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 39(1): 9-15 (2023)

症例報告Case Report

心房内血流転換術後の蛋白漏出性胃腸症2例の経験と文献レビューProtein-Losing Enteropathy after Atrial Switch Operation: Two Case Reports and a Literature Review

1東京大学医学部附属病院小児科Department of Pediatrics, The University of Tokyo Hospital ◇ Tokyo, Japan

2東京大学医学部附属病院心臓外科Department of Cardiothoracic Surgery, The University of Tokyo Hospital ◇ Tokyo, Japan

受付日:2022年10月30日Received: October 30, 2022
受理日:2022年12月23日Accepted: December 23, 2022
発行日:2023年2月1日Published: February 1, 2023

蛋白漏出性胃腸症(protein-losing enteropathy: PLE)は,先天性心疾患領域でしばしば経験される合併症だが,Fontan例に典型的であるのに比して,非Fontan例での報告は少ない.今回,修正大血管転位症の心房内血流転換術後遠隔期に発症したPLEの2例に対し,それぞれ手術による下大静脈baffle狭窄解除と,経皮的血管形成術による上大静脈baffle狭窄解除を施行し,PLEの寛解を得た.これまでに心房内血流転換術後のPLEは自験例を含めて12例の報告があり,うち11例(92%)にbaffle狭窄ないし閉塞が認められた.狭窄部位は上大静脈のみが5例,下大静脈のみが2例,その両者が4例であった.狭窄を認めた11例全てで外科手術またはカテーテル治療による狭窄解除が施行され,11例中8例(73%)で有効性が報告された.baffle狭窄は心房内血流転換術後に発生するPLEの主要な原因であり,上下大静脈いずれの狭窄例でもPLEの発症が起こり得る.また外科手術やカテーテル治療による狭窄解除の治療効果は高く,積極的に検討すべきである.

Protein-losing enteropathy (PLE) is a commonly experienced complication in the field of congenital heart disease. However, there are few reports on the PLE of non-Fontan cases compared with Fontan cases. We report two patients who developed PLE after an atrial switch operation for congenitally corrected transposition of the great arteries. Each of them received surgical reconstruction of the obstructed inferior caval pathway and balloon dilatation of the superior caval pathway, respectively, and remissions of PLE were obtained for both cases. Based on previous literature, we reviewed 12 PLE cases after atrial switch operation, including our cases, and 11 (92%) of them had stenosis or complete occlusion of the intra-atrial baffles. The sites of stenosis were superior vena cava in 5 cases, inferior vena cava in 2 cases, and both in 4 cases. All 11 patients with baffle obstruction were treated with surgical or transcatheter treatments and enhancement in PLE was observed in 8 of 11 patients (73%). Baffle obstruction is a major cause of PLE after atrial switch operation, and either superior vena cava or inferior vena cava obstruction can trigger PLE. Surgical or transcatheter intervention for stenotic regions is highly efficient and should be the first-line treatment option.

Key words: protein-losing enteropathy; atrial switch operation; congenitally corrected transposition of the great arteries; baffle obstruction

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