Managing Your PAH

Risk status: Today’s approach to PAH

Achieving stability is an important milestone in PAH. Your symptoms may have leveled off, and you may feel better than you felt before your diagnosis.

Stability, although important, focuses on how you have been doing. Today, PAH experts frame your care around risk status. That’s because risk status goes further. It looks at how you’re doing now, how you can feel better today, and how you may be doing in the future. Studies show that patients who optimize their therapy early on and improve their risk status may have a better prognosis over the next 5 years.

Are you still having symptoms? Do you decline invitations or avoid making plans because you may not feel up to it? Knowing your risk status helps your healthcare provider understand how well your treatment is working—and whether there might be a way to help you feel better and do more. 


Managing your PAH and life expectancy image

Welcome back to the PAH Initiative video series. I’m Dr. Lana Melendres-Groves, a pulmonary arterial hypertension specialist and director of the Pulmonary Hypertension Program at the University of New Mexico. I have been treating pulmonary diseases for over 12 years, with a specialization in PAH for over 9 years. My clinic has treated over 5,000 patients, and I currently oversee 250 PAH patients on PAH-specific medicines. In this video, we’ll discuss the goals of treatment and plans to address those goals. If you haven’t seen the PAH Basics video, it’s better to watch that first. Goals are important to help us assess whether treatment is working well enough or if there’s need to make treatment changes. My PAH patients usually have their own personal goals in mind, and as a pulmonary arterial hypertension specialist, I have clinical goals for my patients as well. Fortunately, these goals work hand in hand because achieving clinical goals may help patients achieve their personal goals as well. Although the words are different, the important thing is that you and your doctor discuss your treatment goals and make sure you understand each other.

Personal goals for patients with PAH often include experiencing fewer symptoms such as fatigue and shortness of breath, spending less time in the hospital or not being hospitalized at all, being more active, or even spending more time with friends and family, and getting out more. Physician PAH treatment goals include improving right ventricular heart function, having healthier blood vessels in the lungs, and slowing disease progression. Let’s talk about how you and your doctor can work together to reach these goals. I want to start by familiarizing you with the phrase, risk status, which you might not have heard of before. Risk status is a different way of looking at your PAH. It involves some of the tests that your doctor’s already using to monitor your status. But it goes beyond just looking at how you’re doing today. A risk assessment uses the results to predict how your PAH may change over the next 5 years. PAH specialists now consider improving your risk status as an important treatment goal.

Risk status is either low risk, intermediate risk, or high risk. Low risk means that there’s a low chance of your PAH getting worse. If your level is intermediate or high risk, there’s a higher chance that your disease will get worse. When PAH disease gets worse, it’s referred to as disease progression. If your status is high or intermediate risk, your doctor may want to consider changes in your care by adjusting your treatment plan to help improve your risk status and lower the chance of your disease getting worse. Your doctor will look at a number of different factors to evaluate your risk status. Three commonly used assessments are the 6-minute walk test, NT-proBNP and BNP, which are blood tests, and Functional Class.

Let’s consider each of these assessments a little more closely. You may already be familiar with the 6-minute walk test. It is a test to see how far you can walk in 6 minutes. Performing a 6-minute walk test at each doctor’s visit provides an important view of your status over time. This helps your doctor assess whether your treatment might need adjustment. You might be interested to know that the average walking distance for patients with PAH in 6 minutes is approximately 350 meters. Patients considered low risk can walk more than 440 meters in the walk test. And the average distance for people who do not have PAH is between 590 and 640 meters.

Did you know that a meter is slightly longer than a yard? In other words, a 100-yard football field is a little over 91 meters long, so 440 meters is equal to a little less than 5 football fields. Being able to walk further during your test indicates that your heart, lungs, and blood vessels are functioning better than when you did not walk as far. Improving walk distances over time is a goal PAH doctors set for many of their patients. This is one part of the larger goal of trying to improve risk status.

Another test that’s considered in risk assessment is a blood test called NT-proBNP or BNP. BNP is a chemical the heart makes when it is strained, NT-proBNP is another form of this chemical. So it makes sense that there’s more BNP if the heart is under greater strain. Although no one test determines your risk status, an NT-proBNP greater than 300 nanograms per liter, and BNP greater than 15 nanograms per liter indicates that the heart may be working too hard.

Functional Class is another type of evaluation that can be used to help assess risk status and is an indicator of your capacity to physically function. How much activity you are able to do without experiencing symptoms determines your Functional Class. Class 1 means you have no symptoms with ordinary physical activity. Class 2 means you have symptoms that slightly limit your activity. Class 3 means that your symptoms significantly limit your activity. And if you have symptoms even while resting, that is considered a class 4. Functional Class 1 and 2 are associated with low-risk status. What I tell most of my patients who are high risk or intermediate risk, is that we should strive for low-risk status. In 3 to 6 months, we’ll see if we’ve gotten there, and if not, we will consider what else we can do to help them try to achieve their goal. Talking to your physician about your risk status is a starting point for setting goals and creating a treatment plan.

Once again, healthcare providers assess risk status using 6-minute walk test, BNP blood tests, and Functional Class, as well as other tests. Changes in your test results can show how your treatment is working and if your treatment needs to be adjusted. I’ll leave you with this tip, be proactive by making a list of your treatment goals to see if they match your healthcare provider’s goals. Ask your provider if lowering your risk status is one of your treatment goals and what more you can do to meet your goals. Thank you for watching PAH treatment goals. Watch the next video in the PAH Initiative video series to learn about working with your healthcare provider to learn about treatment options available today.

Understanding risk status 

In this PAH Treatment Goals video, Dr. Lana Melendres-Groves explains that healthcare providers use risk status to help set appropriate treatment goals.  

Testing to understand your health today

You are probably familiar with these evaluations, which can help you and your healthcare provider understand how you’re doing today.

6 minute walk test for PAH icon
6-minute walk test

Evaluates how far you can walk in 6 minutes

PAH WHO functional class icon
WHO Functional Class

Shows how your symptoms impact your day-to-day activities

BNP or nt-proBNP PAH icon
BNP or NT-proBNP

Measures a chemical in your blood that shows how much strain is on your heart

But what about the future?

Experts in PAH have looked at thousands of patients and thousands of data points to create a way to take your test results a step further.

Now, experts have discovered a method of combining many factors—including some of your test results—to see how you could feel better today and predict how you will be doing in the future. It’s called risk assessment.  

Among PAH experts, risk assessment is an established practice, and risk assessment has become part of the standard guidelines for treating PAH.

What happens during a risk assessment?

Treatment guidelines recommend that your healthcare provider perform a risk assessment for you every 3 to 6 months. Your healthcare provider performs a risk assessment by combining many of your evaluations.

Once the risk assessment is complete, your healthcare provider uses that information to determine your risk status. Risk status helps predict your survival over the next 5 years. When developing your treatment plan, your healthcare provider may try to improve your risk status.

PAH risk assessment and risk status

How is risk status related to life expectancy?

By knowing your risk status, healthcare providers are better able to predict survival over the next 3 to 5 years. Risk status can be low, intermediate, or high. As your risk status improves and moves toward “low,” your probability of survival increases. 


Low risk means there is a better chance of survival over the next 5 years


Intermediate and high risk mean there is a lower chance of survival over the next 5 years


Intermediate and high risk mean there is a lower chance of survival over the next 5 years

Deciding on the right goal for you

Individual goals will be different for everyone. You and your healthcare provider will decide if low risk is the right goal for you—and whether changes to your medication could help get you there.

If your risk status is low today, chances are you’ll be doing better over the next 5 years. Ask your healthcare provider what you can do to help improve your risk status.

Improving your risk status

The key to achieving low-risk status is reaching the low-risk goal on several tests. Achieving low risk on 1 or 2 tests might make you stable, but you still may not be at low risk. Talk to your healthcare provider about your tests results, what they say about your risk, and how you might be able to achieve more low-risk goals.

Improving your risk status can be a challenge, but it pays off. You are investing in yourself. When you improve your risk status and experience fewer symptoms, you can:

  • Feel better today
  • Do more in your day
  • Enjoy more “good days”
  • Look forward to the days ahead


Image of woman who is happy about her improved PAH risk status

What are your treatment options?

To help improve your risk status, your healthcare provider may adjust your PAH medication or add a medication.

Common tests used in risk assessment

You are probably undergoing tests at every appointment. Listed below are just some of the tests that PAH experts use to understand your risk status, how you are doing now, and how things may have changed since your last visit.

PAH 6 minute walk test stopwatch icon

6-minute walk test

During a risk assessment, your healthcare provider will measure how far you’re able to walk in 6 minutes. This 6-minute walk test gives clues about how your heart, lungs, and blood vessels are doing. In fact, studies show that out of all the tests your healthcare provider gives you, the 6-minute walk test is the best predictor of how you will be doing over the next 3 to 5 years.

6-minute walk test goals

Goals will vary for each person, and your risk status will affect your goal. The average 6-minute walk distance for a person with PAH is about 350 meters (about 380 yards). Your healthcare provider will work with you to set your appropriate goal and value.

6 minute walk test norms

Are your walk distances changing?

What’s important about your 6-minute walk distance is improvement—try to improve your walk distance. Your results on this test may indicate whether your treatment is working effectively or if an adjustment is needed.

image of improved 6 minute walk test distance

If you are able to walk farther than you did at your last appointment, your PAH may be getting better.

Image of unchanged 6 minute walk test distance

If your distance hasn’t changed, your PAH may not be improving.

Image of worsening 6 minute walk test distance

If you can’t walk as far as you walked at your last appointment, your PAH may be getting worse.

WHO functional class for PAH icon

WHO Functional Class

The World Health Organization (WHO) developed a system to place people with PAH into 1 of 4 Functional Classes. Functional Class is based on the symptoms you experience when doing everyday activities. 

Functional Class 1 and Functional Class 2 are associated with lower risk status, whereas Functional Class 3 and Functional Class 4 are associated with higher risk status.

Functional Class 1

Low Risk

Functional Class 2

Low Risk

Functional Class 3

Intermediate Risk

Functional Class 4

High Risk

Functional Class is a strong predictor of life expectancy, and it’s an important factor that your healthcare provider uses when choosing your PAH treatments.

Which classification are you? Your healthcare provider will help you determine your WHO Functional Class. See the questions below to learn more about how each Functional Class is defined.

Choose yes or no for each question

Can you ride a bike or go for a long walk without getting breathless?

WHO Functional Class 1

You experience no symptoms at rest or with ordinary physical activity, such as biking or walking.

Do you need to pause to catch your breath when you climb stairs or go shopping?

WHO Functional Class 2

You are comfortable at rest but may feel symptoms or have slight limitations with activity.

Can you walk to the end of the driveway without stopping to rest?

WHO Functional Class 3

You notice a marked limitation of activity. You feel symptoms even with light activity.

Do you experience symptoms even when resting?

WHO Functional Class 4

You experience symptoms even when you are resting or with any activity.  

BNP or nt-proBNP PAH icon

NT-proBNP/BNP blood test

When your heart works harder than usual, it releases high levels of molecules found naturally in your body. These include the hormone called B-type natriuretic peptide, or BNP, and its precursor, NT-proBNP.

If this test shows high levels of NT-proBNP/BNP, it means your heart is straining and working harder than normal. The strain may be caused by narrow blood vessels in the lungs because of PAH.

Based on guidelines, your NT-proBNP/BNP results can be categorized as follows:

Low Risk

NT-proBNP <300 ng/L

BNP <50 ng/L

Intermediate Risk

NT-proBNP 300-1400 ng/L

BNP 50-300 ng/L

High Risk

NT-proBNP >1400 ng/L

BNP >300 ng/L

PAH knowledge is PAH power

True or false? Risk assessment is a method of combining many factors—including your test results—to predict how you will be doing over the next 3 to 5 years.

How many meters can an average person with PAH walk in the 6-minute walk test?

Risk status: What can you do?

PAH is a chronic condition. There is no cure today, but there are steps you can take to help you feel better today and possibly improve your risk status.

Have you found the right healthcare provider for you?  

PAH is a complex disease, it requires a  healthcare provider who has experience with treating PAH. You can find one here. These experienced providers are committed to treating PAH.

Are you asking your healthcare provider about exploring all treatment options?

By keeping your healthcare provider informed about how you’re feeling and what your goals are, you can work together to determine how to treat your PAH—through multiple treatment pathways. 

Have you started keeping a symptom journal?

Write down your symptoms every day in a journal—on paper, on your tablet, laptop, or phone. Bring it with you to your next appointment with your healthcare provider. Knowing your symptoms and details about how you’ve been feeling helps your healthcare provider determine which treatments are right for you.

What small changes have you made to your daily routine and habits?

-Eat a healthy diet

-Use less salt on your food 


-Watch your weight

-Stay informed about the latest PAH treatments

-Know all your medicines and their potential side effects

-Get rest and relieve stress

How is PAH treated today?

PAH is going to slow you down, but you can adapt and move forward. And the right treatment can help you manage your symptoms so you can pursue your goals.