Coagulation profile, hepatic function, and hemodynamics following Fontan-type operations

Abstract

We analyzed retrospectively the relationship between coagulation profile, and either hepatic function or hemodynamics, in patients who had undergone a Fontan-type procedure, comparing them, first, with a control group of 12 patients without significant hemodynamic abnormality, and, second, with a group of 14 patients who had not undergone a Fontan procedure, but whose mean right atrial pressure exceeded 8 mmHg. Follow-up catheterization had been performed in all 30 patients submitted to the Fontan-type operation. Prothrombin time, and factor XIII, were significantly lower in those who had undergone the Fontan procedure than in the other groups. Those submitted to the Fontan operation also had lower levels of protein C than controls, and their levels of plasminogen were lower than the patients with high right atrial pressure. Both aspartate aminotransferase and alanine aminotransferase were higher in those undergoing the Fontan procedure than in the other groups, while gamma-glutamyltranspeptidase in these patients was higher than in the control group. Mean right atrial pressure was highest in those undergoing the Fontan procedure, while cardiac index was lowest. Prothrombin time was correlated to some extent with aspartate aminotransferase, mean right atrial pressure, and cardiac index. Protein C correlated with both aspartate aminotransferase and mean right atrial pressure, while factor XIII correlated with alanine aminotransferase, mean right atrial pressure, and cardiac index. Aspartate aminotransferase, alanine aminotransferase, and gamma-glutamyltranspeptidase, parameters of hepatic function, correlated significantly with mean right atrial pressure. In those who had undergone the Fontan procedure, decreased synthesis of pro-and anti-coagulant factors is a risk factor for both thrombosis and bleeding. Abnormal hemodynamics, in the absence of a right sided pumping chamber, may predispose to subclinical hepatic dysfunction, leading to selective disturbances of protein synthesis.

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

“…Coagulation factor abnormalities were observed in patients after bidirectional cavopulmonary anastomosis with presumably low splanchnic venous pressure, 12 23 but also in patients with single ventricle physiology before the bidirectional cavopulmonary anastomosis. 24 No relation between haemodynamic variables and coagulation factors was confirmed in studies of patients after the Fontan procedure, 1 5 12 except in a study by Tomita and colleagues 8 in which protein C and prothrombin time correlated with right atrial pressure. 8 Recently a higher superior vena cava pressure was found to be a strong predictor of an increased factor VIII concentration in 13 patients followed up after the Fontan operation, 13 but factor VIII is produced by liver sinusoidal endothelium and the kidney rather than by the hepatocytes.…”

Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease

“…83 Several studies have described intrinsic and acquired alterations in thrombophilia factors such that single-ventricle patients may be predisposed to both bleeding and thrombosis. 140,141,146,[268][269][270] These alterations may be related to inflammation, ventricular dysfunction, lower cardiac output, higher venous pressures, nonpulsatile systemic venous flow, chamber dilatation, subclinical hepatic dysfunction, and lower systemic oxygenation. The role of these alterations in clinical decision making is unknown in terms of their contribution to risk and requirement for screening and management.…”

Older Age at Completion of Fontan Procedure Is Associated with Improved Percentage of Predicted Maximum Oxygen Uptake

“…Several other investigators have shown that younger age at Fontan is associated with improved exercise performance. 1,22 Yet again, a significant portion of the study (28) 71.9 ± 21.8 Extracardiac (27) 82.1 ± 16.8…”

“…25 There is evidence that, after Fontan completion, hepatic dysfunction ensues. Several investigators have shown that hepatic synthetic dysfunction, 26 coagulopathy, 27 and cirrhosis 28,29 are timedependent phenomena after the Fontan operation.…”