Abstract 15662: Type IV Collagen 7S And Hyaluronic Acid Predict The Early Stage Of Liver Cirrhosis In Patients After Fontan Procedure
Introduction: The aim of this study was to identify useful biochemical fibrosis markers for the early detection of developing liver fibrosis or liver cirrhosis in patients after Fontan operation.
Methods: 66 consecutive patients who had undergone extra cardiac Fontan procedure (Fontan group) between 1994 and 2009 were included. The mean age was 9.2±5.8 years and mean elapsed time since the initial Fontan operation was 5.9±2.8 years. 20 patients of post-operative tetralogy of Fallot (TOF group) with moderate to severe pulmonary and/or tricuspid regurgitation, and 16 patients with chronic hepatitis (hepatitis group) served as control groups. We quantitated biochemical fibrosis markers including hyaluronic acid(HA), retinol binding protein (RBP), procollagen III peptide(PIIIP), and type IV collagen 7S(IVC7S) in each group. In patients in the Fontan and TOF groups, we assessed hemodynamic parameters during cardiac catheterization, including mean pulmonary artery pressure (mPAP). Abdominal CT scan and abdominal ultrasonography were performed as well.
Results: Biochemical fibrosis markers were different between the Fontan and TOF groups. The serum levels of IVC7S and HA were significantly higher in the Fontan group than those in the TOF group and the hepatitis group (IVC7S: 7.87±2.02 ng/ml, HA: 36.2±17.6 ng/ml in Fontan group vs. 5.77±1.85, 12.4±6.48 in TOF group vs. 4.79±2.55, 28.3±10.7 in hepatitis group, respectively; P<0.01). Moreover the serum levels of HA and IVC7S showed a positive correlation with mPAP in the Fontan group (r>0.50, P<0.01). Of note, 5 patients with HA>50 ng/ml and IVC7S>6.0 ng/ml in the Fontan group had significantly higher mPAP levels compared to the remainder of the Fontan group (P<0.01). In those patients, CT images suggested the presence of liver fibrosis. Whereas serum levels of HA and IVC7S did not correlate with the post-operative period after Fontan procedure.
Conclusion: We conclude that the combination of blood levels of IVC7S and HA is the most predictable parameter for early detection of liver fibrosis and liver cirrhosis in patients with Fontan circulation. After the Fontan procedure, regular evaluation of the hepatic condition is required, especially in patients with high mPAP, using noninvasive biochemical markers.
- © 2011 by American Heart Association, Inc.