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 32(1): 43-47 (2016)


Fontan術後に発症した低蛋白血症にspironolactoneの追加投与が奏功した一例Effectiveness of the Additional Administration of Spironolactone on Hypoproteinemia after the Fontan Procedure

1佐賀大学医学部小児科Department of Pediatrics, Faculty of Medicine, Saga University ◇ Saga, Japan

2福岡市立こども病院循環器科Department of Cardiology, Fukuoka Children’s Hospital ◇ Fukuoka, Japan

受付日:2015年5月10日Received: May 10, 2015
受理日:2015年11月13日Accepted: November 13, 2015
発行日:2016年1月1日Published: January 1, 2016

Fontan手術後に発症した低蛋白血症にspironolactoneの追加投与が奏功した症例を経験したので報告する.症例は3歳男児.大動脈縮窄を合併した左室型単心室に対してFontan手術(total cavo-pulmonary connection, TCPC)を施行された.術後6カ月後に,明らかな誘因なく血清総蛋白4.3 g/dL,アルブミン2.5 g/dL,IgG 182 mg/dLと低下を認めた.尿蛋白陰性であり,患者の既往歴からPLEよる低蛋白血症を疑った.患者は既にtorasemide,tadaraphilを内服していたところでの発症であったため,免疫グロブリン製剤の補充を行うと共にtolvaptanの投与を開始したが,3カ月の観察中に十分な効果は得られなかった.発症3カ月後にspironolactone 20 mg(1.5 mg/kg/day)を追加したところ,翌日より眼瞼浮腫は消失し,2週間後には血液検査値も正常化し現在に至っている.本例では,これまでに有効性が示されている薬剤(利尿剤・phosphodiesterase type 5阻害剤・抗利尿ホルモン受容体拮抗薬)が既に導入されている状況での発症であったが,spironolactone追加投与が極めて有効であった.Fontan術後の低蛋白血症の治療においてspironolactoneが有用な選択肢の一つである.

We report a patient who showed dramatic improvement in his condition after additional administration of spironolactone for hypoproteinemia following the Fontan procedure. The patient was a 3-year-old boy with a univentricular heart and coarctation of the aorta. After the Fontan procedure, he was administered torasemide and tadalafil to maintain his circulatory status. He attended our hospital because of vomiting and intermittent abdominal pain 6 months after the procedure, and hypoproteinemia (serum protein, 4.3 g/dL, serum albumin, 2.5 g/dL and IgG, 182 mg/dL) was observed. Because proteinuria was not detected, we speculated that the patient was in the initial phase of protein-losing enteropathy (PLE) based on his previous medical history. He was initially administered immunoglobulin and tolvaptan, an antidiuretic hormone receptor blocker, and was followed up by periodical supplementation of immunoglobulin. However, 3 months after the treatment, no improvement was observed in his condition and his serum protein, albumin, and immunoglobulin levels did not recovered to normal levels. We therefore added spironolactone to his therapy, which resulted in a dramatic improvement in his hypoproteinemia without any adverse effects. The patient’s condition has remained stable since then. This case shows that the administration of spironolactone is a therapeutic choice for PLE in patients who undergo the Fontan procedure.

Key words: Fontan; hypoproteinemia; spironolactone

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