日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

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.