Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
Pediatric Cardiology and Cardiac Surgery 33(2): 180-186 (2017)

症例報告Case Report

Fontan循環に併発したショック肝Shock Liver in Fontan Circulation

1あかね会土谷総合病院小児科The Department of Pediatrics, Tsuchiya General Hospital ◇ Hiroshima, Japan

2あかね会土谷総合病院心臓血管外科The Department of Cardiovascular Surgery, Tsuchiya General Hospital ◇ Hiroshima, Japan

3済生会横浜市東部病院小児肝臓消化器科The Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohama City Tobu Hospital ◇ Kanagawa, Japan

受付日:2016年10月21日Received: October 21, 2016
受理日:2016年12月19日Accepted: December 19, 2016
発行日:2017年3月1日Published: March 1, 2017


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.

Key words: cholestasis; shock liver; Fontan associated liver disease; hypoxic hepatopathy; hyperbilirubinemia

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