両方向性Glenn手術後に発症した原発性肺静脈狭窄症の2例

Primary Pulmonary Vein Stenosis Developed after Bidirectional Glenn Procedure；Report of Two Cases

肺静脈（PV）還流異常を伴わない複雑心奇形に対する両方向性Glenn（BDG）手術後に高度の肺静脈狭窄（PVS）が発症した2例を報告する．

We experienced 2 cases of primary pulmonary vein stenosis （PVS），which developed after a bidirectional Glenn procedure was performed for complex heart defects with normal pulmonary venous return. Although the patients successfully underwent primary sutureless repair for left PVS, restenosis of the affected pulmonary veins occurred several months after surgery in both patients. Stent implantation followed by balloon angioplasty was performed for stent stenosis in 1 patient without effect. However, the patient later underwent a successful fenestrated Fontan procedure. Catheter intervention was contraindicated in the 2nd patient due to almost complete obstruction of the left pulmonary veins with upstream hypoplasia. To improve the results of PVS treatment, earlier diagnosis by quantitative lung perfusion scintigraphy, magnetic resonance imaging, and close echocardiographic observation together with earlier, aggressive treatment combining surgery and catheter interventions are recommended.