Fontan-associated liver disease: pathophysiology, investigations, predictors of severity and management

Cardiac hepatopathy is the liver injury resulting from congestion and ischaemia associated with acute or chronic heart failure. The improved longevity of adults with operated congenital heart disease who develop heart failure as an increasingly late event makes this form of liver injury increasingly clinically relevant. Patients with congenital heart disease with a single ventricle anomaly, who require creation of a Fontan circulation, are particularly vulnerable as they have elevated venous filling pressures with chronic liver congestion. Progression to liver fibrosis and eventually cirrhosis may occur, with its associated risks of liver failure and hepatocellular carcinoma. This risk likely increases over the patient's lifetime, related to the duration post-surgical repair and reflects the chronicity of congestion. Liver biopsy is rarely performed due to a higher risk of complications in the setting of elevated venous pressures, and the frequent use of anticoagulation. Non-invasive methods of liver assessment are poorly validated and different factors require consideration compared to other chronic liver diseases. This review discusses the current understanding of cardiac hepatopathy in congenital heart disease patients with a Fontan circulation. This entity has recently been called Fontan Associated Liver Disease in the literature, with the term useful in recognising that the pathophysiology is incompletely understood, and that long-standing venous pressure elevation and hypoxaemia are presumed to play an additional significant role in the pathogenesis of the liver injury.