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Updated: Jun 13, 2026

Catheter-based Endovascular Angioplasty for Fibrosing Mediastinitis-associated Pulmonary Vein Stenosis
Published on: August 26, 2025
Standard PAH therapy improves long term survival in CTEPH patients
Hans-Juergen Seyfarth1, Michael Halank, Heinrike Wilkens
1Department of Respiratory Medicine, University of Leipzig, Liebigstrasse 20, 04103, Leipzig, Germany. hans-juergen.seyfarth@uniklinik-leipzig.de
Background:
Chronic thromboembolic pulmonary hypertension (CTEPH), subsequent to pulmonary embolism is a relatively frequent cause of pulmonary hypertension. Similar to patients with pulmonary arterial hypertension (PAH), CTEPH carries a poor prognosis. There is no hard evidence for any other therapy except pulmonary endarterectomy and none for those patients that are not eligible for this procedure.
Patients And Methods:
Fifty patients with confirmed, inoperable CTEPH receiving specific vasodilative therapy (prostanoids, endothelin receptor antagonists, PDE 5-inhibitors or combination) were included in this retrospective study (mean age 55 years, range 16-76 years; 36 female, 14 male). Kaplan-Meier plots of these patients were compared with Kaplan-Meier plots of two historical CTEPH patient groups without any specific vasodilative treatment by log rank tests.
Results:
CTEPH patients treated with specific vasodilative compounds as used for therapy of PAH were followed up for 52 +/- 30 months and had a significantly improved survival compared with patients treated without PAH type vasodilators (p < or = 0.0002).
Conclusion:
Our data may generate the hypothesis that specific vasodilative treatment improves outcome in patients with inoperable CTEPH.
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