Treatment of Fibrosing Mediastinitis in Pulmonary Hypertension
When fibrosing mediastinitis causes pulmonary hypertension, the vascular and hemodynamic complexities of this patient population require careful consideration of which management strategies are appropriate.
This protocol addresses fibrosing mediastinitis presenting with pulmonary hypertension as the primary complication. In this population, therapies that may be used in other pulmonary hypertension contexts require caution given the distinct vascular and hemodynamic profile these patients carry.
The approach centres on non-surgical interventional palliation targeting the pulmonary vasculature. The full protocol specifies which procedures are indicated and the circumstances in which each applies — available via the link below.
Clinical targets include improvement in WHO functional class, six-minute walk test performance, and overall clinical symptoms.
References
DOI: 10.1007/s13665-025-00382-3
- In patients with pulmonary hypertension, it has been reported that pulmonary vasodilators have been used as off-label therapies but would caution the use given the vascular and hemodynamic complexities in this patient population.
- Non-surgical interventions include either pulmonary artery and/or endobronchial angioplasty and stenting.
- Less commonly performed are pulmonary vein balloon angioplasty; the largest study examining pulmonary vein stenosis/angioplasty involved 31 patients, with noted improvement in WHO functional class, six-minute walk test, and clinical symptoms.