Screening modalities for the diagnosis of Fontan-associated liver disease: evidence from the past for future development.

ABSTRACT

Approximately 70,000 patients who have undergone the Fontan operation worldwide survive into adulthood, however the majority of these patients are faced with long-term post-operative complications due to specific hemodynamic changes. Fontan-associated liver disease (FALD) is a challenging complication characterized by various spectra. Of these, liver congestion and liver fibrosis potentially lead to cirrhosis and liver nodules. The most serious condition associated with the development of liver nodules is hepatocellular carcinoma. Various non-invasive modalities including blood tests, ultrasound scans of the upper abdomen, ultrasound elastography of the liver, computed tomography scans, magnetic resonance imaging and magnetic resonance elastography of the liver have been used as alternatives to liver biopsies for FALD assessment in post-Fontan patients. To date, a detailed understanding of the pathophysiology and natural history of these patients, and the most appropriate modality for the effective investigation of this condition is incomplete. In this comprehensive review, reports regarding the currently available screening modalities used in the detection of FALD are summarized and discussed. The findings of this review, including identification of any current knowledge gaps, can pave the way for the development of effective future strategies in the surveillance and ultimately the treatment of post-Fontan patients.