Pulmonary hypertension (PH) is a progressive and severe disorder in pulmonary hemodynamics. PH can be fatal if not well managed. Fibrosing mediastinitis (FM) is a rare and benign fibroproliferative disease in the mediastinum, which may lead to pulmonary vessel compression and PH. PH caused by FM (PH-FM) is a pathologic condition belonging to group 5 in the World Health Organization PH classification. PH-FM has a poor prognosis because of a lack of effective therapeutic modalities and inappropriate diagnosis. With the development of percutaneous pulmonary vascular interventional therapy, the prognosis of PH-FM has been greatly improved in recent years. This article provides a comprehensive review on the epidemiology, pathophysiologic characteristics, clinical manifestations, diagnostic approaches, and treatment modalities of PH-FM based on data from published reports and our medical center with the goal of facilitating the diagnosis and treatment of this fatal disease.
Keywords: CT, computed tomography; CTEPH, chronic thromboembolic pulmonary hypertension; CTPA, computed tomography pulmonary angiography; CTPV, computed tomography pulmonary venography; ECG, electrocardiogram; FM; FM dyad; FM, fibrosing mediastinitis; FM triad; H-FM, histoplasma capsulatum infection–related fibrosing mediastinitis; Ig, immunoglobulin; MRI, magnetic resonance imaging; PA, pulmonary artery; PAG, pulmonary artery angiography; PET-CT, positron emission tomography/computed tomography; PH, pulmonary hypertension; PH-FM, pulmonary hypertension caused by fibrosing mediastinitis; PV, pulmonary vein; PVG, pulmonary vein angiography; RHC, right heart catheterization; SPECT/CT, single-photon emission computed tomography/computed tomography; SVC, superior vena cava; TB, tuberculosis; TB-FM, Mycobacterium tuberculosis infection–related fibrosing mediastinitis; V/Q, lung ventilation/perfusion; clinical classification; fibrosing mediastinitis; interventional therapy; pulmonary hypertension.
© 2022 The Authors.