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 gamma 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.

Figure 1

Dot-density plot of the liver tests. Abnormal values (Z value > 2.0 or ⩽ 2.0) are represented by solid circles. The percentages of abnormal values are in parentheses. The overlap of normal and abnormal values is caused by different age related control values. ALT, alanine aminotransferase; AST, aspartate aminotransferase; GGT, γ glutamyltransferase.

Figure 2

Dot-density plot of serum total protein, albumin, and prealbumin. Abnormal values (Z value > 2.0 or ⩽ 2.0) are represented by solid circles. The overlap of normal and abnormal values is caused by different age related control values.

Figure 3

Dot-density plot of the coagulation factors. Abnormal values (Z value > 2.0 or ⩽ 2.0) are represented by solid circles. The percentages of abnormal values are in parentheses. The overlap of normal and abnormal values is caused by different age related control values. INR, international normalised ratio.

Figure 4

Relation between procoagulant and anticoagulant factors with respect to serum prealbumin. The values were standardised (Z value) according to age matched control groups. The 95% confidence and prediction lines are shown.

Figure 5

Relation between procoagulant factor VII and anticoagulant factors protein C and antithrombin III. The values were standardised (Z value) according to age matched control groups. The 95% confidence and prediction lines are shown.

Figure 6

Two dimensional contour graph illustrating the relations between factor VII, protein C, and antithrombin III. The contours of the z axis and the numbers in italics represent the Z values of antithrombin III. All patients with decreased protein C and normal factor VII had either normal or increased antithrombin III concentration.