Pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitis

Fibrosing mediastinitis (FM) is a heterogeneous disease characterized by sclerosing fibrosis of mediastinal structures. Pulmonary hypertension (PH) may complicate the course of the disease and can contribute significantly to the morbidity of FM. Due to the rarity and complexity of the disease, evide...

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Main Authors: Daniel Van Kalsbeek, Abedel Rahman Anani, Karim El-Kersh
Format: Article
Language:English
Published: Elsevier 2024-01-01
Series:Respiratory Medicine Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2213007124000297
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author Daniel Van Kalsbeek
Abedel Rahman Anani
Karim El-Kersh
author_facet Daniel Van Kalsbeek
Abedel Rahman Anani
Karim El-Kersh
author_sort Daniel Van Kalsbeek
collection DOAJ
description Fibrosing mediastinitis (FM) is a heterogeneous disease characterized by sclerosing fibrosis of mediastinal structures. Pulmonary hypertension (PH) may complicate the course of the disease and can contribute significantly to the morbidity of FM. Due to the rarity and complexity of the disease, evidence-based guidelines are not currently available, and the optimal treatment approach is unknown. Management approaches should be individualized, and current techniques are often unsatisfactory. Here, we present two cases of PH complicating FM that were managed using pulmonary artery vasodilator therapy with excellent hemodynamic response.
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spelling doaj.art-fc68d1ea651f4e60a868319acf2821922024-03-23T06:24:10ZengElsevierRespiratory Medicine Case Reports2213-00712024-01-0149102006Pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitisDaniel Van Kalsbeek0Abedel Rahman Anani1Karim El-Kersh2Corresponding author. Division of Pulmonary, Critical Care, and Sleep Medicine, University of Nebraska Medical Center, 982465 Nebraska Medical Center, Omaha, NE, 68198-2465, USA.; University of Nebraska Medical Center, Omaha, NE, USAUniversity of Nebraska Medical Center, Omaha, NE, USAUniversity of Nebraska Medical Center, Omaha, NE, USAFibrosing mediastinitis (FM) is a heterogeneous disease characterized by sclerosing fibrosis of mediastinal structures. Pulmonary hypertension (PH) may complicate the course of the disease and can contribute significantly to the morbidity of FM. Due to the rarity and complexity of the disease, evidence-based guidelines are not currently available, and the optimal treatment approach is unknown. Management approaches should be individualized, and current techniques are often unsatisfactory. Here, we present two cases of PH complicating FM that were managed using pulmonary artery vasodilator therapy with excellent hemodynamic response.http://www.sciencedirect.com/science/article/pii/S2213007124000297Fibrosing mediastinitisMediastinal fibrosisSclerosing mediastinitisPulmonary hypertensionPulmonary vasodilators
spellingShingle Daniel Van Kalsbeek
Abedel Rahman Anani
Karim El-Kersh
Pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitis
Respiratory Medicine Case Reports
Fibrosing mediastinitis
Mediastinal fibrosis
Sclerosing mediastinitis
Pulmonary hypertension
Pulmonary vasodilators
title Pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitis
title_full Pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitis
title_fullStr Pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitis
title_full_unstemmed Pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitis
title_short Pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitis
title_sort pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitis
topic Fibrosing mediastinitis
Mediastinal fibrosis
Sclerosing mediastinitis
Pulmonary hypertension
Pulmonary vasodilators
url http://www.sciencedirect.com/science/article/pii/S2213007124000297
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AT abedelrahmananani pulmonaryarteryvasodilatorsfortreatmentofpulmonaryhypertensioncomplicatingfibrosingmediastinitis
AT karimelkersh pulmonaryarteryvasodilatorsfortreatmentofpulmonaryhypertensioncomplicatingfibrosingmediastinitis