Osama Abdur Rehman, MD1, Fnu Aakash, MD1, Vivek Singh, MBBS2, Shajetha Iyathurai, MD1, Lane Sager, MD3, Gautam Maddineni, MD1, Abdus Sameey Anwar, MBBS4, Silpa Choday, MD5, Aasta Kumari, MD6, Sunny Kumar, MD7, Deepak Kumar, MBBS, MD8, Iryna S. Hepburn, MD, FACG1 1Florida State University, Cape Coral, FL; 2FLORIDA STATE UNIVERSITY, Cape Coral, FL; 3Florida State University, Fort Myers, FL; 4College of Medical Sciences, Bharatpur, Dhanbad, Jharkhand, India; 5Creighton University School of Medicine, Phoenix, AZ; 6North Central Bronx Hospital, New York, NY; 7Wright Center for Graduate Medical Education, Scranton, PA; 8Northwell Health, Port Jefferson, NY Introduction: The Fontan procedure, a palliative surgical intervention for single-ventricle congenital heart defects, redirects systemic venous return directly to the pulmonary arteries. While it improves survival in patients with complex congenital heart disease, it predisposes patients to Fontan-associated liver disease (FALD), a spectrum of hepatic abnormalities ranging from congestion and fibrosis to cirrhosis and hepatocellular carcinoma (HCC).
Case Description/
Methods: A 23-year-old female with congenital tricuspid atresia and hypoplastic right ventricle, status post-Fontan procedure at age 3, presented with fever, chills, abdominal pain, and dysuria. She had been recently treated for a urinary tract infection. Laboratory studies revealed elevated liver enzymes (ALP 163 U/L, AST 79 U/L, ALT 84 U/L), with a normal white blood cell count and stable hemodynamics. Imaging revealed hepatosplenomegaly, nodular liver contour, ascites, and elevated liver stiffness (13.3 kPa on elastography), suggestive of advanced chronic liver disease. MRCP showed no biliary obstruction or discrete lesions. Workup for other causes of cirrhosis including viral and autoimmune hepatitis was negative except for anti-smooth muscle antibody positivity. At this time, a diagnosis of congestive hepatopathy due to elevated right heart pressures was favoured. Patient was referred to tertiary care center for right heart cath and further evaluation for liver transplant. Discussion: FALD is driven by chronic hepatic congestion due to elevated central venous pressure in Fontan physiology, leading to sinusoidal dilation, fibrosis, and ultimately cirrhosis. This condition is being increasingly recognized as a cause of liver cirrhosis especially as the burden of Fontan procedure continues to go up. The progression is often subclinical, with preserved liver function until late stages. In this case, liver disease was incidentally discovered during workup for unrelated symptoms. This underscores the need for heightened clinical vigilance and routine hepatic monitoring in Fontan patients, even when asymptomatic. The absence of specific serologic markers and often non-distinctive clinical presentation necessitate a high index of suspicion, with imaging and elastography playing key roles in diagnosis. Early recognition is crucial for guiding management, preventing complications, and assessing transplant candidacy when appropriate.
Figure: Figure 1: Fontan procedure involving shunting of the venous return from the superior and inferior vena cava into the pulmonary circulation bypassing the Right ventricle.
Disclosures: Osama Abdur Rehman indicated no relevant financial relationships. Fnu Aakash indicated no relevant financial relationships. Vivek Singh indicated no relevant financial relationships. Shajetha Iyathurai indicated no relevant financial relationships. Lane Sager indicated no relevant financial relationships. Gautam Maddineni indicated no relevant financial relationships. Abdus Sameey Anwar indicated no relevant financial relationships. Silpa Choday indicated no relevant financial relationships. Aasta Kumari indicated no relevant financial relationships. Sunny Kumar indicated no relevant financial relationships. Deepak Kumar indicated no relevant financial relationships. Iryna Hepburn indicated no relevant financial relationships.
Osama Abdur Rehman, MD1, Fnu Aakash, MD1, Vivek Singh, MBBS2, Shajetha Iyathurai, MD1, Lane Sager, MD3, Gautam Maddineni, MD1, Abdus Sameey Anwar, MBBS4, Silpa Choday, MD5, Aasta Kumari, MD6, Sunny Kumar, MD7, Deepak Kumar, MBBS, MD8, Iryna S. Hepburn, MD, FACG1. P6105 - The Hidden Burden of Fontan-Associated Liver Disease: Unmasking a Predictable Pathophysiologic Pattern – A Case Report, ACG 2025 Annual Scientific Meeting Abstracts. Phoenix, AZ: American College of Gastroenterology.
