Pulmonary Arterial Hypertension Epidemiology Forecast

DelveInsight’s ‘Pulmonary Arterial Hypertension –Epidemiology Forecast—2030’ report delivers an in-depth understanding of the Pulmonary Arterial Hypertension , historical and forecasted epidemiology in the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan.

Geographies Covered

  • The United States
  • EU5 (Germany, France, Italy, Spain, and the United Kingdom)
  • Japan

Study Period: 2018–2030

Pulmonary Arterial Hypertension Disease Understanding

Pulmonary Arterial Hypertension (PAH) is a progressive disease of the lung vascular system, primarily affecting the small pulmonary arterioles. PH is defined as mean pulmonary artery pressure greater than 25 mm Hg measured during right heart catheterization. The term PAH describes a subset of patients who also have the presence of pre-capillary hypertension, including an end-expiratory pulmonary artery wedge pressure (less than 15 mm Hg) and a pulmonary vascular resistance greater than 3 Woods units. PAH has three subgroups: idiopathic, heritable, and pulmonary arterial hypertension related to risk factors or associated conditions.

 

Elevated pulmonary vascular resistance is caused by the proliferation of endothelial and smooth muscle cells, causing tunica media hypertrophy of small-caliber pulmonary arteries. Moreover, the endothelial cells have impaired the production of nitric oxide and prostacyclin. Patients with idiopathic and heritable forms of pulmonary artery hypertension (HPAH) are distinguished by identifying an inheritable genetic mutation; whereas, idiopathic forms have an underlying genetic predisposition to develop pulmonary hypertension. Regardless, both forms have genetic mutations that overlap each disease process.

 

PAH is most-commonly idiopathic and is characterized by increased vascular resistance and blood vessel narrowing within the pulmonary vasculature. Restricted flow through pulmonary arteries, as found in PAH, is thought to have molecular and genetic causes which lead to hypertrophy of smooth muscle, endothelial cells, and adventitia. In response to the increased resistance, the right ventricle will increase filling and stroke volume, increasing pulmonary arterial pressure. Over time, right ventricular hypertrophy develops. In the other groups of PH, increased pulmonary vascular resistance is similarly due to restricted flow, but it is typically secondary to another process such as left heart disease, chronic lung disease or chronic pulmonary thromboembolism.

 

The cardinal symptom of every form of pulmonary hypertension is progressive exercise dyspnea, often accompanied by fatigue and exhaustion. The symptoms are unspecific, so there is often a delay of many months or even years between the onset of symptoms and diagnosis. With the progression of the disease the symptoms become worse and new symptoms occur, e.g., dyspnea on bending down (bendopnea) and syncope, the latter particularly during or immediately after physical exertion. In patients with pulmonary hypertension, frequent syncope even on slight exertion clearly points to the presence of a life-threatening state associated with high mortality. In the event of cardiac decompensation the right cardiac filling pressures rise, with the typical triad of cervical venous congestion, ascites, and edema. The most frequently occurring signs, often subtle, are peripheral or central cyanosis (often only, or more strongly, during exercise), a pronounced pulmonary valve component of the second heart sound, and a systolic flow murmur reaching its maximum at a left parasternal location in tricuspid valve insufficiency.

 

Early detection and precise classification of the disease are the essential goals of diagnosis in pulmonary hypertension. With physical examination, the basic diagnostic tests in every case of uncertain or progressive exercise dyspnea should include ECG and determination of brain natriuretic peptide (BNP) or the N-terminal fragment of its precursor (NT-proBNP). If both of these show no abnormality, pulmonary hypertension is highly unlikely to be present. Further diagnostic investigations are required only in the case of strong clinical suspicion of pulmonary hypertension or if the results of the tests mentioned above are unclear. Pathologic ECG or BNP findings unequivocally indicate further cardiological investigation.

 

Treatment of PAH has progressed significantly over the past few decades in both its complexity and efficacy. The therapy aims to achieve a low-risk status (maintaining WHO-FC II if possible) to preserve patient function, quality of life and minimize mortality risk. This is generally achieved by optimizing the patient’s 6 minute walk distance (6MWD); however, current target thresholds rely upon cohort studies and expert consensus.

 

PAH management involves a stepwise pragmatic approach from general supportive treatment up to targeted pharmacological interventions.  Selected patients undergo vasoreactivity testing, with those that exhibit sufficient vasodilation subsequently being commenced on regular high-dose calcium channel blockers (CCB). Patients  who  either  fail  to  qualify for  vasoreactivity  testing  or  demonstrate  inadequate  response  are  then  commenced  on  targeted monotherapy or combination therapies that counteract the pathophysiology of the condition.

Pulmonary Arterial Hypertension Epidemiology

The Pulmonary Arterial Hypertension epidemiology division provides insights about the historical and current patient pool along with the forecasted trend for every seven major countries. It helps recognize the causes of current and forecasted trends by exploring numerous studies and views of key opinion leaders. This part of the DelveInsight report also provides the diagnosed patient pool and their trends along with assumptions undertaken.

 

Key Findings

The disease epidemiology covered in the report provides historical as well as forecasted Pulmonary Arterial Hypertension epidemiology segmented as the Prevalence of Pulmonary Arterial Hypertension, Sub-type specific prevalence of Pulmonary Arterial Hypertension, Gender-specific prevalence of Pulmonary Arterial Hypertension. The report includes the Prevalent scenario of Pulmonary Arterial Hypertension  in 7MM covering the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom), and Japan from 2018 to 2030.

Country Wise- Pulmonary Arterial Hypertension Epidemiology

The epidemiology segment also provides the Pulmonary Arterial Hypertension epidemiology data and findings across the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan.

  • The total prevalent population of Pulmonary Arterial Hypertension  associated in 7MM countries was estimated to be 71,816 cases in 2020 and expected to increase at a CAGR of 0.50% for the study period, i.e., 2018–2030.
  • As per the estimates, United States has the highest prevalent population of Pulmonary Arterial Hypertension in 7MM.
  • Among the EU5 countries, Germany had the highest prevalent population of Pulmonary Arterial Hypertension. On the other hand, Spain had the lowest number of case, 2,492 cases in 2020.

Scope of the Report

  • Pulmonary Arterial Hypertension report covers a detailed overview explaining its causes, symptoms, classification, pathophysiology, diagnosis, and treatment patterns
  • Pulmonary Arterial Hypertension Epidemiology Report and Model provide an overview of the risk factors and global trends of Pulmonary Arterial Hypertension  in the seven major markets (7MM: US, France, Germany, Italy, Spain, UK, and Japan)
  • The report provides insight into the historical and forecasted patient pool of Pulmonary Arterial Hypertension  in seven major markets covering the United States, EU5 (Germany, Spain, France, Italy, UK), and Japan
  • The report helps recognize the growth opportunities in the 7MM concerning the patient population
  • The report assesses the disease risk and burden and highlights the unmet needs of Pulmonary Arterial Hypertension
  • The report provides the segmentation of the Pulmonary Arterial Hypertension epidemiology by prevalent Cases of Pulmonary Arterial Hypertension in 7MM
  • The report provides the segmentation of the Pulmonary Arterial Hypertension epidemiology by Prevalence of Pulmonary Arterial Hypertension, Sub-type specific prevalence of Pulmonary Arterial Hypertension, Gender-specific cases of Pulmonary Arterial Hypertension in 7MM

Report Highlights

  • 10-year Forecast of Pulmonary Arterial Hypertension epidemiology
  • 7MM Coverage
  • Total Prevalent Cases of Pulmonary Arterial Hypertension
  • Sub-type Specific Prevalent Cases of Pulmonary Arterial Hypertension
  • Gender Specific Prevalence of Pulmonary Arterial Hypertension 

KOL Views

We interview KOLs, and SME’s opinion through primary research to fill the data gaps and validate our secondary research. The opinion helps understand the total patient population and current treatment pattern. This will support the clients in potential upcoming novel treatment by identifying the overall scenario of the indications.

Key Questions Answered

  • What will be the growth opportunities in the 7MM concerning the patient population about Pulmonary Arterial Hypertension?
  • What are the key Findings of Pulmonary Arterial Hypertension epidemiology across 7MM, and which country will have the highest number of patients during the forecast period (2021–2030)?
  • What would be the total number of patients with Pulmonary Arterial Hypertension across the 7MM during the forecast period (2021–2030)?
  • Among the EU5 countries, which country will have the highest number of patients during the forecast period (2021–2030)?
  • At what CAGR the patient population is expected to grow by 7MM during the forecast period (2021–2030)?
  • What are the disease risk, burden, and unmet needs of Pulmonary Arterial Hypertension?
  • What are the currently available treatments for Pulmonary Arterial Hypertension?

Reasons to buy

The Pulmonary Arterial Hypertension Epidemiology report will allow the user to -

  • Develop business strategies by understanding the trends shaping and driving the global Pulmonary Arterial Hypertension market
  • Quantify patient populations in the global Pulmonary Arterial Hypertension market to improve product design, pricing, and launch plans
  • Organize sales and marketing efforts by identifying the gender based that present the best opportunities for Pulmonary Arterial Hypertension therapeutics in each of the markets covered
  • Understand the magnitude of Pulmonary Arterial Hypertension  population by its prevalent cases
  • Understand the magnitude of Pulmonary Arterial Hypertension population by its clinical manifestation specific cases
  • The Pulmonary Arterial Hypertension epidemiology report and model was written and developed by Masters and PhD level epidemiologists
  • The Pulmonary Arterial Hypertension Epidemiology Model developed by DelveInsight is easy to navigate, interactive with dashboards, and epidemiology based on transparent and consistent methodologies. Moreover, the model supports data presented in the report and showcases disease trends over a 10-year forecast period using reputable sources

Key Assessments

  • Patient Segmentation
  • Disease Risk and Burden
  • Risk of disease by the segmentation
  • Factors driving growth in a specific patient population

1. Key Insights

2. Report Introduction

3. Pulmonary Arterial Hypertension Market Overview at a Glance

3.1. Market Share (%) Distribution of Pulmonary Arterial Hypertension (PAH) in 2018

3.2. Market Share (%) Distribution of Pulmonary Arterial Hypertension (PAH) in 2030

4. Executive Summary of Pulmonary Arterial Hypertension

5. Organizations contributing towards Pulmonary Arterial Hypertension

6. Disease Background and Overview

6.1. Introduction

6.2. Signs and Symptoms

6.3. Classification of Pulmonary Arterial Hypertension (PAH)

6.3.1. WHO classification

6.3.2. Functional Classification of Pulmonary Arterial Hypertension

6.4. Etiology

6.5. Risk factors

6.6. Pathophysiology

6.6.1. Nitric Oxide Pathway

6.6.2. Prostacyclin-Thromboxane A2 Pathway

6.6.3. Endothelin-1 Pathway

6.7. Diagnosis

7. Management and Treatment

7.1. Treatment Guidelines

7.1.1. CHEST Guidelines [The United States]

7.1.2. European Society of Cardiology (ESC) and of the European Respiratory Society (ERS) Guidelines

8. Epidemiology and Patient Population

8.1. Key Findings

8.2. 7MM Total Prevalent Patient Population of Pulmonary Arterial Hypertension

8.3. Assumption and Rationale

8.4. The United States

8.4.1. Total Prevalent Patient Population of Pulmonary Arterial Hypertensionin in the United States

8.4.2. Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in the United States

8.4.3. Gender specific Prevalence of Pulmonary Arterial Hypertension in the United States

8.5. EU5

8.5.1. Germany

8.5.1.1. Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Germany

8.5.1.2. Sub-type Specific Prevalence of Pulmonary Arterial in Germany

8.5.1.3. Gender specific Prevalence of Pulmonary Arterial Hypertension in Germany

8.5.2. France

8.5.2.1. Total Prevalent Patient Population of Pulmonary Arterial Hypertension in France

8.5.2.2. Sub-type Specific Prevalence of Pulmonary Arterial in France

8.5.2.3. Gender specific Prevalence of Pulmonary Arterial Hypertensionin France

8.5.3. Italy

8.5.3.1. Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Italy

8.5.3.2. Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in Italy

8.5.3.3. Gender specific Prevalence of Pulmonary Arterial Hypertension in Italy

8.5.4. Spain

8.5.4.1. Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Spain

8.5.4.2. Sub-type Specific Prevalence of Pulmonary Arterial in Spain

8.5.4.3. Gender specific Prevalence of Pulmonary Arterial Hypertension in Spain

8.5.5. United Kingdom

8.5.5.1. Total Prevalent Patient Population of Pulmonary Arterial Hypertension in United Kingdom

8.5.5.2. Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in United Kingdom

8.5.5.3. Gender specific Prevalence of Pulmonary Arterial Hypertension in the United Kingdom

8.6. Japan

8.6.1. Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Japan

8.6.2. Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in Japan

8.6.3. Gender specific Prevalence of Pulmonary Arterial Hypertensionin Japan

9. Patient Journey

10. Case Reports

11. KOL Views

12. Appendix

13. Bibliography

14. Report Methodology

15. DelveInsight Capabilities

16. Disclaimer

17. About DelveInsight

List of Table

Table 1: Summary of Pulmonary Arterial Hypertension, Market, Epidemiology, and Key Events (2018–2030)

Table 2: Organizations contributing towards Pulmonary Arterial Hypertension (PAH)

Table 3: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in 7MM (2018–2030)

Table 4: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in the United States (2018–2030)

Table 5: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in the United States (2018–2030)

Table 6: Gender specific Prevalence of Pulmonary Arterial Hypertension in United States (2018–2030)

Table 7: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Germany (2018–2030)

Table 8: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in Germany (2018–2030)

Table 9: Gender specific Prevalence of Pulmonary Arterial Hypertension in Germany in 000's (2018–2030)

Table 10: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in France (2018–2030)

Table 11: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in France in 000's (2018–2030)

Table 12: Gender specific Prevalence of Pulmonary Arterial Hypertension in France in 000's (2018–2030)

Table 13: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Italy (2018–2030)

Table 14: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in Italy (2018–2030)

Table 15: Gender specific Prevalence of Pulmonary Arterial Hypertension in Italy (2018–2030)

Table 16: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Spain (2018–2030)

Table 17: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in Spain in 000's (2018–2030)

Table 18: Gender specific Prevalence of Pulmonary Arterial Hypertension in Spain (2018–2030)

Table 19: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in United Kingdom (2018–2030)

Table 20: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in United Kingdom in 000's (2018–2030)

Table 21: Gender specific Prevalence of Pulmonary Arterial Hypertension in United Kingdom (2018–2030)

Table 22: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Japan (2018–2030)

Table 23: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in Japan in 000's (2018–2030)

Table 24: Gender specific Prevalence of Pulmonary Arterial Hypertension in Japan (2018–2030)

Table 25: Pemziviptadil (PB1046), Clinical Trial Description, 2021

Table 26: Sotatercept, Clinical Trial Description, 2021

Table 27: Ralinepag, Clinical Trial Description, 2021

Table 28: LIQ-861, Clinical Trial Description, 2021

Table 29: Rodatristat Ethyl, Clinical Trial Description, 2021

Table 30: AV-101, Clinical Trial Description, 2021

Table 31: RT234, Clinical Trial Description, 2021

Table 32: GB002, Clinical Trial Description, 2021

Table 33: MK-5475, Clinical Trial Description, 2021

Table 34: Treprostinil Palmitil, Clinical Trial Description, 2021

Table 35: L606, Clinical Trial Description, 2021

List of Figures

Figure 1: Some of the common signs & symptoms of Pulmonary Arterial Hypertension (PAH)

Figure 2: Depiction of a Persistent Epithelial Defect (PED)

Figure 3: Functional Classification of PAH

Figure 4: Risk Factors of Pulmonary Arterial Hypertension

Figure 5: Pathophysiology of Pulmonary Arterial Hypertension (PAH)

Figure 6: Pathophysiology of Pulmonary Arterial Hypertension (PAH)

Figure 7: Diagnosis Algorithm of Pulmonary Arterial Hypertension (PAH)

Figure 8: Treatment Options for Pulmonary Arterial Hypertension (PAH)

Figure 9: Treatment Algorithm of Pulmonary Arterial Hypertension (PAH)

Figure 10: Total Prevalent Population of Pulmonary Arterial Hypertension in 7MM (2018–2030)

Figure 11: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in the United States   (2018–2030)

Figure 12: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in the United States   (2018–2030)

Figure 13: Gender specific Prevalence of Pulmonary Arterial Hypertension in United States (2018–2030)

Figure 14: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Germany (2018–2030)

Figure 15: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in Germany (2018–2030)

Figure 16: Gender specific Prevalence of Pulmonary Arterial Hypertension in Germany (2018–2030)

Figure 17: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in France (2018–2030)

Figure 18: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in France (2018–2030)

Figure 19: Gender specific Prevalence of Pulmonary Arterial Hypertension in France (2018–2030)

Figure 20: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Italy (2018–2030)

Figure 21: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in Italy (2018–2030)

Figure 22: Gender specific Prevalence of Pulmonary Arterial Hypertension in Italy (2018–2030)

Figure 23: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Spain (2018–2030)

Figure 24: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in Spain (2018–2030)

Figure 25: Gender specific Prevalence of Pulmonary Arterial Hypertension in Spain (2018–2030)

Figure 26: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in United Kingdom (2018–2030)

Figure 27: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in United Kingdom (2018–2030)

Figure 28: Gender specific Prevalence of Pulmonary Arterial Hypertension in United Kingdom (2018–2030)

Figure 29: Total Prevalent Patient Population of Pulmonary Arterial Hypertension in Japan (2018–2030)

Figure 30: Sub-type Specific Prevalence of Pulmonary Arterial Hypertension in Japan (2018–2030)

Figure 31: Gender specific Prevalence of Pulmonary Arterial Hypertension in Japan (2018–2030)

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