Pulmonary Arterial Hypertension Market

Key Highlights

The total prevalent cases of PAH in the 7MM were estimated to be approximately 87,713 cases in 2021, which are expected to increase by 2032.
According to DelveInsight estimates, in 2021, the total diagnosed prevalent cases of Pulmonary Arterial Hypertension were estimated to be approximately 52,628 in the 7MM. These cases are expected to increase by 2032 at a CAGR of 0.4% during the study period (2019–2032).
Pulmonary Arterial Hypertension epidemiology is segmented as Total Pulmonary Arterial Hypertension Prevalent Cases, Total Pulmonary Arterial Hypertension Diagnosed Cases, Pulmonary Arterial Hypertension Age-specific Cases, Pulmonary Arterial Hypertension Class-specific Cases, Pulmonary Arterial Hypertension Gender-specific Cases, and Pulmonary Arterial Hypertension Subtype-specific Cases in the Pulmonary Arterial Hypertension market report.

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DelveInsight’s ‘Pulmonary Arterial Hypertension - Market Insights, Epidemiology, and Market Forecast–2032’ report deliver 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.

Geography Covered

The United States
EU4 (Germany, France, Italy, and Spain) and the United Kingdom
Japan

Study Period: 2019–2032

Pulmonary Arterial Hypertension Market Disease: Understanding and Treatment Algorithm

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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Pulmonary Arterial Hypertension Epidemiology

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 total prevalent cases of PAH in the 7MM were estimated to be approximately 87,713 cases in 2021, which are expected to increase by 2032.
According to DelveInsight estimates, in 2021, the total diagnosed prevalent cases of Pulmonary Arterial Hypertension were estimated to be approximately 52,628 in the 7MM. These cases are expected to increase by 2032 at a CAGR of 0.4% during the study period (2019–2032).
Among the 7MM, the United States accounted for nearly 58% of the total diagnosed prevalent cases of Pulmonary Arterial Hypertension in the 7MM in the year 2021, which are expected to increase further by 2032.
As per DelveInsight analysis, in EU4 and the United Kingdom, Germany had the highest total diagnosed prevalent cases of Pulmonary Arterial Hypertension with approximately 5,065 in 2021, while Spain had the lowest diagnosed prevalent population of Pulmonary Arterial Hypertension with approximately 2,826 cases in 2021.
In 2021, the diagnosed prevalent population of Pulmonary Arterial Hypertension in Japan was found to be approximately 2,394 cases, which is expected to decrease during the study period of 2019–2032.
According to estimates based on DelveInsight’s epidemiology model for Pulmonary Arterial Hypertension, the gender distribution of the disease suggests a female predominance across the 7MM, with approximately 7,348 male and 23,193 female cases in the US in 2021.

Pulmonary Arterial Hypertension Epidemiology

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.

Pulmonary Arterial Hypertension Drug Chapters

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.