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
Pediatric Cardiology and Cardiac Surgery 33(3): 202-210 (2017)
doi:10.9794/jspccs.33.202

原著Original

Fontan手術後に発症する蛋白漏出性胃腸症の予後に関する検討Treatment Outcomes for Patients with Fontan Associated Protein-losing Enteropathy

栗田 佳彦1，馬場 健児1，近藤 麻衣子1，栄徳 隆裕1，塚原 宏一1，佐野 俊二2，笠原 真悟2，岩崎 達雄3，佐藤 修平4，大月 審一1Yoshihiko Kurita1, Kenji Baba1, Maiko Kondo1, Takahiro Eitoku1, Hirokazu Tsukahara1, Shunji Sano2, Shingo Kasahara2, Tatsuo Iwasaki3, Shuhei Sato4, Shinichi Ohtsuki1

1 岡山大学大学院医歯薬学総合研究科　小児科Department of Pediatrics Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences ◇ Okayama, Japan

2 岡山大学大学院医歯薬学総合研究科　心臓血管外科Department of Cardiovascular Surgery Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences ◇ Okayama, Japan

3 岡山大学大学院医歯薬学総合研究科　麻酔・蘇生科Department of Anesthesiology and Resuscitology Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences ◇ Okayama, Japan

4 岡山大学大学院医歯薬学総合研究科　放射線科Department of Radiology Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences ◇ Okayama, Japan

受付日：2015年8月7日Received: August 7, 2015
受理日：2017年4月17日Accepted: April 17, 2017
発行日：2017年5月1日Published: May 1, 2017
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背景：複雑先天性心疾患におけるFontan手術後に発症する蛋白漏出性胃腸症は発症すると難治・治療困難な病態であり生命予後を規定する．本研究の目的は当院でのPLE症例の予後と治療方針の検討である．

方法：1991～2014年に岡山大学病院でFontan手術後PLEの診断・治療を行った23例を対象にし，転帰の調査と死亡症例の傾向について検討を行った．

結果：Fontan手術施行401例中の23例（5.7％）に発症．フォローアップ期間は0.8～13年（中央値4.7年）．生存率は5年68％，10年54％．死亡原因は敗血症（2例），PLE増悪による多臓器不全（2例），心不全（1例），頭蓋内出血（1例）．死亡症例はPLE診断年齢が高くFontan手術から診断までの期間が長期であった．血行動態評価では治療前後の心係数（CI）と主心室駆出率（vEF），治療後の中心静脈圧（mCVP）に有意差を認めた．治療後CI＜2.8 L/min/mm, vEF＜50％，mCVP≧15 mmHgの症例は死亡率が高かった．

結論：PLEに対する治療は一定の有用性を認めたが，予後は満足できるものではない．血行動態的な治療の目標はCI，vEFの改善とCVPを低下させることと思われる．

Background: Protein-losing enteropathy (PLE) following the Fontan operation has been an extremely difficult complication to treat successfully. This study aimed to assess the prognosis and to establish appropriate treatment strategies for PLE.

Methods: From 1991 to 2014, 23 patients with PLE following the Fontan operation were identified from clinical database at Okayama University Hospital. Data were collected retrospectively.

Results: A total of 401 patients underwent Fontan operation at our institution during the study period. In this cohort, the 5.7％ incidence of PLE was relative to the total number of Fontan operations. Following PLE diagnosis, patients had a 68％ and 54％ survival rate at 5 and 10 years, respectively. The causes of death included sepsis, multiple organ failure, heart failure, and intracranial bleeding. In the treatment of PLE, 23 (100％) patients were treated with medication, 10 (43％) patients underwent catheter intervention and 15 (65％) patients underwent surgery. Patients who died were older at the time of PLE diagnosis and were associated with a longer interval between the Fontan operation and PLE diagnosis. Decreased survival was observed in patients with the following conditions post-PLE treatment: a cardiac index (CI)＜2.8 L/min/mm, decreased ventricular ejection fraction (vEF)＜50％, and high central venous pressure (meanCVP)≧15 mmHg.

Conclusion: Our current treatment for PLE was only partially successful. Strategies for reducing CVP and improving both CI and vEF may be effective. Further studies are necessary to determine better treatment strategies for the future.

Key words: protein-losing enteropathy; Fontan operation; central venous pressure; ventricular ejection fraction; cardiac index

© 2017 特定非営利活動法人日本小児循環器学会© 2017 Japanese Society of Pediatric Cardiology and Cardiac Surgery

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