Assessing risk is a key step in the treatment and management of pulmonary arterial hypertension (PAH). It’s a process in which your doctor will gather information about your PAH and overall health. Risk assessment helps doctors figure out how severe your PAH is and how it’s progressing. If you’re living with PAH, it’s helpful to be familiar with the different risk assessment factors. Knowing what to expect at your doctor visits can help you better understand your disease and how to manage it.

Doctors typically conduct a risk assessment for people with PAH during or soon after their initial PAH diagnosis. They organize PAH into risk categories:

• Low-risk PAH
• Intermediate-risk PAH
• High-risk PAH

These categories help doctors make a prognosis (prediction of how your PAH will go), estimate how long you may live with PAH, and discuss appropriate treatment goals. If you have PAH, your doctor may have told you that your PAH is low, intermediate, or high risk.

At follow-up appointments, doctors use risk assessments to keep track of how your PAH is doing and how your symptoms have changed. They also use risk assessments to understand if your treatments are working and if you need different or additional therapies.

If you or a loved one is living with PAH, this article can help you better understand risk assessment factors. Your doctor may perform other tests and exams in addition to those discussed in this article. Let’s dive into six facts to know about PAH risk assessment.

1. People With PAH Should Receive a Risk Assessment Every Three to Six Months

Risk assessment for PAH should be conducted regularly — every three to six months at follow-up appointments. Ask your doctor how often they recommend you receive a risk assessment.

2. Many Factors Are Important for a PAH Risk Assessment

During a PAH risk assessment, doctors will make note of your sex, age, and type of PAH. Certain factors are associated with a poorer outlook with PAH, like being male and over 60 years old.

The type of PAH you have is essential in a risk assessment. If doctors don’t know the type of PAH you have, they may take a medical history or recommend genetic testing to determine it.

There are four types of PAH:

• Idiopathic PAH — Of an unknown cause
• Heritable PAH — Also called genetic or familial PAH
• Drug- and toxin-induced PAH — Caused by drugs like aminorex, fenfluramine, dasatinib, and methamphetamines
• Associated PAH — PAH related to another condition, including connective tissue disease, congenital heart disease, HIV infection, portal hypertension, or schistosomiasis

Familial PAH and PAH associated with systemic sclerosis or systemic connective tissue disease may be more complicated than idiopathic PAH. Your doctor will need to know the type of PAH you have to decide on the best treatment.

3. Doctors Use Exercise Tests To Measure Heart and Lung Health With PAH

Doctors measure exercise capacity, or your ability to be physically active for a given period of time. Exercise tests are one way to understand how severe someone’s PAH may be and how it may affect their daily lives. Exercise capacity is commonly assessed through the six-minute walk test (6MWT) and the cardiopulmonary exercise test (CPET).

The 6MWT measures your six-minute walk distance, or how far you can walk in six minutes. Changes in your 6MWT may be used to help make PAH therapy decisions. The CPET is a more in-depth measure of exercise capacity. If you’re unable to walk a distance within the expected range during the 6MWT, it could indicate a more severe case of PAH.

4. Tests That Assess Heart Size and Function Help Monitor PAH Disease Progression

Doctors will run tests to measure the size and function of your heart as a part of your PAH risk assessment. Common procedures include:

• Echocardiogram
• Cardiac MRI
• Right heart catheterization

An echocardiogram is a tool that produces an image of the heart. In PAH risk assessment, doctors use echocardiography to measure right ventricular function, size, and strain. A doctor might specifically check the size of the right atrium and test for fluid buildup around the heart, as these factors are associated with high-risk PAH.

Cardiac MRI is increasingly being used to generate images of the heart in people with PAH. MRI scans can provide clearer pictures of the right ventricle, potentially offering better-quality images.

Right heart catheterization is used to diagnose PAH and for follow-up risk assessment. It’s a procedure in which a small catheter (a thin, flexible tube) is inserted in a vein in the neck, groin, or arm. A doctor guides the catheter to the right side of the heart and the pulmonary arteries to measure the functioning and pressure in the heart and lungs. This procedure helps doctors measure factors like:

• Right atrial pressure — The amount of pressure in the upper-right chamber of the heart
• Pulmonary vascular resistance — Measures how tough it is for blood to flow through the blood vessels in the lungs, giving a sense of how hard the right side of the heart is working
• Venous oxygen saturation — Tells how much oxygen is in the blood returning to the heart from the body, indicating how well oxygen is being delivered to the body’s tissues
• Cardiac index — Measures how effectively the heart is pumping blood to the body, considering a person’s size and giving a more accurate picture of heart function than just looking at cardiac output alone

5. Brain Natriuretic Peptide Is an Important Blood Marker for PAH Risk Assessment

Another factor that doctors look at when assessing your risk with PAH is your levels of brain natriuretic peptide (BNP), a protein in your blood. Your heart makes more BNP when it has to work harder to pump blood. If you have high BNP levels, this could be a sign of heart failure.

People with a BNP of less than 50 nanograms per liter (ng/L) are considered lower risk, whereas people with a BNP above 300 are considered higher risk.

Another form of BNP is called N-terminal proBNP. Doctors may also measure your levels of N-terminal proBNP. Levels below 300 ng/L are considered lower risk, and those above 1400 ng/L are considered higher risk.

Your doctor will likely monitor your BNP and N-terminal proBNP levels over time with regular blood tests.

6. You Should Look Out for These Worrisome Symptoms

It’s very important to tell doctors about symptoms like shortness of breath, fatigue, chest pain, and fainting when you have PAH. These symptoms help them understand your heart and lung health and how your PAH affects you. Changes in these symptoms can help your doctor understand if your PAH is getting better or worse and how well medications are working.

In PAH risk assessment, heart- and lung-related symptoms help doctors understand your functional class. PAH functional class is a tool developed by the New York Heart Association and World Health Organization to predict the severity of PAH. Called the WHO FC for short, this tool assigns PAH to classes 1 through 4 — class 1 indicates less severe PAH, and class 4 indicates more severe PAH.

The number of hospitalizations you’ve had in the past six months or year may also help your doctor figure out your PAH risk. Hospitalizations, even if they were not related to your PAH, can be a sign of worsening PAH.

Connect With Others Who Understand

It’s easy to feel stressed and alone when living with pulmonary hypertension, but it may be easier with the support of others who understand. On myPHteam, the social network for those who live with PH and PAH, more than 53,000 members come together to ask questions, give advice, and share their experiences of life with pulmonary hypertension and pulmonary arterial hypertension.

Have you had a risk assessment for PAH? What advice do you have for others during this process? Share your experience and tips in the comments below or by posting on your Activities page.