Fontan-associated liver disease [FALD]

This code represents a specific form of chronic liver disease that develops in patients who have undergone a Fontan procedure, a palliative surgery for complex congenital heart defects. FALD is characterized by progressive hepatic fibrosis and dysfunction resulting from chronic venous congestion and altered hemodynamics post-Fontan.

Use this code for patients with a documented diagnosis of liver disease directly attributed to their history of a Fontan procedure. Documentation should clearly link the liver pathology (e.g., cirrhosis, fibrosis, portal hypertension) to the Fontan circulation, often confirmed by imaging, biopsy, or clinical assessment by a cardiologist or hepatologist.

• Ensure documentation explicitly states "Fontan-associated liver disease" or clearly links liver pathology to the Fontan procedure.
• Do not use this code for other forms of liver disease in Fontan patients that are not directly related to the Fontan circulation.
• Consider sequencing this code after the primary congenital heart defect (e.g., Q20-Q26) if the heart condition is still the primary reason for the encounter.