Related Experiment Video
Updated: Dec 18, 2025

Catheter-based Endovascular Angioplasty for Fibrosing Mediastinitis-associated Pulmonary Vein Stenosis
Published on: August 26, 2025
Does combination therapy work in chronic thromboembolic pulmonary hypertension?
M C J van Thor1, R J Snijder2, J C Kelder3
1Dept of Cardiology, St. Antonius Hospital, Nieuwegein, the Netherlands.
Dual combination therapy for chronic thromboembolic pulmonary hypertension (CTEPH) showed similar 5-year survival rates compared to monotherapy. This finding is notable given combination therapy patients had more severe baseline conditions.
Area of Science:
- Cardiology
- Pulmonology
- Pharmacology
Background:
- Chronic thromboembolic pulmonary hypertension (CTEPH) treatment options are evolving.
- Experience with combination therapy for CTEPH remains limited.
- Understanding long-term survival outcomes is crucial for treatment optimization.
Purpose of the Study:
- To present the first 5-year survival results for dual combination therapy versus monotherapy in CTEPH.
- To compare survival outcomes between different medical therapy strategies in CTEPH patients.
- To evaluate the efficacy of combination therapy in a real-world CTEPH population.
Main Methods:
- Retrospective analysis of consecutive non-operated CTEPH patients receiving PH-specific medical therapy (2002-2019).
- Comparison of survival between monotherapy and dual combination therapy (upfront or sequential) up to 5 years.
- Inclusion of 183 patients (83 monotherapy, 100 combination therapy) with detailed baseline and follow-up data.
Main Results:
- Patients on combination therapy presented with more severe baseline characteristics (higher NT-proBNP, mean pulmonary artery pressure, pulmonary vascular resistance; lower cardiac index).
- Estimated 5-year survival was 79% for monotherapy and 70% for combination therapy.
- No significant difference in survival was observed between the monotherapy and dual combination therapy groups (p=0.22).
Conclusions:
- Dual combination therapy for CTEPH demonstrated comparable 5-year survival to monotherapy.
- These findings suggest combination therapy is a viable option, even in patients with more severe baseline disease.
- Further research may elucidate optimal combination strategies and patient selection for CTEPH treatment.
Related Concept Videos
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care
Venous Thrombosis III: Interprofessional Care
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...