• Risk assessment for pulmonary arterial hypertension (PAH) helps doctors monitor how your disease progresses and your response to treatment.
• Using personal health factors, PAH symptoms, and test results, PAH risk assessment helps estimate life expectancy for people with PAH.
• People with PAH should have risk assessments at diagnosis and follow-up appointments for their PAH care.

Risk assessment is an important tool in the management of pulmonary arterial hypertension and can help doctors predict the worsening, or progression, of disease. For a risk assessment, doctors consider personal health factors and test results to estimate your prognosis (outlook) with PAH.

Risk assessment can help PAH specialists detect and treat the worsening of PAH. If you’re living with PAH, having regular risk assessments is important for making sure you get the care you need.

The European Society of Cardiology (ESC) and European Respiratory Society (ERS) set guidelines for how conditions like PAH should be evaluated and treated. In 2022, the two groups issued updated guidelines. The ESC/ERS guidelines recommend that people with PAH undergo a risk assessment at diagnosis and follow-up appointments (about every three to six months).

The results of these pulmonary hypertension guidelines provide a risk profile that can help doctors track your progression of PAH. Your risk profile is personal to you and your case of PAH. This profile can also help doctors determine how well treatments are working and whether the current treatment plan or combination therapy should be adjusted.

Approaches to PAH Risk Assessment

Risk assessment for PAH incorporates many factors. Your doctor will consider your health characteristics. These include your age, heart rate, blood pressure, and comorbidities (coinciding medical conditions), along with the results of a physical exam, blood tests, cardiovascular exercise capacity tests, and cardiac imaging.

Two major registries were developed to interpret risk measurements and determine the stability of PAH cases through risk scores.

Registry To Evaluate Early and Long-Term PAH Disease Management

The Reveal registry (Registry To Evaluate Early and Long-Term PAH Disease Management) began in 2006 and is based in the United States. It monitors the progression of pulmonary vascular disease in people being treated for PAH.

The Reveal registry generates a simplified PAH risk assessment score that doctors can use to see how a person’s disease changes over time. Various factors were found to significantly alter the estimated life expectancy with PAH, including:

• Age
• Sex
• Brain natriuretic peptide levels
• Blood test results
• Echocardiography results
• World Health Organization functional class (WHO-FC)
• Pulmonary function tests
• Atrial pressure and left ventricular dysfunction

French Pulmonary Hypertension Network Registry

The French Pulmonary Hypertension Network (FPHN) registry began in 2002. It collects data on people with PAH over a three-year follow-up period. The FPHN registry can help identify the best ways to predict how PAH progresses.

Three factors at the time of a PAH diagnosis are used to predict life expectancy with PAH:

• The six-minute walk distance (6MWD) test — Sometimes called exercise testing, this measures walk distance during a six-minute time period. The farther the distance, the longer the predicted length of time one will live with PAH.
• Cardiac output (cardiac index) — A measure of the amount of blood the heart pumps in a minute. Higher cardiac output at the time of diagnosis is associated with longer PAH survival.
• Sex — The FPHN found that being male predicts poorer survival. However, more recent research indicates that, compared with men, women with PAH may be at higher risk of dying (risk of mortality) from PAH.

The FPHN registry equation was designed to predict life expectancy with PAH from the time of diagnosis, whereas the Reveal model continually measures PAH disease progression.

The goal of both models is to predict prognosis — the course a case of PAH is likely to take. They also try to figure out which factors have the most impact on how the disease progresses.

Both models use a formula to compare PAH risk status to large groups of people who’ve been diagnosed with PAH. The value of these models has been scientifically validated. They’re effective tools in clinical practice for predicting PAH outlook.

Understanding PAH Risk Categories

PAH risk is assessed as low, intermediate, or high, based on the estimate of a person’s chance of dying within one year. Your PAH risk category may change based on the results of tests designed to figure out how PAH is affecting your body.

Low Risk

Cases of PAH are categorized as low risk if there are no signs of right heart failure and no current worsening of PAH symptoms. Scores on the 6MWD test are farther than a quarter mile, or about 440 meters. The WHO-FCs associated with low-risk PAH are classes 1 and 2. In low-risk PAH, the chance of dying in one year is estimated to be lower than 5 percent.

Intermediate Risk

Like those in the low-risk category, people who fall into the intermediate-risk range have no signs of right heart failure. However, there may be some signs of slow disease progression. Scores on the 6MWD test typically range from one-tenth to one-quarter mile (165 to 440 meters). Intermediate risk is classified as WHO-FC 3. In intermediate PAH, the chance of dying in one year is estimated at between 5 percent and 20 percent.

High Risk

People who fall into the high-risk category have notable signs of right heart failure and quickly worsening symptoms of PAH. High risk is also linked to a score of less than one-tenth of a mile (165 meters) on the 6MWD test and a classification of WHO-FC 4. In high-risk PAH, the chance of dying in one year is estimated to be higher than 20 percent.

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

Benefits of Risk Assessment in PAH

Risk assessment helps doctors predict PAH progression, set treatment goals, and monitor response to therapy for long-term PAH disease management. Risk assessment is also used in listings for lung transplantation.

Many types of testing and various factors go into a doctor’s risk assessment for PAH. Understanding what your doctor may ask you can help you provide clear information, which could lead to better management of your symptoms and disease.

Sometimes, it may be more difficult to see your PAH specialist in person. Remote risk assessment using online doctors’ visits can be an effective way to receive vital monitoring for your PAH. Work with your doctor to get what you need for your PAH, and discuss your risk assessment options with your pulmonologist.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 54,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Have you had regular risk assessments for your PAH? Have you had a remote risk assessment via telemedicine? Share your experience in the comments below, or start a conversation by posting on your Activities page.