Risk assessment is a key part of managing pulmonary arterial hypertension (PAH). It helps doctors understand how your condition may change over time and guides important care decisions. For a risk assessment, doctors look at your symptoms, test results, and overall health to estimate your prognosis (outlook) with PAH. If you’re living with PAH, having regular risk assessments is important to make sure your treatment plan stays on track.

In 2022, the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) updated their treatment guidelines for PAH. The ESC/ERS recommendations include doing a risk assessment when you’re first diagnosed and then at least every three months during follow-up visits.

Following these pulmonary hypertension guidelines provides a risk profile that can help your doctor see if your condition is staying the same, improving, or getting worse. Each assessment also shows how well your treatment is working and whether your current treatment plan should be adjusted.

Understanding PAH Risk Categories

Your healthcare provider will classify your PAH as low, intermediate, or high risk. This risk category may change based on the results of tests designed to figure out how PAH affects your body.

Low-Risk PAH

PAH is considered low risk when there are no signs of right heart failure and no worsening of symptoms. One important test is the six-minute walk distance (6MWD), which checks how far you can walk in six minutes. A distance of more than 440 meters is a good sign.

Doctors also use the World Health Organization Functional Class (WHO-FC) system to rate how much PAH affects a person’s daily life. Low-risk PAH usually falls into class 1 (no symptoms with activity) or class 2 (mild symptoms with regular activity). If you’re in the low-risk group, your chance of dying within one year is estimated to be less than 5 percent.

Intermediate-Risk PAH

People who fall into the intermediate-risk range of PAH have no signs of right heart failure, but doctors may see signs of slow disease progression. Scores on the 6MWD typically range from one-tenth to one-quarter mile (165 to 440 meters).

Intermediate risk is classified as WHO-FC 3, which means symptoms happen with less-than-normal physical activity. In intermediate PAH, the chance of dying within one year rises to 5 percent to 20 percent.

High-Risk PAH

People with high-risk PAH often have clear signs of right heart failure and quickly worsening symptoms. These individuals usually walk less than one-tenth of a mile on the 6MWD test.

High-risk PAH is typically WHO-FC 4, which means symptoms occur even at rest or with very little activity. In this group, the chance of dying within one year is estimated to be higher than 20 percent.

A heart rate over 96 beats per minute is considered a sign that PAH is worsening.

Overall, the goal of PAH treatment is to help you reach and maintain a low-risk status for as long as possible. Keeping up with your risk assessments can help reduce complications and improve your life expectancy with PAH.

PAH Risk Assessment Factors

Risk assessment for PAH includes many factors. Your doctor will consider characteristics of your health, such as your:

• Heart rate
• Blood pressure
• Comorbidities (coexisting medical conditions)

They’ll also review results from your physical exam, blood tests, cardiovascular exercise capacity tests, and cardiac imaging. In addition, certain demographic factors (personal characteristics), like being a male over age 60, are considered risk factors for PAH, according to the journal Global Cardiology Science and Practice.

Here’s a breakdown of some key measures for PAH risk assessment:

Heart Rate

How fast your heart beats during rest is one way doctors monitor PAH. A heart rate over 96 beats per minute can be a sign that PAH is worsening.

Blood Pressure

Blood pressure is measured with two numbers: systolic (the top number) and diastolic (the bottom number). For PAH, systolic blood pressure is especially important. A systolic blood pressure under 110 millimeters of mercury (mm Hg) may be a predictor of more severe disease.

Comorbidities

Having other medical conditions can make PAH more difficult to manage. Although some people with comorbidities are still considered low risk, this is less common.

In one study, 42 percent of people who were being treated for idiopathic PAH (PAH from an unknown cause) but had no comorbidities were considered low risk. However, that number dropped to 12 percent for people with one or two comorbidities and to just 3 percent for those with at least three comorbidities.

Other Test Results

Your healthcare provider may also look at several other important variables, such as signs of right heart failure, syncope (fainting), and your ability to exercise, as well as blood test results.

Ideally, people with PAH should have echocardiograms every three to six months to check the heart’s structure and function.

Although PAH is a lung disease, right heart failure is a major cause of death in people with PAH. Ideally, people with PAH should have echocardiograms every three to six months to check the heart’s structure and function. Your healthcare provider may also order cardiopulmonary exercise testing, which measures how your heart and lungs work during exercise and rest.

A helpful blood test for PAH checks for a biomarker called B-type natriuretic peptide (BNP). This chemical is made by the heart and rises when pressure in the lungs is too high. Levels between 50 and 199 nanograms per liter suggest low to intermediate risk, and levels between 200 and 800 nanograms per liter are intermediate to high risk.

Doctors may also test for N-terminal pro B-type natriuretic peptide (NT-proBNP), an inactive form of BNP that’s easier to measure in the blood. NT-proBNP levels below 1,100 nanograms per liter are linked with low risk, while values above 1,100 nanograms per liter are associated with higher risk.

Benefits of Risk Assessment in PAH

Risk assessment plays a key role in long-term PAH disease management. This tool can help you and your healthcare provider understand how your condition may change, set personalized treatment goals, and track how well your therapy is working. By identifying your risk level, you and your care team can take steps to improve both your quality of life and life expectancy with PAH. Risk assessments are also important if you’re being considered for a lung transplant, since they help determine who may benefit most from advanced treatment options.

Talk with your healthcare provider about doing regular risk assessments and what the results mean for your PAH care. Risk assessments are among the most powerful ways to stay in control of your health.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 57,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Do your healthcare providers talk to you about PAH risk assessment tools or your personal risk score? Share your experience in the comments below, or start a conversation by posting on your Activities page.