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

Abstract

Protein-losing enteropathy (PLE) is a commonly experienced complication in the eld 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 a er 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 a er atrial switch operation, including our cases, and 11 (92%) of them had stenosis or complete occlusion of the intra-atrial ba es. e 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 ba e obstruction were treated with surgical or transcatheter treatments and enhancement in PLE was observed in 8 of 11 patients (73%). Ba e obstruction is a major cause of PLE a er 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 e cient and should be the rst-line treatment option.