Fontan-associated liver disease (FALD) may thus develop, particularly in patients with long-term hepatic venous congestion and hypoxia. Systemic venous hypertension developing secondary to establishment of the Fontan circulation decreases the venous return, in turn reducing the cardiac output, which increases cardiac pressure and dilates the sinusoid. Postoperatively, the superior vena cava drains into the distal right pulmonary artery (the “Fontan circulation”). The Fontan procedure is a palliative operation for patients with single-ventricle congenital heart disease.
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Multivariate Cox regression analyses indicated that the complication of polysplenia and a higher MELD-XI score (HR: 1.148, both p < 0.01) were independent risk factors for FALD-HCC. Kaplan–Meier curve analysis indicated that patients with T-BIL levels ≥ 2.2 mg/dL, HA levels ≥ 55.5 ng/mL, and MELD-XI scores ≥ 18.7 were at high risk of HCC, a generally poor prognosis, and both polysplenia and esophageal varices. Within approximately 20 years of the Fontan procedure, 10 patients died (survival rate, 96.9%). At the time of HCC development, the hyaluronic acid (HA) level (p = 0.04) and the fibrosis-4 index (p = 0.02) were significantly higher in FALD-HCC patients than in non-HCC patients the total bilirubin (T-BIL) level (p = 0.07) and the model for end-stage liver disease score (p = 0.06) tended to be higher in FALD-HCC patients. Compared with non-HCC patients, FALD-HCC patients exhibited higher incidences of polysplenia and esophageal varices.
![meld score 12 meld score 12](https://image.slidesharecdn.com/hepbandcscreeningmanagementsimonstowns-120621145635-phpapp02/95/hep-b-and-c-screening-management-simons-towns-31-728.jpg)
The incidence of HCC was 4.9% in FALD patients within 20 years after the Fontan procedure.