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

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
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 33(2): 180-186 (2017)
doi:10.9794/jspccs.33.180

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
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Fontan関連肝疾患(FALD)の病態は不明な点が多く,高い中心静脈圧に起因するうっ血肝等により肝組織障害が発現すると考えられている.症例は13歳男児.Ebstein奇形,心室中隔欠損のため2歳時にFontan手術施行.4歳時蛋白漏出性胃腸症(PLE)発症.8歳時門脈体循環シャント(PSS)指摘.11歳時PLE再発.13歳8ヶ月時PLE再々発.経過中直接ビリルビン上昇が徐々に増悪し,13歳10ヶ月で永眠.肝組織で毛細胆管内の胆汁うっ滞,中心静脈・類洞拡大,肝細胞融解性壊死等を認め,循環不全に伴うショック肝と判断した.肝線維化は軽度であり類洞などへの血栓形成は認めなかった.PSSに伴う門脈血流減少に加え,心不全進行による中心静脈圧上昇があり,繰り返し肝細胞への虚血を生じた結果ショック肝に至ったと推察した.本症例もFALDの一病態と考えられる.今後の症例の積み重ねが大切である.

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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This page was last modified on 2017-04-12T20:17:59.254+09:00


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