Liver lesions in Fontan-associated liver disease: current challenges and future directions

Hepatic lesions develop in nearly half of the Fontan population, yet clinicians lack tools to distinguish benign lesions from malignancy, including hepatocellular carcinoma (HCC). Standard Liver Imaging Reporting and Data System (LI-RADS) criteria are not applicable to Fontan-associated liver disease (FALD). To address this critical diagnostic gap, risk-stratified surveillance approaches have been proposed.

We review the spectrum of hepatic lesions in FALD, emphasizing the diagnostic challenge in distinguishing benign from malignant nodules. Noninvasive fibrosis assessment, including transient elastography, is discussed for risk stratification, though liver biopsy remains the only reliable tool for characterizing suspicious lesions. HCC in FALD demonstrates distinct features and carries poor prognosis primarily due to presentation with advanced disease. Despite treatment modalities, outcomes remain suboptimal, with most therapies limited by cardiac comorbidities, abnormal hemodynamics, and advanced FALD.

The optimal surveillance and diagnostic strategy for hepatic lesions in patients with FALD remains undefined. Multimodal assessment, multidisciplinary care, and a low threshold for biopsy are essential. Liver biopsy remains the only reliable method for characterizing suspicious lesions and staging fibrosis severity in the context of FALD. FALD-specific diagnostic criteria and evidence-based surveillance protocols are urgently needed to guide early detection and improve outcomes.