Fontan-associated lymphatic dysfunction

This code represents a specific type of lymphatic dysfunction that develops as a complication following a Fontan procedure. It involves impaired lymphatic flow, often leading to protein-losing enteropathy, chylous effusions, or other systemic lymphatic issues in patients who have undergone this palliative surgery for complex congenital heart disease.

Use this code when documentation explicitly states "Fontan-associated lymphatic dysfunction" or describes symptoms and findings consistent with lymphatic complications directly attributable to the Fontan circulation. This typically includes patients presenting with chylothorax, chylous ascites, or protein-losing enteropathy post-Fontan.

• Always ensure the documentation clearly links the lymphatic dysfunction to the Fontan procedure.
• Code any specific manifestations of the lymphatic dysfunction (e.g., protein-losing enteropathy, chylothorax) as secondary diagnoses.
• Do not confuse with other forms of lymphatic dysfunction not related to a Fontan procedure.