Open AccessFibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis
Author(s) -
Yidan Li,
Xiangli Meng,
Yidan Wang,
Yuanhua Yang,
Xiuzhang Lu
Publication year - 2018
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000009694
Subject(s) - medicine , pulmonary vein stenosis , complication , pulmonary hypertension , stenosis , mediastinitis , cardiology , radiology , surgery , pulmonary vein , atrial fibrillation
Fibrosingmediastinitis (FM) is caused by a proliferation of fibrous tissue in the mediastinum encasing the mediastinal viscera that results in compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) is a severe complication of FM caused by extrinsic compression of pulmonary blood vessels. Case Presentation: Here, we present the case of a 47-year-old man who presented with a 10-year history of progressive hemoptysis and a 2-year history of shortness of breath, in whom a diagnosis of FM was made. Occlusion of the superior pulmonary veins was noted, with stenosis of the inferior pulmonary veins, leading to PH. Because the patient was a poor candidate for interventional catheterization, the preferred treatment for FM, his PH has been managed with diuretics, and he remains stable. Conclusions: FM is a serious, potentially life-threatening condition that is best managed in specialized centers.