I27.840

Clinical Causes: Altered blood flow in the liver caused by the Fontan procedure, which directs venous blood directly to the pulmonary arteries, bypassing the heart. Increased central venous pressure leading to liver congestion. Chronic passive congestion resulting in fibrosis and potential cirrhosis over time. Long-term changes in blood flow dynamics that induce hepatic inflammation and scarring.

Key Symptoms: Many individuals with FALD are asymptomatic in early stages. Abdominal discomfort or fullness, particularly in the upper right area. Swelling in the abdomen (ascites) due to fluid buildup. Fatigue and general weakness. Potential development of spider angiomata or enlarged veins on the skin. In advanced cases, signs of liver failure such as jaundice or easy bruising.

Diagnosis Path: Diagnosis of FALD involves a combination of clinical evaluation and specific tests: - Imaging studies such as ultrasound, MRI, or CT scans to assess liver size, structure, and presence of fibrosis. - Blood tests to evaluate liver function, including liver enzymes, bilirubin levels, and clotting factors. - Elastography techniques to measure liver stiffness, indicating fibrosis extent. - Liver biopsy may be considered in certain cases to confirm fibrosis or cirrhosis, though non-invasive methods are preferred when possible.

Treatment Protocols: Currently, there is no specific therapy to reverse FALD. Management focuses on monitoring and preventing complications: - Regular liver function testing and imaging to track disease progression. - Treating symptoms such as fluid retention with diuretics. - Addressing complications like variceal bleeding or ascites with appropriate medications or procedures. - Optimizing overall heart health and managing other related conditions. - In advanced cases, consideration for liver transplantation may be necessary. - Multidisciplinary care involving cardiologists, hepatologists, and other specialists is essential for comprehensive management.