Being told that you have pulmonary arterial hypertension (PAH) can feel scary and leave you wondering what this diagnosis means for your future. PAH is a type of pulmonary hypertension that raises the pressure in the pulmonary arteries, which are the blood vessels that carry blood from your heart to your lungs.

In PAH, these blood vessels become narrower, making it harder for blood to flow through. PAH is a progressive lung disease, which means it gets worse over time, and it can lead to serious and life-threatening symptoms if it’s not treated.

The good news is that PAH treatments have improved a lot in recent years. These advances have helped extend the life expectancy (how long a person is expected to live) of people with PAH by several years.

If you have PAH, your prognosis (expected outlook with the disease) depends on several factors. Keep reading to learn more about factors that can affect life expectancy with PAH.

1. Race and Ethnicity

Various studies have looked at how race and ethnicity might affect the outlook for people with pulmonary arterial hypertension. Some researchers have reported that Black and Asian people in the United States tend to have a poorer outlook with PAH, even if they don’t have other health problems. A 2008 study published in the Journal of the National Medical Association found that African American women, in particular, had a worse prognosis compared with other racial and gender groups with PAH.

However, not all research findings agree. A large five-year U.S. study found no link between race or ethnicity and the risk of death in people with PAH. This 2020 study used data from more than 3,000 people in the Registry to Evaluate Early and Long-Term PAH Disease Management (Reveal registry), which tracked people with PAH in the United States.

Differences in Type of PAH and Outlook

Researchers have reported that the type of PAH a person has may differ by race. For example, the 2020 study found that Black Americans were more likely to have connective tissue disease-associated PAH, which is linked to autoimmune diseases like lupus or scleroderma. Other studies have shown that this type of PAH carries a higher risk of death than idiopathic PAH (PAH with no known cause).

The Reveal registry study showed that Hispanic people were more likely to have portopulmonary hypertension, which is linked to liver disease and has a worse prognosis than idiopathic PAH. Asian Americans were more often diagnosed with congenital heart disease-associated PAH, which is linked to heart problems present at birth.

2. Socioeconomic Status

Socioeconomic status refers to factors such as a person’s income, education level, and type of job. People with lower socioeconomic status may have less access to financial and health resources than those with a higher socioeconomic status. Studies have found that these disparities can affect the health outlook of people with PAH.

An analysis of the Reveal registry showed that people with lower socioeconomic status had to wait longer for a PAH diagnosis.

An analysis of the Reveal registry showed that people with lower socioeconomic status often waited longer to be diagnosed with PAH compared with others. Early diagnosis and treatment can lead to better outcomes, so delays may result in a worse prognosis. The study also found that Black and Hispanic people were more likely than other groups to have lower incomes. In addition, a 2022 study of low-income U.S. veterans found that this group often faced major delays in getting treatment, which can make PAH outcomes worse.

3. Age

Older age is linked to a higher risk of dying from PAH. A 2020 study from France looked at people with connective tissue disease-associated PAH and found that life expectancy improved between 2006 and 2017 for most people — but not those who were at least 70 years of age. Older adults are more likely to have other health conditions, such as high blood pressure, heart failure, or renal (kidney) problems, which can make PAH harder to manage.

Also, older adults may not respond as well to PAH treatment and are more likely to have to stop treatment due to serious side effects.

4. Type of Pulmonary Arterial Hypertension

Different types of PAH are diagnosed based on the cause of the condition. Each type can lead to different outcomes. However, no matter the type, the right treatment can help you live longer and feel better with pulmonary arterial hypertension.

Idiopathic PAH

Idiopathic PAH is diagnosed when there’s no known cause for the disease. Without treatment, people with this type often have a poor outlook. On average, people who don’t get treatment for idiopathic PAH live about two to three years after they’re diagnosed.

On average, people who aren't treated for idiopathic PAH live about two to three years after they’re diagnosed. It's essential to get treatment for this condition.

Heritable PAH

Heritable PAH — which includes familial PAH and cases with known gene mutations (changes), even without a family history— can be passed down from parents to their children. Women are up to three times more likely than men to develop familial PAH, according to the Pulmonary Hypertension Association. However, a 2018 study in the journal Heart Vessels found that males with this type tend to have worse health outcomes.

People at risk of familial PAH may be able to improve their outcomes by getting regular checkups to watch for early signs of the disease.

Drug-Induced PAH

Drug-induced PAH is caused by certain substances that damage the lungs’ blood vessels. These substances include some prescription medications (such as older appetite suppressants), amphetamines (stimulant drugs), and illegal drugs. People with methamphetamine-induced PAH may have lower survival rates than people with idiopathic PAH.

Associated PAH

Some health conditions can increase the risk of PAH. These conditions include connective tissue diseases such as systemic scleroderma (also called systemic sclerosis), lupus, and mixed connective tissue disease.

One study found that people aged 70 or younger with scleroderma-associated PAH had a three-year survival rate of nearly 82 percent. This means 82 out of 100 people with this type of PAH were alive three years after diagnosis.

5. Functional Class of Pulmonary Arterial Hypertension

The functional class is a way to describe how much PAH affects your daily life. Pulmonary hypertension (PH) and pulmonary arterial hypertension are grouped into four classes defined by the World Health Organization (WHO). Class 1 is the mildest form, and class 4 is the most severe.

Functional class is also used to help predict life expectancy. People with class 4 PAH are in the high-risk category and, without treatment, may live less than six months, on average.

6. Complications of Pulmonary Arterial Hypertension

Your life expectancy with PAH may also depend on other health problems or complications caused by the disease.

Right-sided heart failure is a serious and common PAH complication. This condition can happen when high pressure in the lungs makes the right side of the heart work too hard to pump blood. Over time, this can weaken the heart. People with high right atrial pressure (increased pressure in the heart’s right upper chamber) often have a worse prognosis with PAH.

Right-sided heart failure, a common complication of PAH, can affect your outlook.

7. Treatments for Pulmonary Arterial Hypertension

Over the years, PAH treatments have improved significantly. As a result, pulmonary hypertension life expectancy with treatment is also better.

Calcium Channel Blockers

Calcium channel blockers help lower blood pressure and relax blood vessels so that more blood can flow through. Although these are useful medications, only about 5 percent of people with PAH respond well to them — and only if they meet strict criteria based on vasoreactivity testing, which checks if the lung arteries can relax in response to medication.

Prostaglandins

Prostaglandins also help open and relax blood vessels. Like calcium channel blockers, they’re effective for some people but not everyone. These medications may help ease symptoms and slow down how quickly PAH gets worse. When used as part of combination therapy, prostaglandins may help improve life expectancy.

Endothelin Receptor Antagonists

Endothelin receptor antagonists block endothelin, a substance in the blood that causes blood vessels to tighten. By lowering endothelin levels, the drugs help keep blood vessels open. Research shows that these medications may slow PAH progression and even reverse some damage. A study in the European Respiratory Journal found that these drugs significantly improved survival in people with PAH.

Phosphodiesterase-5 Inhibitors

Phosphodiesterase-5 (PDE5) inhibitors work by blocking the PDE5 enzyme to help regulate blood flow and widen the arteries in the lungs. These medications have been shown to increase survival and improve symptoms in people with PAH.

Activin Signaling Inhibitors

Activin signaling inhibitors are a newer type of PAH treatment that helps prevent narrowing of blood vessels in the lungs. Researchers don’t yet know how these medications may affect life expectancy, but clinical trials — research studies that test how well new therapies work in people — show that they can significantly improve how much physical activity someone with PAH can do.

Soluble Guanylate Cyclase Stimulators

Soluble guanylate cyclase stimulators help relax blood vessels in the lungs, making it easier for blood to flow. Long-term use of these medications may help improve other factors that can affect life expectancy, such as functional class.

Supportive Therapies

Supportive therapies help manage symptoms and improve quality of life. For example, anticoagulants (blood thinners) may help prevent blood clots and could increase life expectancy with PAH. Other treatments such as diuretics (water pills) can reduce swelling, and oxygen therapy may help you breathe more comfortably.

Work With Your Doctor

There isn’t a one-size-fits-all treatment for pulmonary arterial hypertension. But thanks to advances in medicine, people with PAH now have more options to manage symptoms, improve daily life, and extend life expectancy. Working closely with your healthcare provider can help you find the right treatment plan for your specific type of PAH and give you the best chance at feeling better and living longer.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 56,000 members come together to ask questions, give advice, and share their experiences of life with pulmonary hypertension.

Are you or a loved one living with pulmonary arterial hypertension? What has your experience been like? Share your thoughts in the comments below, or start a conversation by posting on your Activities page.