• The life expectancy for people living with pulmonary arterial hypertension (PAH) has vastly improved in recent years due to new treatments.
• The type of PAH you have affects your prognosis (outlook) with the disease.
• Several treatments help manage PAH and improve life expectancy and quality of life.

If you’ve recently received a PAH diagnosis, you may be wondering about your prognosis. Fortunately, treatments for PAH have vastly improved in recent years. Advancements have helped extend the life expectancy (how long a person is expected to live) of those with PAH by several years.

If you have PAH, your outlook depends on several factors, including the type of PAH you’re diagnosed with, your overall health, your race or ethnic background, and the type of treatment you receive.

What Is PAH?

PAH is a rare disease that’s often difficult to diagnose. This is because many people don’t show early signs of PAH, and it’s typically not seen during a routine physical exam.

PAH is caused by the narrowing of blood vessels in your lungs, which increases blood pressure and something called pulmonary vascular resistance. This puts strain on the right side of the heart as it continues to pump blood. Over time, increased blood pressure in the lungs can cause damage, more noticeable symptoms, and in some cases, right-sided heart failure.

Functional Classes of Pulmonary Hypertension

Pulmonary hypertension (PH) and PAH are broken down into four different classes defined by the World Health Organization (WHO). Class 1 is the mildest form and class 4 is the most severe. Your PAH class depends on how much your physical and daily activities are limited by PAH. The classes are as follows:

• Class 1 — You have no PH or PAH symptoms at rest or while exercising.
• Class 2 — You have no PH or PAH symptoms at rest, but you do have shortness of breath performing everyday activities, such as climbing the stairs or walking around a store.
• Class 3 — You may not have PH or PAH symptoms at rest, but you’re greatly limited in performing everyday activities due to shortness of breath or fatigue.
• Class 4 — You have PH or PAH symptoms at rest and with everyday activities. You may also have edema (swollen feet and ankles) and fainting or lightheadedness.

Types of PAH

There are four types of PAH that are diagnosed based on the cause of the condition. Each is associated with different outcomes, but they can be treated to improve the length and quality of your life with the condition.

Idiopathic PAH

Idiopathic pulmonary arterial hypertension (IPAH) is diagnosed when there’s no known cause for the disease. Your doctor won’t be able to connect it to other common causes. People with IPAH tend to have a poor outlook if it’s left untreated. The average life expectancy for people who don’t get treatment for their IPAH is two to three years after it’s first diagnosed.

Functional class is another measure used to predict life expectancy. Those with class 4 PAH are in the high-risk category and tend to have an average survival of less than six months if they don’t get treatment. Another predictor is how well the right ventricle (lower right chamber of your heart) is working.

Recent advancements in treatment have helped extend the life expectancy of many people with PAH to seven years or more. Getting diagnosed and treated early can help you live longer with a better quality of life.

Heritable PAH

Familial PAH (FPAH), or heritable PAH, is passed down from parents to their children. This form tends to run in families that carry the BMPR2 gene. To develop FPAH, you only need one copy of this gene from either parent — however, only around 20 percent of people who have the gene develop PAH. Although women are twice as likely to develop FPAH than men, men tend to have worse health outcomes.

Drug-Induced PAH

Drug-induced PAH is caused by certain medications that damage your lung’s blood vessels. Many of these medications are used as appetite suppressants or amphetamines. Illicit substance use can also cause drug-induced PAH. Studies show the outlook for drug-induced PAH is similar to that for IPAH.

Associative PAH

There are several other health conditions associated with developing PAH. For example, people with connective tissue diseases such as scleroderma, lupus, or mixed connective tissue disease are at an increased risk of developing PAH. One study found that those aged 70 and younger with scleroderma-associated PAH (also called systemic sclerosis) had a three-year survival rate of nearly 82 percent.

Other conditions that may cause PAH include:

• Congenital heart disease (heart defect at birth)
• High blood pressure in the main vein that leads to the liver
• Sleep apnea
• HIV infection
• Advanced liver disease
• Lung disease
• Left heart disease

Impact of Race and Ethnicity on PAH Outlook

Some studies have looked into how race, ethnicity, and socioeconomic status play a role in the prognosis of PAH. These have found that Black and Asian populations in the U.S. have a poorer outlook with PAH, regardless of whether they have other health issues.

African Americans tend to develop associative PAH more often, especially PAH related to connective tissue diseases and liver problems. Another study from the Journal of the National Medical Association showed that African American women specifically have a poorer outlook with PAH when compared to other gender or racial groups.

Scientists are looking to address diagnosis and treatment gaps in these groups to improve health outcomes.

Complications Can Affect PAH Prognosis

Life expectancy with PAH may also depend on other risk factors, medical conditions, and complications. As time passes, PAH can cause other conditions that complicate PAH treatment and affect outcomes. Accordingly, these conditions are called complications.

Right heart failure is a common complication of PAH that can happen when the buildup of pulmonary arterial pressure makes the right ventricle of the heart work harder to move blood to the lungs.

Blood from the body returns to the right side of the heart. When right heart failure occurs, fluid can build up in the venous system and overwhelm the heart’s ability to pump enough blood.

Outcomes With Different PAH Treatments

Over the years, PAH treatments have improved significantly. As a result, the life expectancy of those with the disease has also improved. Many treatments are given as combination therapy (using more than one treatment at a time) to help relax blood vessels, treat high blood pressure, and even reverse damage from PAH.

Calcium Channel Blockers

Calcium channel blockers help lower blood pressure by blocking calcium from entering the cells in the arteries and heart. This prevents them from contracting, allowing them to relax and let more blood flow through. Although these are useful medications, only around 5 percent of people with PAH respond well to them in the long term. There aren’t many studies available that show if calcium channel blockers improve life expectancy for people with PAH.

Prostaglandins

Prostaglandins are a class of medications that mimic the effects of the hormone prostacyclin (prostanoids) in the body, which helps relax blood vessels. Known as vasodilators, these medications help reduce blood pressure and may be effective in some people for treating PAH. Like calcium channel blockers, only a certain group of people respond well to them.

These medications may help improve daily symptoms and keep PAH from getting worse. Examples of medications that work on the prostacyclin pathway include:

• Epoprostenol (Flolan)
• Iloprost (Ventavis)
• Selexipag (Uptravi)
• Treprostinil (Orenitram, Remodulin, Tyvaso)

Endothelin Receptor Antagonists

Endothelin receptor antagonists work by limiting the amount of endothelin in the blood, which prevents your blood vessels from narrowing. Research shows that these medications may slow PAH progression and even reverse damage. Per a study in the European Respiratory Journal, these medications have been shown to significantly improve people’s survival with PAH.

The U.S. Food and Drug Administration (FDA) has approved three endothelin receptor antagonists for treating PAH:

• Ambrisentan (Letairis)
• Bosentan (Tracleer)
• Macitentan (Opsumit)

Phosphodiesterase-5 Inhibitors

Phosphodiesterase-5 (PDE-5) inhibitors work by blocking the PDE-5 enzyme to help regulate blood flow and widen the arteries in the lungs. These medications have been shown to improve survival rates and disease outcomes in those with PAH.

The FDA has approved two PDE-5 inhibitors for PAH, sildenafil (Revatio) and tadalafil (Adcirca).

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 53,000 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 experience in the comments below, or start a conversation by posting on your Activities page.