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 32(4): 344-349 (2016)
doi:10.9794/jspccs.32.344

症例報告Case Report

β遮断薬およびrenin–angiotensin–aldosterone系拮抗薬によってsteroid療法から離脱し得たFontan手術後タンパク漏出性腸症の2自験例Protein-losing Enteropathy after a Fontan Operation Resolved by Beta-blockers and Renin–Angiotensin–Aldosterone System Blockers Following Steroid Therapy: Two Consecutive Cases

1榊原記念病院小児循環器科Department of Pediatric Cardiology, Sakakibara Heart Institute ◇ Tokyo, Japan

2榊原記念病院小児循環器外科Department of Pediatric Cardiovascular Surgery, Sakakibara Heart Institute ◇ Tokyo, Japan

受付日:2015年2月13日Received: February 13, 2015
受理日:2016年6月7日Accepted: June 7, 2016
発行日:2016年7月1日Published: July 1, 2016
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Fontan循環における肺循環へ血液を拍出する心室を欠く特殊性による中心静脈圧(CVP)の上昇,心拍出量低下はタンパク漏出性腸症(PLE)の発症要因と考えられている.我々は開窓部閉塞を契機にPLEを発症し,prednisolone(PSL)による寛解後,直ちに血行動態を評価し血管拡張療法を強化したところ,寛解を維持したままPSLを中止し得た2症例を経験したので報告する.2症例ともPLE寛解直後のカテーテル検査で心係数(CI)と右室駆出率(RVEF)の低下,体血管抵抗(SVR)と右室の拡張能を表すと考えられる右室拡張末期圧(RVEDP)/CIの上昇が認められた.右室拡張障害の改善を主目的としてβ遮断薬(BB),アンギオテンシン変換酵素阻害薬(ACEi),アンギオテンシンII受容体拮抗薬(ARB),spironolactone(SPL)の併用療法を強化したところ,PSL中止時にはSVR, RVEDP/CIは低下しCI, RVEFは改善していた.PLE発症には心拍出量低下への反応であるSVR上昇と,それによる体循環心室機能低下が関与するものと推測された.また,交感神経系抑制薬とrenin–angiotensin–aldosterone(RAA)系抑制薬の併用療法は,Fontan手術後に発症するPLE治療の一選択肢と考えられた.

Due to the lack of a ventricle to pump blood to the pulmonic circuit, Fontan circulation induces high central venous pressure and low cardiac output. This may lead to the onset of protein-losing enteropathy (PLE). We report on two patients with PLE after Fontan type operation who were treated successfully with Beta-blockers and Renin–Angiotensin–Aldosterone System Blockers following steroids. Both patients had right isomerism, a functionally single ventricle, and post-operative fenestrated total cavopulmonary connection. They developed PLE after fenestration closure. We initially used prednisolone (PSL) to improve PLE and performed cardiac catheterization when protein loss ceased. Based on hemodynamic data, we administered four agents and increased their doses: a beta blocker, an angiotensin-converting enzyme inhibitor, an angiotensin II receptor blocker, and spironolactone. The PSL dose was tapered gradually over 7–8 months. On remission by PSL, both cases showed reduced cardiac index (CI) and right ventricular ejection function (RVEF), and increased systemic vascular resistance (SVR) and ratio of the right ventricular end-diastolic pressure to CI (RVEDP/CI). When PSL was discontinued, both cases showed reduced SVR and RVEDP/CI and improved CI and RVEF. Concomitant administration of a sympathetic blocking agent and renin–angiotensin–aldosterone system inhibitors may be a treatment option for PLE after a Fontan operation. Increased SVR may occur as a reaction to low cardiac output, and it may cause dysfunction of the systemic ventricle and further low cardiac output. These sequences may be a cause of PLE.

Key words: Fontan operation; protein-losing enteropathy; beta blocker; angiotensin-converting enzyme inhibitor; angiotensin II receptor blocker

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This page was last modified on 2016-07-26T15:32:53.185+09:00


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