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Risk assessment is an important tool in the management of pulmonary arterial hypertension (PAH). 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.
The 2015 European Society of Cardiology/European Respiratory Society guidelines recommend people with PAH have a risk assessment every three to six months. Results can help doctors determine whether PAH is progressing, how well treatments are working, and whether the current treatment plan should be changed.
Risk assessment incorporates many factors. Your doctor will consider your personal health characteristics, such as age, heart rate, and blood pressure, along with the results of a physical exam, blood tests, exercise capacity tests, and cardiac imaging.
Read more about how risk is assessed in PAH.
Two major registries have been developed to interpret these measurements and determine the stability of PAH cases.
The U.S.-based REVEAL registry was initiated in 2006 and monitors 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 one-year risk of mortality, including:
The FPHN registry was initiated in 2002 and collects data on people with PAH throughout a three-year follow-up period. The FPHN registry was used to identify the best prognostic predictors of disease progression.
Three baseline factors may be measured at the time of diagnosis to predict survival outcome:
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 a tool for predicting PAH prognosis.
Risk is assessed as low, intermediate, or high, based on the chance of death within one year.
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 class 1 or 2.
In low-risk PAH, the chance for death in one year is estimated to be lower than 5 percent.
Like 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 for death in one year is estimated at between 5 percent and 10 percent.
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 for death in one year is estimated to be higher than 10 percent.
Overall, the goal for PAH treatment is to reach and maintain low-risk status for a long period of time.
Risk assessment helps doctors predict PAH progression, set treatment goals, and monitor response to therapy for long-term PAH disease management. Risk stratification 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 about 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. Read more about risk assessment via telehealth.
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