Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by precapillary pulmonary hypertension (PH) on right heart catheterization (RHC) and imaging evidence of chronic thromboembolism and is a long-term complication of pulmonary embolism (PE). Protheragen provides preclinical research services for rare cardiovascular diseases such as CTEPH. Our goal is to expedite drug discovery and development by providing end-to-end solutions that are specifically designed to address the unique challenges of these diseases.
Introduction to Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Chronic thromboembolic pulmonary hypertension (CTEPH) is a chronic and progressive pulmonary vascular disease resulting from chronic obstruction of the major pulmonary arteries by flow-limiting organized thrombi. These longer-standing obstructions become organized and fibrotic, and accompanied by pulmonary vascular remodeling, can result in pulmonary hypertension, right heart failure, and death. The true prevalence and annual incidence of CTEPH are not known, but some reports have estimated the incidence of this disorder at approximately 5 cases per million people per year.
Fig.1 Risk factors for CTEPH. (Inacio Cazeiro, D., et al., 2025)Pathogenesis of Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
CTEPH is considered a complication of thromboembolic events in the pulmonary vasculature. The pathophysiology of CTEPH is complex and involves both deficient fibrinolysis as well as endothelial dysregulation and hypoxic adaptation. Broadly there are two kinds of vascular lesions that lead to increased pulmonary vascular resistance (PVR), namely perennial intravascular occlusion of the pulmonary artery by organized fibrotic clots and secondary microvasculopathy.
Fig.2 Types of vascular lesions in CTEPH. (Inacio Cazeiro, D., et al., 2025)Therapeutics Development for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
| Drug Name | Mechanism of Action | Targets | NCT Number | Research Phase |
|---|---|---|---|---|
| Riociguat | Activating soluble guanylate cyclase to raise cyclic GMP, resulting in vasodilation and decreased pulmonary vascular resistance. | sGC | / | Approved |
| Treprostinil | As an analog of prostacyclin, it induces vasodilation and is an inhibitor of platelet aggregation, thus it decreases pulmonary and systemic vascular resistance. | PTGIR | NCT01416636 | Phase III |
| Macitentan | Through its antagonistic effects at the endothelin receptors, resulting in vasodilatation and decreasing pulmonary vascular resistance. | ETA, ETB | NCT05179876 | Phase III |
Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a therapy plan recommendation. For guidance on therapy options, please visit a regular hospital.
Our Services
A complete range of services are provided by our company to cover all important steps of drug development. The services and expertise we provide in diagnostics, therapeutics, and the development of disease models enforce an excellent basis for innovation in therapy. Furthermore, we also provide a wide range of preclinical services including pharmacokinetic analysis and drug safety tests to ensure drugs are efficacious and safe enough to move on to later-stage trials.
Animal Model Development for CTEPH
Animal models are useful to investigate the progression, and hemodynamic changes of CTEPH and its response to pharmacologic therapy. With an experienced, talented research group and advanced facilities, our company offers customized animal model generation services to help you with your CTEPH studies.
The surgical induction model of VSD was performed by making accurate septal incisions in the animal heart.
- Transcatheter puncture method
- Other models
In the chemical induction model, teratogenic agents are employed to perturb normal cardiac septation.
- Dimethadione (DMO)-induced model
- Retinoic acid-induced model
- Other models
Partner with us and access leading-edge facilities and a highly skilled team, passionate about developing life-changing therapies for individuals with rare cardiovascular conditions. Collectively, Protheragen can work through the challenges of developing new drugs and contribute to positive change in worldwide health. Please don't hesitate to get in touch to discuss how we can help with your R&D requirements.
Reference
- Inacio Cazeiro, Daniel et al. "Chronic thromboembolic pulmonary hypertension: A comprehensive review of pathogenesis, diagnosis, and treatment strategies." Revista portuguesa de cardiologia: orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology: an official journal of the Portuguese Society of Cardiology 44.2 (2025): 121-137.
For research use only, not for clinical use.