日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

There is a lack of information on the frequency, mechanism, and pathology of Fontan-associated liver disease (FALD). Liver congestion and ischemia associated with high central venous pressure are considered to be major causes of hepatopathy. We describe a male patient with shock liver in Fontan circulation, protein-losing gastroenteropathy (PLE), and a portosystemic shunt (PSS), all accompanied by conjugated hyperbilirubinemia after hospitalization for PLE. Despite intensive care, the patient died of liver failure. Autopsy findings revealed cholestasis in bile capillaries, dilated central veins and sinusoids, and necrotic centrilobular hepatocytes. These histological features suggested that shock liver caused hepatic failure. We speculated that PSS decreased blood flow in the portal vein, inadequate hepatic circulation became progressively pronounced in a low-output state associated with heart failure, and this was followed by shock liver. The primary etiology of FALD is described as hepatic fibrosis, but shock liver should also be considered in the potential etiology of FALD. More information from similar patients needs to be accumulated for developing effective treatment strategies.