Pratiksha Moliya, MD1, Natapat Chaisidhivej, MD1, Shravya R. Ginnaram, MD1, Jainil Shah, MD2, Fedja A. Rochling, MBBCh, MBA3 1University of Nebraska Medical Center, Omaha, NE; 2Jefferson Regional Hospital, Little Rock, AR; 3UNMC, Omaha, NE Introduction: Over 900 Fontan procedures are performed annually in the US, offering long-term survival for these patients. As this population ages, FALD are increasingly recognized. The risk of HCC adds complexity to long-term care. We present a unique FALD case with a liver lesion that posed a significant diagnostic dilemma.
Case Description/
Methods: A 35-year-old male with hypoplastic left heart syndrome requiring a Fontan procedure at age 3 presented for heart transplant due to Fontan failure. He had a 6-year history of cirrhosis attributed to FALD. He reported a recent ascites with no history of hepatic encephalopathy, variceal bleeding. His MELD-3.0 score was 25. Labs showed: WBC 3.1, Hb 15.5, Plt 117, INR 1.3, Na 129, Cr 2.02, ALP 101, AST 19, ALT 8, GGT 22, TBili 1.0 and AFP 3.7. Abdominal ultrasound revealed cirrhosis, signs of portal hypertension, and a possible exophytic lesion in the left hepatic lobe. MRI identified a 1.5 cm lesion with arterial phase enhancement, washout, and pseudocapsule. Following multidisciplinary discussion, the lesion was classified as HCC without histopathological confirmation. The patient was listed for combined heart-liver transplantation and underwent TACE as bridging therapy. Unfortunately, He developed right ventricular failure and vasogenic shock after the transplant and passed away. Discussion: The Fontan procedure is a palliative surgery for single-ventricle congenital heart disease. FALD stems from chronic venous congestion, ischemia, and elevated central venous pressure, leading to sinusoidal dilation and cirrhosis. Nearly all patients has histologic fibrosis by 10–15 years post-Fontan. FALD poses unique challenges, Elastography is unreliable due to congestion, biopsy carries risks in anticoagulated patients & METAVIR isn’t validated for congestive hepatopathy. HCC surveillance in FALD is complex. The incidence of HCC is 0.18–1.3%, it can occur even without cirrhosis, with a mean diagnosis age of 30 and median tumor size of ~4 cm. 1-year survival of ~50%. Our patient had lesion meeting OPTN-5A. Yet OPTN/LI-RADS are not validated in FALD, as regenerative nodules can mimic HCC. Despite normal AFP (3.7), the lesion was labeled HCC in high-risk FALD context. He underwent TACE and was listed for transplant. This case underscores the diagnostic uncertainty in FALD, the limitations of current imaging criteria, and the need for validated surveillance protocols.
Disclosures: Pratiksha Moliya indicated no relevant financial relationships. Natapat Chaisidhivej indicated no relevant financial relationships. Shravya Ginnaram indicated no relevant financial relationships. Jainil Shah indicated no relevant financial relationships. Fedja Rochling indicated no relevant financial relationships.
Pratiksha Moliya, MD1, Natapat Chaisidhivej, MD1, Shravya R. Ginnaram, MD1, Jainil Shah, MD2, Fedja A. Rochling, MBBCh, MBA3. P3914 - Liver Lesions in Fontan-Associated Liver Disease: Benign Finding or Red Flag?, ACG 2025 Annual Scientific Meeting Abstracts. Phoenix, AZ: American College of Gastroenterology.
