Fontan-Associated Liver Disease (FALD)

Physiologic changes in patients with a Fontan procedure:

(1) high systemic venous pressure

(2) low cardiac output

(3) low arterial oxygen saturation

Features of FALD:

(1) liver fibrosis, portal and centrilobular

(2) vascular congestion extending into the hepatic sinusoids

(3) no or low inflammatory activity

(4) portal hypertension

Possible mechanism: sinusoidal thrombosis associated with the release of fibronectin from the activated hepatic stellate cell

Complications may include:

(1) cirrhosis

(2) hepatocellular carcinoma