PUBLISHER: DelveInsight | PRODUCT CODE: 1179462
PUBLISHER: DelveInsight | PRODUCT CODE: 1179462
DelveInsight's 'Pulmonary Arterial Hypertension - Market Insights, Epidemiology, and Market Forecast-2032' report delivers an in-depth understanding of the Pulmonary Arterial Hypertension, historical and forecasted epidemiology as well as the Pulmonary Arterial Hypertension market trends in the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan.
The Pulmonary Arterial Hypertension market report provides current treatment practices, emerging drugs, market share of individual therapies, and the current and forecasted 7MM Pulmonary Arterial Hypertension market size from 2019 to 2032. The Report also covers current Pulmonary Arterial Hypertension treatment practice, SWOT analysis, reimbursement, market access, and unmet medical needs to curate the best of the opportunities and assesses the underlying potential of the market.
Study Period: 2019-2032
Pulmonary Arterial Hypertension Overview
Pulmonary Hypertension (PH) is defined as mean pulmonary artery pressure greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise as measured by right heart catheterization. It is often characterized by a progressive and sustained increase in pulmonary vascular resistance (PVR) that eventually leads to right ventricular failure. It can be a life-threatening condition if untreated. The World Health Organization (WHO) has divided PH into five groups based on similarities in pathophysiology, clinical presentation, and therapeutic options. Of these PAH belongs to WHO Group 1.
PAH is a rare, progressive disorder characterized by hypertension in the pulmonary arteries for no apparent reason. It was defined by the 6th World Symposium on Pulmonary Hypertension (WSPH) as a resting mean pulmonary artery pressure (mPAP) of 20 mm Hg or greater, a normal end-expiratory pulmonary artery wedge pressure (PAWP) less than or equal to 15 mm Hg, and a PVR of greater than or equal to 3 Wood units. PAH is further classified by the WHO into the following types: idiopathic PAH (IPAH), heritable PAH (HPAH), drug and toxin-induced PAH, and PAH associated with other diseases and disorders. The associated conditions include cirrhosis, HIV, congenital heart disease, and connective tissue diseases like scleroderma among others.
The initial symptom includes severe shortness of breath following exertion, including excessive fatigue, weakness, chest pain, dizzy spells, and fainting episodes. Hemoptysis, hypotension, and hoarseness due to compression of a nerve in the chest by an enlarged pulmonary artery are also observed. In advanced stages, syncope, tachypnea, cyanosis, and hypertrophy resulting in diminished functioning of the right portion of the heart are observed, often leading to right heart failure.
The most common form of PAH is idiopathic, characterized by elevated vascular resistance and pulmonary vasculature blood vessel constriction. It is considered that molecular and genetic factors, which result in the hypertrophy of smooth muscle, endothelial cells, and adventitia, are the root causes of restricted flow via pulmonary arteries, as seen in PAH.
The exact cause of PAH is unknown but in HPAH, an autosomal dominant genetic condition, mutations in the BMPR2 gene are the most common cause. PAH leads to disruption in one or more of three key pathways: nitric oxide (NO), prostacyclin (PGI2), thromboxane A2 (TXA2), and endothelin-1 (ET-1) leading to vasoconstriction, increased smooth muscle cell formation, inflammation, and thrombosis. These imbalances contribute to changes in the blood vessels, which further impact the progression of this disease.
Pulmonary Arterial Hypertension Diagnosis
Symptoms usually go unnoticed and may be present for up to 2 or 3 years before a diagnosis is made, often leading to misdiagnosis or a delay in early-stage diagnosis. While the global guidelines for PAH promote cardiac catheterization as the gold standard for diagnosis, transthoracic echocardiography is the most prevalent method of diagnosis.
Electrocardiogram (ECG), chest radiography, pulmonary function tests, and blood tests are also done to determine PAH. With improvement in disease understanding, biomarker-based assay and genetic testing are also being done to ascertain PAH. The Six-Minute Walk Test (6MWT) is also a widely used test to determine a patient's activity tolerance and estimated 1-year mortality.
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Pulmonary Arterial Hypertension Treatment
Treatment of patients with PAH is based on a patient's vasoreactivity test and functional capacity as determined by the WHO functional capacity classification. The goal is to increase vasodilation of the pulmonary vasculature and decrease PAP to increase CO.
The systematic step-by-step approach to managing PAH starts with general supportive care and progresses to focused pharmacological therapies. Individuals with PAH who respond to a vasoreactivity test performed during their RHC are treated with calcium channel blockers (CCB) such as nifedipine, diltiazem, and amlodipine. Targeted monotherapy or combination treatments that combat the pathophysiology of the illness are the treatment choice for those who either do not meet the criteria for vasoreactivity testing or exhibit an insufficient response.
The current mainstay therapies that include PDE5 inhibitors, sGC stimulators, ERAs, prostacyclin analog, and agonists, for the treatment of PAH target the three major pathways, NO/cGMP, endothelin, and prostacyclin.
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The disease epidemiology covered in the report provides historical as well as forecasted epidemiology segmented by Total Prevalent Cases of Pulmonary Arterial Hypertension, Total Diagnosed Cases of Pulmonary Arterial Hypertension, Age-specific Cases of Pulmonary Arterial Hypertension, Class-specific Cases of Pulmonary Arterial Hypertension, Gender-specific Cases of Pulmonary Arterial Hypertension, and Subtype-specific Cases of Pulmonary Arterial Hypertension scenario of Pulmonary Arterial Hypertension in the 7MM covering the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan from 2019 to 2032.
Key Findings
The epidemiology segment also provides the Pulmonary Arterial Hypertension epidemiology data and findings across the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan.
The drug chapter segment of the Pulmonary Arterial Hypertension report encloses a detailed analysis of Pulmonary Arterial Hypertension marketed drugs, mid-phase, and late-stage pipeline drugs. It also helps to understand the Pulmonary Arterial Hypertension clinical trial details, expressive pharmacological action, agreements and collaborations, approval, and patent details of each included drug, and the latest news and press releases.
Pulmonary Arterial Hypertension Marketed Drugs
Various therapies are approved for the treatment of PAH across FCs like UPTRAVI (selexipag), REMODULIN (treprostinil) (IV, SC), TYVASO (treprostinil, inhaled), ADEMPAS (riociguat), OPSUMIT (macitentan), ORENITRAM (treprostinil), and others.
UPTRAVI (selexipag): Johnson & Johnson/Nippon Shinyaku
UPTRAVI is a selective prostacyclin receptor agonist structurally distinct from prostacyclin. It is hydrolyzed by carboxylesterase 1 to yield an active metabolite more potent than selexipag. The prostacyclin receptor is one of the five major types of prostanoid receptors (IP, EP, DP, TP, and FP). In contrast to other prostanoid receptors, selexipag and its active metabolite are both selective for the prostacyclin receptor. Activation of the prostacyclin receptor induces vasodilation in pulmonary circulation and inhibits the proliferation of vascular smooth muscle cells, the key factor in PAH pathogenesis. Thus by dilating blood vessels in PAH individuals, selexipag decreases the pressure in the vessels supplying blood to the lungs.
Products detail in the report…
REMODULIN/ TREPROST (treprostinil): United Therapeutics/ Mochida Pharmaceuticals
REMODULIN is a prostacyclin mimetic formulated for SC or IV administration to treat PAH (WHO Group 1) and to diminish associated symptoms with exercise. It is included in patients with NYHA FC II-IV symptoms and etiologies of IPAH, HPAH, PAH associated with CTD, and PAH associated with congenital systemic-to-pulmonary shunts. It is also recommended for patients with PAH requiring transition from epoprostenol, as it diminishes the rate of clinical deterioration. United Therapeutics is currently conducting Phase I studies to develop a new prodrug called RemoPro (treprostinil) to enable SC delivery of treprostinil analog therapy without any site pain that is associated with SC REMODULIN (treprostinil).
Products detail in the report…
TYVASO (treprostinil): United Therapeutics
TYVASO is a drug-device combination therapy comprised of a dry powder formulation of treprostinil and a small, portable dry powder inhaler. It is a single-dose plastic cartridge filled with 1% treprostinil, a prostacyclin mimetic for administration by oral inhalation using the TYVASO DPI inhaler. TYVASO DPI incorporates the dry powder formulation technology and Dreamboat inhalation device technology used in MannKind's Afrezza (insulin human) inhalation powder product, which the FDA approved in 2014. DPI device represents a convenient option for the administration of treprostinil therapy. TYVASO was initially approved as an inhalation solution for oral inhalation using the TYVASO Inhalation System, which consisted of Optineb-ir, an ultrasonic, pulsed-delivery device.
Products detail in the report…
List of products to be continued in the report…
Pulmonary Arterial Hypertension Emerging Drugs
The potential drugs expected to launch in the forecast period include Sotatercept (MK-7962) (Merck), ralinepag (United Therapeutics), RT234 (vardenafil inhalation powder) (Respira Therapeutics), seralutinib (GB002) (Gossamer Bio), YUTREPIA (inhaled dry powder formulation of treprostinil) (Liquidia Technologies), TNX-201 (Tenax Therapeutics), and others.
Sotatercept (MK-7962): Merck
Sotatercept is an investigational activin receptor type IIA-Fc (ActRIIA-Fc) fusion protein designed to be a selective ligand trap for members of the TGF-beta superfamily to re-balance antiproliferative (BMPRII/Smad1/5/8-mediated) and pro-proliferative (ActRIIA/Smad2/3-mediated) signaling, which is a key molecular driver of PAH. Recently, Merck announced positive results of the Phase III STELLAR trial evaluating the safety and efficacy of Sotatercept as an add-on to stable background therapy for treating PAH (WHO Group 1). Additional Phase III trials evaluating Sotatercept in patients with PAH include ZENITH and HYPERION. Merck is also conducting Phase II trials to assess the safety and efficacy of Sotatercept in children with PAH.
Products detail in the report…
Ralinepag: United Therapeutics
Ralinepag is a novel, oral, selective, and potent prostacyclin receptor agonist being developed by United Therapeutics for treating PAH. In vitro studies indicate that Ralinepag has high binding affinity and selectivity at the human prostacyclin (IP) receptor. United Therapeutics is currently enrolling two Phase III studies of Ralinepag, ADVANCE OUTCOMES, and ADVANCE CAPACITY. Both of these studies are global, multicenter, placebo-controlled trials of patients on approved oral background PAH therapies.
Products detail in the report…
RT234 (vardenafil inhalation powder): Respira Therapeutics
RT234 is a first-in-class inhaled therapy intended for as-needed (PRN) use to improve exercise tolerance and provide acute relief from breathlessness and fatigue, the most commonly reported symptoms in PAH patients (Group 1 in the WHO's classification of PH indications). RT234 is a drug-device combination of a capsule and the novel axial oscillating sphere (AOS) dry powder inhaler. Vardenafil is a dry powder; the inhaler is specifically designed to deliver this powdered drug to the lungs. Respira intends to pursue additional indications for RT234 in other WHO PH patient groups and is currently conducting Phase IIb trials (VIPAH-PRN 2B) to treat PAH.
Products detailed in the report…
Seralutinib (GB002) (Gossamer Bio)
Seralutinib is an inhaled, small molecule, selective platelet-derived growth factor, or PDGF, receptor kinase inhibitor in development for the treatment of PAH. Seralutinib targets PDGFRa/B, CSF1R, and c-KIT and upregulates BMPR2 protein expression; these pathways play important roles in PAH. It significantly reduces the right ventricular systolic pressure and mean pulmonary artery pressure. Recently, Gossamer Bio announced the topline results from the Phase II TORREY study, favoring seralutinib with enhanced effects in patients with more severe disease at baseline. Gossamer Bio had commenced investment in operational activities to enable the commencement of a registrational Phase III clinical program by the third quarter of 2023.
Products detailed in the report…
List of products to be continued in the report…
The current mainstay therapies to treat PAH act to dilate the pulmonary vasculature, decreasing pulmonary vascular resistance and secondarily improving right ventricular function, thereby improving functional capacity. The overall treatment goal is to improve survival, quality of life, exercise capacity, symptom burden, and clinical worsening, with risk stratification tools increasingly used to guide therapy and improve each of these elements. Current treatment recommendations weigh the use of multiple factors, including WHO FC, exercise ability, lab indices, and hemodynamic and echocardiographic variables to establish the overall severity of the disease and guide the intensity of therapy. Initial therapy choices and subsequent therapy changes are determined to achieve a low-risk category that helps improve overall survival and functional status in PAH.
The four drug classes (PDE5 inhibitors, sGC stimulators, ERAs, prostacyclin analog, and agonists) widely used for treating target three major signaling pathways, prostacyclin, endothelin, and nitric oxide, are responsible for PAH. The marketed therapies approved for the treatment of PAH across various WHO Functional Classes (FC) include Johnson & Johnson/Nippon Shinyaku's UPTRAVI (selexipag) and OPSUMIT (macitentan), United Therapeutics' REMODULIN (treprostinil) (IV, SC), TYVASO (treprostinil, Inhaled), and ORENITRAM (treprostinil), Bayer/Merck's ADEMPAS (riociguat). Other therapies which were approved but have their generics in the market include REVATIO (sildenafil), ADCIRCA (tadalafil), LETAIRIS/ VOLIBRIS (ambrisentan), VENTAVIS (iloprost), TRACLEER (Bosentan), BERAPROST (TRK-100), and VELETRI (epoprostenol).
Recently a combination therapy of LETAIRIS/ VOLIBRIS (ambrisentan) and ADCIRCA (tadalafil) also received approval. Most of these therapies are approved for adults except sildenafil, though trials are ongoing in the pediatric population for UPTRAVI, ADEMPAS, and OPSUMIT. Janssen Pharmaceuticals is also developing OPSUMIT with tadalafil as a fixed-dose combination formulation.
None of these drugs are curative and have side effects associated with their long-term use. The market has a thirst for therapies that address the shortcomings associated with the current treatment regime. Newer treatments with a novel mechanism of action are expected to enter the market and change the treatment and market regime.
According to DelveInsight, the overall dynamics of the Pulmonary Arterial Hypertension market is anticipated to change in the coming years owing to the expected launch of emerging therapies.
Key Findings
This section provides the total Pulmonary Arterial Hypertension market size and market size by therapies (therapeutic and prophylactic) in the United States.
The total Pulmonary Arterial Hypertension market size and market size by therapies (therapeutic and prophylactic) in Germany, France, Italy, Spain, and the United Kingdom are provided in this section.
The total Pulmonary Arterial Hypertension market size and market size by therapies (therapeutic and prophylactic) in Japan are provided.
This section focuses on the rate of uptake of the potential drugs recently launched in the Pulmonary Arterial Hypertension market or expected to get launched in the market during the study period 2019-2032. The analysis covers the Pulmonary Arterial Hypertension market uptake by drugs; patient uptake by therapies; and sales of each drug.
This helps in understanding the drugs with the most rapid uptake, and the reasons behind the maximal use of new drugs and allows, the comparison of the drugs based on market share and size which again will be useful in investigating factors important in market uptake and in making financial and regulatory decisions.
Pulmonary Arterial Hypertension Development Activities
The report provides insights into different therapeutic candidates in phase II, and phase III stages and also analyzes key players involved in developing targeted therapeutics.
Pipeline Development Activities
The report covers detailed information on collaborations, acquisitions, mergers, licensing, and patent details for Pulmonary Arterial Hypertension emerging therapies.
Reimbursement Scenario in Pulmonary Arterial Hypertension
Approaching reimbursement proactively can have a positive impact both during the late stages of product development and well after product launch. In the report, we consider reimbursement to identify economically attractive indications and market opportunities. When working with finite resources, the ability to select the markets with the fewest reimbursement barriers can be a critical business and price strategy.
Competitive Intelligence Analysis
We perform competitive and market Intelligence analysis of the Pulmonary Arterial Hypertension market by using various competitive intelligence tools that include-SWOT analysis, PESTLE analysis, Porter's five forces, BCG Matrix, Market entry strategies, etc. The inclusion of the analysis entirely depends upon the data availability.
Key Questions
Market Insights:
Epidemiology Insights:
Current Treatment Scenario, Marketed Drugs, and Emerging Therapies: