Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare form of pulmonary hypertension. It occurs when blood clots do not fully dissolve in the lungs. These clots become organized fibrotic tissue. Over time, this narrows or blocks the pulmonary arteries. The result is increased pressure in the lungs and strain on the heart.
CTEPH is a life-threatening condition. However, recent years have brought new hope. Treatments have evolved. This article explains the newest options available for managing CTEPH in 2025. The goal is to present simple, clear, and evidence-based information.
Traditional Treatments: A Brief Review
The gold standard for treating CTEPH has been pulmonary endarterectomy (PEA). This surgical procedure removes chronic clots from the pulmonary arteries. It can be curative. But not all patients qualify for surgery. Some clots are too small or located in distal vessels.
For patients who are not surgical candidates, riociguat, a soluble guanylate cyclase stimulator, has been the only approved medical therapy. In some cases, balloon pulmonary angioplasty (BPA) is also used. It helps to open narrowed vessels.
Recent Advances in CTEPH Treatment
1. Improved Balloon Pulmonary Angioplasty (BPA)
BPA is gaining momentum as a treatment option for inoperable CTEPH. It is a minimally invasive procedure. A catheter with a balloon is inserted into the pulmonary artery. The balloon inflates to widen narrowed vessels.
In recent years, BPA has become safer and more effective. Studies from Japan and Europe show improved outcomes with staged procedures. Each session targets different lung segments. This reduces complications like reperfusion pulmonary edema.
New imaging techniques now guide BPA with precision. Optical coherence tomography (OCT) and intravascular ultrasound (IVUS) help identify the structure of the blockages. These technologies ensure safe and accurate dilation of vessels.
2. Advanced Drug Therapy
Drug treatment is no longer limited to riociguat. Clinical trials are exploring additional medications. These include:
Macitentan: An endothelin receptor antagonist. Early results show improvement in pulmonary hemodynamics and exercise capacity when combined with BPA or used alone.
An oral prostacyclin receptor agonist. It may improve symptoms in non-operable patients and those with residual hypertension after surgery.
Sotatercept: A novel investigational agent. It targets the TGF-beta signaling pathway. It is being studied in pulmonary arterial hypertension (PAH), and trials in CTEPH are expected.
Combination therapy using two or more classes of drugs may enhance outcomes. Trials are assessing these approaches. Early data is promising, especially for patients with persistent symptoms after PEA or BPA.
3. Personalized Treatment Plans
Precision medicine is improving care in CTEPH. Specialists now consider factors like:
- Location and size of clots
- Heart function
- Presence of co-existing diseases
- Genetic markers and inflammation
Machine learning algorithms help predict treatment outcomes. This supports better patient selection for surgery, BPA, or medical therapy. It also minimizes risks and maximizes benefits.
4. Hybrid Treatment Approaches
Some patients benefit from a hybrid approach. This combines surgery, BPA, and drug therapy. For example, a patient may undergo PEA and later receive BPA for residual lesions. In some cases, BPA is used first to prepare high-risk patients for surgery.
These combined strategies show better long-term outcomes. They reduce pulmonary vascular resistance and improve right heart function. More centers are now using hybrid protocols for complex CTEPH cases.
Emerging Research and Clinical Trials
Several clinical trials are underway in 2025. These include:
MERIT-CTEPH Study: Investigates macitentan in inoperable CTEPH.
SEQUENT-CTEPH Trial: Evaluates the benefit of selexipag after PEA.
TRANSFORM Trial: Assesses BPA plus riociguat versus riociguat alone.
These trials are expected to publish results within the next year. They will provide stronger evidence for expanding drug options and treatment combinations.
Use of Biomarkers
Researchers are identifying blood biomarkers. These help detect early CTEPH or measure response to treatment. Examples include:
- NT-proBNP: Indicates heart strain and treatment effectiveness
- D-dimer: May predict ongoing clot activity
- Galectin-3 and growth differentiation factor-15: Markers of fibrosis and inflammation in the lung
These markers are still under investigation. But they may soon guide therapy selection and monitoring.
Technological Innovations in Diagnosis and Follow-Up
Better tools are now available to assess CTEPH progression. These include:
Dual-energy CT (DECT): Provides detailed perfusion maps of the lungs.
Cardiac MRI: Offers clear visualization of right heart function.
Ventilation/perfusion (V/Q) SPECT scans: Allow more accurate detection of small clots.
Telemedicine also supports frequent follow-up. Patients can monitor symptoms and oxygen levels remotely. This ensures early intervention when needed.
Pulmonary Rehabilitation as a Supportive Therapy
Exercise and education improve outcomes. Pulmonary rehabilitation programs are now included in treatment plans. They help patients regain stamina and manage symptoms.
Rehabilitation may include:
- Supervised exercise training
- Breathing techniques
- Nutritional counseling
- Psychological support
Studies show improved quality of life, six-minute walk distance (6MWD), and fewer hospitalizations.
Patient-Centered Care and Shared Decision-Making
CTEPH is complex. The best outcomes happen when patients are fully involved in treatment decisions. Shared decision-making is now standard in expert centers. Patients receive detailed explanations of their condition and options. They work with a team that includes cardiologists, pulmonologists, and surgeons.
Clear communication helps reduce anxiety. It also improves adherence to therapy. Tools such as treatment algorithms and patient apps now support informed decisions.
Multidisciplinary CTEPH Centers: The Standard of Excellence
Experts recommend care at accredited CTEPH centers. These centers offer:
- Advanced diagnostics
- Skilled surgical teams
- Access to clinical trials
- Multidisciplinary case discussions
Data shows that patients treated at these centers have better survival rates and fewer complications. Networks like the European Society of Cardiology (ESC) and Pulmonary Hypertension Association (PHA) support center accreditation and quality improvement.
Challenges and Future Directions
While treatment has advanced, several challenges remain:
- Late diagnosis is still common. Many patients go undetected for years.
- Access to expert care is limited in some regions.
- Drug development is slow due to the rarity of CTEPH.
Future research may address these gaps. Focus areas include:
- AI-based screening tools
- Gene therapy for vascular remodeling
- Long-acting prostacyclin analogs
Conclusion
CTEPH once carried a grim outlook. But in 2025, patients have more choices than ever. From safer BPA to new medications, treatments are improving both survival and quality of life.
The key to success is early diagnosis, personalized care, and access to expert centers. Continued research will bring even better options in the near future.
Patients, families, and healthcare providers should stay informed. Together, they can make the best decisions for each individual. The fight against CTEPH is stronger—and more hopeful—than ever before.
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