The Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Market is estimated to be valued at USD 1.41 Bn in 2024 and is expected to reach USD 2.03 Bn by 2031, growing at a compound annual growth rate (CAGR) of 7.11% from 2024 to 2031. The market is primarily driven by the increasing incidence and diagnosis rates of CTEPH disease globally. As the disease remains under-diagnosed, initiatives by governments and healthcare organizations to spread awareness are expected to boost the market during the forecast period.

The Chronic Thromboembolic Pulmonary Hypertension Market is expected to witness positive growth over the next few years. There is no standard treatment available for CTEPH currently. However, the approval and launch of new drugs to treat the disease is anticipated to provide opportunities for growth of the market. In addition, ongoing clinical trials evaluating novel drugs and treatment options for CTEPH will further aid market expansion through 2031.

Market Driver - The Growing Awareness of CTEPH And its Complications Is Boosting Early Diagnosis and Treatment.

The growing knowledge and understanding about Chronic Thromboembolic Pulmonary Hypertension amongst both patients and physicians have played a crucial role in improving early detection of the disease. In the past, CTEPH often went undiagnosed for longer periods which led to worse health outcomes and higher mortality rates. However, with greater public awareness initiatives by patient advocacy groups and medical organizations in recent years, people are now more informed about the signs and symptoms of CTEPH.

Patients who experience persistent symptoms like dyspnea, fatigue, chest pain after removal of Venous Thromboembolism are not ignoring it and promptly consulting their doctors. At the same time, medical education programs for pulmonologists and cardiologists have enhanced their ability to correctly identify CTEPH even in its early stages. This is allowing for timely referral of suspected cases to expert facilities for confirmation through right heart catheterization - the gold standard diagnostic test. Early diagnosis combined with prompt initiation of recommended treatments like pulmonary endarterectomy and pharmacotherapy is proving critical to better management of the disease.

Moreover, many healthcare systems across the developed world have started recognizing CTEPH as a distinct post-thrombotic entity. This official recognition of the disease has helped put it on the radar of more providers and health insurers. It is encouraging further research into CTEPH while also making affordable treatment options accessible to growing number of patients. The growing patient volumes are attracting greater participation of hospitals, doctors and investment from pharmaceutical companies in this therapeutic area. Overall, enhanced awareness of CTEPH as a serious but treatable medical condition if diagnosed early has significantly benefited stakeholders and augmented efforts to study and address this idle disease.

Market Driver - Emergence of New Therapies Fuels the Market Developments.

Over the past decade, significant therapeutic advances have provided fresh hope to individuals affected by Chronic Thromboembolic Pulmonary Hypertension. For those who are not eligible for pulmonary endarterectomy due to anatomical reasons or recurrent disease, highly effective drug therapies and newer minimally-invasive procedures are substantially improving clinical outcomes. The approval of modern pharmacological agents like Riociguat, Selexipag and Macitentan by regulatory authorities was a breakthrough. These innovative oral drugs target multiple pathophysiological pathways of CTEPH like enhancing nitric oxide signaling, reducing cell proliferation and lowering pulmonary arterial pressure.

Likewise, the establishment of percutaneous balloon pulmonary angioplasty for inoperable CTEPH patients has been a game changer. This catheter-based technique uses balloons to literally break up clots inside the lungs through mechanical dilation and restore blood flow. It is proving to be a highly beneficial therapeutic option for a select group of individuals. Ongoing research is further assessing the long-term safety, efficacy and potential of newer alternative treatments such as pulmonary thromboendarterectomy via video-assisted thoracoscopy.

All these medical advances have led to remarkable symptomatic and functional improvements in complex CTEPH cases which earlier had limited treatment recourse. It is lifting survival rates while allowing patients to regain higher quality of life. This revolution in domain of CTEPH care means that now majority of newly diagnosed patients can benefit from some form of intervention rather than just best medical therapy alone. Continued innovation and clinical progress hold strong promise to make cure and effective disease-control a reality for more individuals impacted by CTEPH in the future.

Market Challenge - The Costs Associated with Surgeries Like PTE And Lifelong Drug Therapies May Limit Patient Access.

One of the key challenges facing the Chronic Thromboembolic Pulmonary Hypertension Market is the high costs associated with treatment. CTEPH often requires invasive surgical procedures such as pulmonary endarterectomy (PEA) to remove blood clots and blockages from the lungs. However, not all patients are eligible for or see success with PEA. For those who cannot undergo surgery or who experience persistent PH after PEA, lifelong drug therapies are frequently required instead. While these medications have been shown to improve symptoms and quality of life, they come with lofty price tags. For example, the prostacyclin therapy treprostinil is estimated to cost over USD 100,000 per year. The economic burden of such expensive treatments can make full access difficult for some patients and healthcare systems. Insurers may refuse coverage, and individuals may opt out of therapy due to high out-of-pocket costs. These financial barriers could negatively impact treatment rates and clinical outcomes if left unaddressed. Stakeholders need to explore cost containment strategies like financial assistance programs and value-based payment models to help alleviate the cost pressures surrounding CTEPH management.

Market Opportunity- Emergence of Innovative Therapies to Enhance Quality of Clinical Research.

A bright spot on the horizon for the Chronic Thromboembolic Pulmonary Hypertension Market is the ongoing research into new and improved treatment approaches. One area showing promise is novel drug classes, like the prostacyclin analogue treprostinil. Clinical trials have demonstrated treprostinil's ability to significantly reduce pulmonary arterial pressure and improve functional capacity when administered through subcutaneous injection or infusion. Its favorable side effect profile compared to older therapies like epoprostenol also enhances patient adherence. If future studies continue corroborating treprostinil's safety and efficacy profile, it could become a new standard of care option and help more CTEPH patients achieve meaningful symptomatic control. Beyond treprostinil, pharmaceutical companies and research institutes are exploring modified prostanoids, soluble guanylate cyclase stimulators, and other pipeline candidates. The development of innovative therapies holds potential for offering broader treatment choices that may address unmet needs within select patient subgroups. It could help expand the reach of pharmacologic interventions for CTEPH.

Thromboembolic Pulmonary Hypertension (TEPH) can be treated through a stepped approach with multiple lines of therapy. For newly diagnosed cases, or mild TEPH, prescribers typically start with oral medications such as Endothelin receptor antagonists (ERAs). Ambrisentan (Letairis) and macitentan (Opsumit) are commonly prescribed ERAs at this stage.

As the disease progresses to moderate TEPH, doctors may add a Phosphodiesterase Type-5 inhibitor (PDE-5i) to the ERA. Tadalafil (Adcirca) is a popular PDE-5i prescribed in combination. For patients who do not respond sufficiently, prostanoids may be introduced. Epoprostenol (Flolan) is administered via continuous IV infusion, while treprostinil (Remodulin) can be delivered subcutaneously.

In severe TEPH cases unresponsive to the above regimens, prescribers generally recommend transitioning to prostacyclin analogs like Riociguat (Adempas), a soluble guanylate cyclase stimulator. It provides an oral option with less invasive administration than prostanoids.

The choice of medication is also influenced by factors like a drug's route of administration, side effect profile, insurance coverage and patient preference/adherence. Less invasive options are preferred unless response to therapy is unsatisfactory.

Thromboembolic pulmonary hypertension (TEPH) has four WHO functional classes (FC) based on symptoms and limitation in physical activity. For FC I-II patients with no or mild limitation, anticoagulation therapy with warfarin is the first-line treatment to prevent new clots.

For FC III patients with marked limitation, additional pulmonary vasodilator therapy may be used. Endothelin receptor antagonists (ERAs) like ambrisentan (Letairis) and macitentan (Opsumit) are effective, improving 6-minute walk distance and delaying clinical worsening. Phosphodiesterase type-5 inhibitors (PDE-5Is) like sildenafil (Revatio), tadalafil (Adcirca) can also be used.

FC IV patients have severe symptoms at rest requiring parenteral prostanoids. Epoprostenol (Flolan) given as a continuous IV infusion is the gold standard, improving hemodynamics, functional class and survival. Iloprost (Ventavis) can be administered via nebulization 6-9 times daily. Treprostinil (Remodulin) is given subcutaneously via infusion pump.

For patients who remain severely symptomatic despite combination oral therapy, atrial septostomy may be considered to relieve right heart pressure. In highly selected cases, lung transplantation can be life-saving. Close monitoring of treatment response and prognostic indicators helps determine progression between stages and optimize management for thromboembolic pulmonary hypertension.

The Chronic Thromboembolic Pulmonary Hypertension Market has seen significant growth in recent years due to increased research activities focusing on developing treatments for this life-threatening disease. Leading pharmaceutical companies have adopted collaborative strategies to expand their product pipelines.

For example, in 2021 Bayer collaborated with Merck & Co. to develop and commercialize vericiguat, an experimental drug in late-stage development. This allowed the companies to pool resources and share risks/rewards. The drug has shown promise in reducing morbidity/mortality in patients with CTEPH or pulmonary hypertension. If approved, it will give the partners a strong foothold in the market.

Another strategy seen is acquisitions of smaller companies developing novel treatment options. In 2019, Johnson & Johnson acquired PhaseBio for USD2.1 billion to gain rights to its experimental CTEPH therapy bentracimab. This gave J&J early access to a potential blockbuster drug while avoiding long development timelines. Bentracimab is currently in Phase 3 trials and has the potential to replace invasive therapies if approved.

Companies also focus on life-cycle management of existing drugs through supplemental approvals. For example, based on positive Phase 3 data, Bayer received FDA approval in 2020 for additional populations for its drug Opsumit to treat CTEPH following pulmonary endarterectomy. This expanded the eligible patient pool, boosting sales opportunities for an already approved product.

Such strategies of partnerships, acquisitions and supplemental filings have led to a spike in drug approvals in recent years, translating to higher market revenue. Collaborations allow risks and larger patient populations to be addressed, benefiting patients, while supplemental approvals extract additional value from existing brands.

Insights, By Type of Treatment Surgical Segment Leads the Bandwagon in the Forecast Period.

The surgical treatment segment currently dominates the Chronic Thromboembolic Pulmonary Hypertension Market share with 51.5% in 2024 owing to the inadequacy of pharmacological options for many patients. Pulmonary endarterectomy (PEA) has long been established as the treatment of choice for proximal CTEPH, offering the only potentially curative option. When performed by experienced surgeons at specialized centers, PEA has been shown to significantly improve hemodynamics and functional status in the majority of eligible patients.

Although PEA carries risk of complications, long-term data indicates excellent outcomes for properly selected candidates, with 10-year survival rates exceeding 80% in recent studies. Due to the technical complexity and risk profile, PEA is only recommended for patients with predominantly proximal clots in the main, lobar, or segmental pulmonary arteries that can be safely removed. For those with distal clots that are inoperable via PEA, no alternative surgical therapies currently exist.

The lack of established surgical alternatives for distal CTEPH points to the critical need for pharmacological therapies to treat this patient subset. However, Riociguat   is the only approved drug specifically indicated for CTEPH, and long-term data on efficacy is limited. While Riociguat   offers an important non-surgical option, its modest hemodynamic effect sizes have left many patients still symptomatic on maximally tolerated therapy. Novel drugs targeting the dysfunctional pathways in CTEPH pathology could widen treatment options beyond Riociguat alone.

Ongoing research also explores less invasive interventional therapies like pulmonary angioplasty and thromboendarterectomy using catheter-based devices. While holding promise, these tools have not yet gained widespread adoption due to technical hurdles and lack of definitive outcome data versus PEA and medical management. In summary, the dominance of PEA reflects an unmet need for safer and more effective non-surgical alternatives, especially for distal CTEPH.

Insights, By Route of Administration, Oral Administration Drives Pharmacological Treatment Uptake

Within the pharmacological treatment segment, the dominance of the oral administration route stems from practical advantages over intravenous options. The market is expected to witness 51.8% share growth in 2024. For patients diagnosed with CTEPH, daily oral therapy offers simplicity, stability, and avoidance of invasive administration procedures compared to intermittent IV therapies. This leads to improved adherence and persistence with medication schedules.

The only approved oral therapy Riociguat provides a convenient, outpatient treatment approach administered twice daily with or without food. This dosing flexibility fits well into most patients' daily routines. In contrast, investigational intravenous prostacyclin therapies require complex inpatient initiations and involve frequent hospital visits or home infusions via portable pumps. The inconvenience of IV administration presents challenges to real-world usage that have hindered uptake.

Additionally, oral absorption appears well-suited to the pathology of CTEPH, as it avoids first-pass liver metabolism and enables stable drug levels that impact the pulmonary vasculature over 24-hour periods. This sustained exposure profile may more effectively counter chronic clot remodeling versus intermittent IV boluses. Overall, the attributes of simplicity, flexibility and patient-centeredness associated with oral Riociguat   help explain its dominance in driving pharmacological treatment for CTEPH. New oral options could further expand this convenient administration route.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare but severe condition with significant implications for the healthcare system. Despite its rarity, the disease carries a high mortality rate if left untreated, and it often goes undiagnosed due to symptom overlap with other diseases. The market for CTEPH treatment is poised for substantial growth over the coming decade, driven by the introduction of novel therapies, improved diagnostic techniques, and heightened disease awareness. As of now, pulmonary endarterectomy remains the gold standard for treatment, but not all patients are suitable for surgery. For inoperable cases, medical treatments such as ADEMPAS (Riociguat) provide a lifeline, helping to improve hemodynamics and quality of life. Additionally, emerging therapies, such as Treprostinil by SciPharm Sarl, are showing promise in clinical trials. With the increasing number of available treatment options and the anticipated entry of new drugs into the market, the competitive landscape is expected to evolve, offering more opportunities for patients and stakeholders alike.

The major players operating in the Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Market include Bayer, SciPharm Sarl, Actelion Pharmaceuticals Ltd, Pfizer, United Therapeutics, AOP Pharmaceuticals AG, GSK Plc, Acceleron Pharma, Jansen Pharmaceuticals and Merck & Co. Inc.

• In May 2024, Bayer launched new clinical trials to extend the use of ADEMPAS to additional patient groups, expanding its market dominance.
• In April 2023, SciPharm Sarl announced positive results in the Phase III trials of Treprostinil, demonstrating improved outcomes for inoperable patients.

• By Type of Treatment
  • Surgical Treatment
  • Pharmacological Treatment
  • Intervention
  • Transplantation
• By Route of Administration
  • Oral
  • Intravenous