• Fontan Procedure
• Outcome Assessment, Health Care
• Biomarkers

Single ventricle patients are born into a world of ambiguity, uncertainty and a future that may never come. Surviving the Fontan operation and entering adulthood should be cause for respite, but for many patients, growing older also means coping with new problems and comorbidities. This is counterbalanced with hope and data suggesting our modern Fontan patients are doing better—with 20 and even 30-year survival rates approaching 80%–90%.1 However, focusing solely on survival is no longer an acceptable benchmark for patients or their physicians; instead, we strive for an exceptional quality of life, which means minimising comorbidities. Fontan-associated liver disease (FALD) is one of those conditions difficult to define, yet somewhat intrinsic to the Fontan circulation. This duality of this diagnosis, being both ambiguous and inevitable, is what makes FALD so unnerving for our patients.

Francis Fontan’s publication describing the original operation that bears his name was published in 1971.2 It would take another 10 years before the first report of an autopsy on a Fontan patient described a liver with nutmeg appearance and histological evidence for cirrhosis.3 An additional 24 years would pass before an autopsy series of nine patients would provide us a broader understanding that the long-term sequelae of Fontan physiology have a spectrum of findings resulting in chronic hepatic venous congestion, liver cirrhosis (LC), hepatic adenoma and hepatocellular carcinoma (HCC).4 Eventually, we would add hepatic fibrosis and focal nodular hyperplasia to the clinical picture and group all of these outcomes under the heterogeneous moniker of FALD.

Numerous aspects of Fontan physiology (central systemic venous hypertension, low cardiac output, cyanosis, lymphatic congestion, cardiopulmonary bypass, etc) create a perfect physiological storm …