing enteropathy. [194][195][196] Some biochemical markers may be used as surrogates for liver biopsy to estimate the degree of liver fibrosis.…”

Hypercoagulable State and Methylenetetra- hydrofolate Reductase (MTHFR) C677T Mutation in Patients with Beta-Thalassemia Major in Kuwait

“…In our patients acquired deficiencies are most probably attributable to liver dysfunction caused by multiple blood transfusions, hemosiderosis, chronic liver disease and viral infections, which affect the synthetic ability of the liver for these factors. The prevalence of liver dysfunction in thalassemic patients and the role of the liver in synthesis of anticoagulant factors (PC, PS, AT) [20] lead to the conclusion that low levels of these natural anticoagulants detected in patients with thalassemia major in Kuwait is most probably due to acquired deficiencies resulting from liver dysfunction as evidenced by high levels of ALT and AST liver enzymes (table 1), resulting from iron overload and infection. …”