Hepatic Coma Secondary to Fontan Conduit Thrombosis

Area of Science:

• Cardiology
• Hepatology
• Critical Care Medicine

Background:

• The Fontan procedure is a palliative surgery for single ventricle congenital heart defects, significantly improving long-term survival.
• Long-term complications include Fontan-associated liver disease (FALD) due to chronic passive congestion, potentially leading to cirrhosis, ascites, gastrointestinal bleeding, and encephalopathy.

Purpose of the Study:

• To report a case of a patient with a history of Fontan surgery who experienced severe neurological deterioration due to Fontan conduit thrombosis during COVID-19 pneumonia.
• To highlight the successful management of Fontan circulation thrombosis and its impact on neurological recovery.

Main Methods:

• A 43-year-old male with a history of Fontan surgery presented with COVID-19 pneumonia and rapid clinical decline.
• Computed tomography (CT) imaging revealed thrombosis of the Fontan conduit.
• The patient underwent successful recanalization of the Fontan circulation.

Main Results:

• The patient experienced rapid clinical and neurological deterioration, progressing to coma and shock.
• Following recanalization of the Fontan conduit, the patient showed progressive improvement from coma.
• This case underscores the potential for acute complications like thrombosis in Fontan circulation, especially during systemic illness.

Conclusions:

• Fontan-associated liver disease is a significant long-term complication of the Fontan procedure, stemming from altered hemodynamics.
• Prompt recognition and intervention for complications such as conduit thrombosis are crucial for patient outcomes.
• Regular long-term surveillance is essential for patients with Fontan circulation to prevent and manage systemic complications effectively.