Pulmonary Arterial Hypertension Epidemiology

Key Highlights

• Pulmonary arterial hypertension is a rare, progressive disorder characterized by hypertension in the pulmonary arteries for no apparent reason. 
• Pulmonary arterial hypertension is 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 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.
• 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. 
• According to DelveInsight’s estimates, the total prevalent cases of Pulmonary arterial hypertension in the 7MM were estimated to be around 112,500 cases in 2024, which are expected to increase by 2034. 
• In 2024, the total diagnosed cases of PAH in the US were 43,300 which as per DelveInsight’s estimates, are expected to increase by 2034.
• In 2024, there were 900 cases of Pulmonary arterial hypertension in the < 18 yrs age group and 16,500 cases in the>75 yrs age group in the US.
• In EU4 and the UK, Stage III accounted for the highest number of cases in 2024.
• Females are more affected than males by PAH.
• In 2024, Idiopathic/Heritable PAH accounted for the highest number of cases of PAH which is 18,500 in the US.

DelveInsight’s “Pulmonary arterial hypertension– Epidemiology Forecast – 2034” report delivers an in-depth understanding of Pulmonary arterial hypertension, historical and forecasted epidemiology in the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan.

The table given below further depicts the key segments provided in the report:

Pulmonary arterial hypertension: Disease Understanding 

Pulmonary arterial hypertension Overview, and Diagnosis

Pulmonary arterial hypertension (PAH), characterized by increased pulmonary vascular resistance and arterial pressure, affects an estimated 15–60 per million of the population and is more commonly diagnosed in women. PAH causes a range of nonspecific symptoms (including breathlessness, fatigue, chest pain, and weakness) and is associated with significant morbidity and mortality triggered by the debilitating progressive nature of the disease, which eventually leads to right heart failure and death. The effect of disease symptoms on the patient’s physical mobility and emotional state adversely affects health-related quality of life (HRQoL).

PAH, symptoms generally occur due to insufficient oxygen in the blood; signs and symptoms of PAH worsen with the progression of the disease. The main symptom is shortness of breath; fatigue, dizziness, swelling in the limbs, blue lips, irregular heartbeats, and chest pain are also reported. Each person with PAH will experience a different assortment of symptoms. The severity of the symptoms will also differ from person to person. One person’s journey with having and treating PAH would not necessarily be helpful to another person because the path with PAH and the treatment options are individualized.

PAH is characterized by similar symptoms that appear during heart and lung conditions. Several techniques and blood tests, such as chest X-rays, blood tests, cardiac catheterization, electrocardiograms, and echocardiograms, are used for diagnosing PAH.

The Pulmonary arterial hypertension report provides an overview of PAH pathophysiology, diagnostic approaches, and detailed diagnostic algorithm along with a real-world scenario of a patient’s journey beginning from the first symptom, and the time taken for diagnosis. 

Pulmonary arterial hypertension Epidemiology

The Pulmonary arterial hypertension epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by total prevalent cases, total diagnosed prevalent cases, age-specific cases, gender-specific cases, class-specific cases, subtype-specific cases, and total treated cases of Pulmonary arterial hypertension in the United States, EU4 (Germany, France, Italy, Spain) and the United Kingdom, and Japan from 2021 to 2034.

• The United States accounted for the highest proportion of diagnosed prevalent cases, representing approximately 45% of the total cases across the 7MM, followed by Japan with around 25%.
• In 2024, Idiopathic/Heritable PAH recorded the highest number of cases, with around 19,000, while Pulmonary Veno-Occlusive Disease had the lowest, with approximately 200 cases in the US.
• Among EU4 and the UK, in 2024 Germany had the highest prevalent cases of PAH, with 8,500, while Spain had the lowest, with 5,000 cases.
• In 2024, France reported approximately 1,700 cases among males and 4,000 among females, as PAH is more prevalent in women compared to men.
• In 2024, Italy reported approximately 50% of cases of Class III, followed by around 35% of Class II, 10% of Class I, and about 5% of Class IV.
• The total number of diagnosed cases of PAH in Japan was around 23,000 in 2024 which accounted for around 25% of the total cases of 7MM.
• In 2024, Japan reported approximately 3,800 cases of pulmonary arterial hypertension (PAH) in the 46–55 age group, making it the most affected age group. The 56–65 age group followed closely, with around 3,500 reported cases.

Scope of the Pulmonary arterial hypertension Epidemiology Report

• The report covers a segment of key events, an executive summary, and a descriptive overview of Pulmonary arterial hypertension, explaining its causes, signs and symptoms, pathogenesis, and current diagnosis methods.
• Comprehensive insight into the epidemiology segments and forecasts, the future growth potential of diagnosis rate, and disease progression have been provided.
• A detailed review of current challenges in establishing diagnosis and diagnosis rate is provided.

Pulmonary Arterial Hypertension Report Insights

• Pulmonary arterial hypertension Patient Population
• Country-wise Epidemiology Distribution

Pulmonary Arterial Hypertension Report Key Strengths

• Ten-year Forecast
• The 7MM Coverage 
• Pulmonary Arterial Hypertension Epidemiology Segmentation

Pulmonary Arterial Hypertension Report Assessment

• Pulmonary arterial hypertension Epidemiology Segmentation
• Current Pulmonary arterial hypertension Diagnostic Practices

FAQs

Pulmonary arterial hypertension Epidemiology Insights

• What are the disease risks, burdens, and unmet needs of Pulmonary arterial hypertension? What will be the growth opportunities across the 7MM concerning the patient population pertaining to Pulmonary arterial hypertension?
• What is the historical and forecasted Pulmonary arterial hypertension patient pool in the United States, EU4 (Germany, France, Italy, Spain) and the United Kingdom, and Japan?
• What is the diagnostic pattern of Pulmonary arterial hypertension?
• Which clinical factors will affect Pulmonary arterial hypertension?
• Which factors will affect the increase in the diagnosis of Pulmonary arterial hypertension?

Reasons to buy Pulmonary arterial hypertension Epidemiology Report

• Insights on disease burden, details regarding diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.
• To understand the change in Pulmonary arterial hypertension cases in varying geographies over the coming years.
• A detailed overview of the total prevalent cases of Pulmonary arterial hypertension, total diagnosed prevalent cases, age-specific cases, gender-specific cases, class-specific cases, and subtype-specific cases of Pulmonary arterial hypertension of Pulmonary arterial hypertension is included.
• To understand the perspective of key opinion leaders around the current challenges with establishing the diagnosis and insights on the treatment-eligible patient pool.
• Detailed insights on various factors hampering disease diagnosis and other existing diagnostic challenges