Background : Several studies have shown that Fontan circulation may lead to liver congestion and possible structural liver alteration. The aim of this study is to analyze the relationships between biochemical fibrosis markers and hemodynamic parameters in the long term after the Fontan operation.
Methods : The study enrolled 51 patients who underwent total cavopulmonary connection between March 1994 and July 2010. We analyzed the relationships between the 5 liver fibrosis markers (hyaluronic acid, retinol-binding protein, procollagen type III peptide, type IV collagen 7S, type IV collagen) and the 6 hemodynamic parameters (pulmonary artery pressure, pulmonary artery index, pulmonary vascular resistance, ejection fraction, atrioventricular valve regurgitation, cardiac index).
Results : Hyaluronic acid and type IV collagen 7S positively correlated with pulmonary artery pressure. Hyaluronic acid negatively correlated with ejection fraction, and type IV collagen 7S positively correlated with atrioventricular valve regurgitation in patients followed up for more than 8 years after Fontan completion. Pulmonary artery pressure was significantly higher in patients in whom type IV collagen 7S was elevated. Hyaluronic acid correlated with pulmonary vascular resistance (p=0.0035) and ejection fraction (p=0.014), as well as type IV collagen 7S with pulmonary artery pressure (p=0.0001) by multiple regression analysis.
Conclusion : Hyaluronic acid and type IV collagen 7S reflected the degree of hepatic congestion, and cardiac function, in the long term after the Fontan operation.

A male patient with single ventricle pulmonary stenosis, and persistent left superior vena cava underwent original Blalock-Taussig shunt (BTS) at 2 years of age and suffered from infective endocarditis at 38 years of age. A systemic work-up detected dural arteriovenous fistula and aneurysmal dilatation of the original BTS. Cardiac catheterization and cardiac magnetic resonance imaging revealed an appropriate pulmonary vasculature for bidirectional Glenn anastomosis and sufficient antegrade pulmonary blood flow through the pulmonary valve. Bilateral bidirectional Glenn anastomosis and resection of the aneurysm of the BTS-associated aneurysm were successfully performed.