• Risk assessment for pulmonary arterial hypertension (PAH) helps monitor disease progression and response to therapy.
• Using personal health factors, test results, and predictive algorithms, risk assessment estimates the risk of death from PAH within one year.
• People with PAH should have risk assessments every three to six months.

Risk assessment is an important tool in the management of pulmonary arterial hypertension and can help doctors predict progression of the disease. Taking into account personal health factors and test results, doctors use predictive algorithms to estimate the risk of death within one year.

Risk assessment can help PAH specialists detect and treat clinical worsening of PAH, which predicts mortality risk. If you are living with PAH, having regular risk assessments is important for optimizing your PAH care.

In 2015, the European Society of Cardiology/European Respiratory Society (ESC/ERS) issued guidelines for risk assessment. ESC/ERS guidelines recommend that people with PAH have a risk assessment every three to six months. Risk profile results can help doctors determine if PAH is progressing, how well treatments are working, and whether the current treatment plan or combination therapy should be adjusted.

Approaches to PAH Risk Assessment

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

It’s important to note that some people with PAH have more significant risks of death from the condition. Some studies have shown that African Americans with PAH are at a significantly greater risk of death than white people who have PAH. Women and people who are Asian have also been shown to have higher risks of mortality from PAH, according to results published in the journal Advances in Pulmonary Hypertension. Regular risk assessments are especially important among subgroups with higher risks.

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 U.S.-based REVEAL registry (Registry To Evaluate Early and Long-Term PAH Disease Management), which was initiated in 2006, monitors pulmonary vascular disease progression in people who are being treated for PAH. From the REVEAL registry, a simplified PAH risk assessment score was designed to be used by doctors for repeated assessment. Various factors were found to significantly alter the one-year risk of mortality, 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 was initiated in 2002 and collects data on people with PAH throughout a three-year follow-up period. The FPHN registry is used to identify the best prognostic predictors of disease progression.

Three baseline factors at the time of diagnosis are used to predict survival outcome, including exercise testing:

• The six-minute walk test (6MWD) measures walk distance during a six-minute time period. The farther the distance, the longer the predicted survival.
• Cardiac output (cardiac index) is 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.
• The FPHN found that being male predicts poorer survival, although more recent research indicates that, compared with men, women with PAH may be at higher risk of mortality from PAH.

The FPHN registry equation was designed to predict survival from the time of diagnosis, rather than the REVEAL model’s continual assessment to monitor disease progression.

The goal of both models is to predict prognosis — the course a case of PAH is likely to take. Both models use an algorithm to compare PAH status against large registries of people who have been diagnosed with PAH. The value of these models has been validated to show their effectiveness as risk assessment tools in clinical practice for predicting PAH prognosis.

Understanding PAH Risk Categories

PAH risk is assessed as low, intermediate, or high, based on the chance of death within one year.

Low Risk

Cases of PAH are categorized as low risk if there is no sign of right heart failure and no current progression of PAH symptoms. Scores on the 6MWT 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 death 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 6MWT 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 death in one year is estimated at between 5 percent and 10 percent.

High Risk

People who fall into the high-risk category have notable signs of right heart failure and quickly progressing clinical symptoms of PAH. High risk is also linked to a score of less than one-tenth of a mile (165 meters) on the 6MWT and a classification of WHO-FC 4.

In high-risk PAH, the chance of death in one year is estimated to be higher than 10 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 stratification — another term for 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 more accurate risk stratification and better disease management.

During the COVID-19 pandemic, 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. You can read more about working with your doctor to get what you need for your PAH and discussing 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 48,000 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.