Fibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis

Fibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis AbstractIntroduction: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. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Medicine Wolters Kluwer Health

Fibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis

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Publisher
Wolters Kluwer Health
Copyright
Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc.
ISSN
0025-7974
eISSN
1536-5964
D.O.I.
10.1097/MD.0000000000009694
Publisher site
See Article on Publisher Site

Abstract

AbstractIntroduction: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.

Journal

MedicineWolters Kluwer Health

Published: Jan 1, 2018

References

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