How Knowing Your PAH Risk Status Helps Put You and Your Doctor in Control

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by United Therapeutics |

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Do you know your PAH risk status? Guidelines recommend that doctors check your risk status every 3-6 months when you have PAH because it can give you insights into your treatment progress today while also helping to predict your future. Monitoring your risk status over time can provide important information on how your treatments and lifestyle changes are working or whether additional changes are needed. Your risk status combines your results from several tests, as there is no one test or data point that can provide you or your doctor with complete information on your risk status.

You may have some control over your risk status
Importantly, some of the factors that contribute to your risk are modifiable, whereas others are not. For example, age and sex are often considered when assessing risk status even though we do not control our age or sex. Other factors, however, such as exercise capacity (as measured by 6-minute walk distance), also provide information on PAH risk and can improve with effective treatment.

Finding the most effective treatment to help you keep improving is another element you and your doctor can control. Because research has shown that low-risk status is associated with higher survival rates and that patients can reduce their risk, it is important that you understand how PAH risk works and collaborate with your doctor to reduce your risk as much as possible.

Tools validated in clinical studies can help you and your doctor understand your risk status and how your risk changes over time
Clinical studies have identified several validated approaches that doctors use to assess risk in patients with PAH, including the U.S. Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk score and the European Society of Cardiology / European Respiratory Society (ESC/ERS) guidelines risk assessment table. These tools were developed based on large studies of patients with PAH. Certain doctors may prefer one approach over another, but they work in similar ways, determining risk status by incorporating PAH-relevant variables that represent your symptoms, biomarkers, functional capacity, and cardiovascular function.

Lowering your risk improves outcomes
Regardless of which of these approaches your doctor uses to assess your risk status, recent research has confirmed that patients who lower their risk – as measured with either of these approaches – have better outcomes than PAH patients who do not improve their risk. People with PAH who improve their risk status have higher 5-year survival rates. It should comfort and motivate PAH patients to know that one study showed those who were once considered intermediate risk or high risk who improved to the low-risk status at their next follow-up appointment (within one year) had 5-year survival outcomes that are comparable to those who were initially categorized as low-risk. Low-risk status is also associated with fewer PAH symptoms and the ability to do more daily activities for longer periods of time.

Common attributes of low-risk patients
No two PAH patients are the same, and people who are considered low-risk may look very different from each other. Low-risk status stems from a doctor’s assessment using multiple test results that were shown in clinical studies to correspond with higher 5-year survival. However, there are some trends that tend to be observed across low-risk patients.

Patients who are low-risk, for example, tend to have no clinical signs of right heart failure. They also tend not to experience a progression or worsening of their symptoms. Imaging tests usually show that low-risk patients do not have excess fluid between their hearts and the sacs surrounding their hearts. Low-risk patients also have lower levels of substances in their systems that would indicate that the heart is strained. As mentioned above, low-risk patients also tend to be able to exercise better and perform daily activities more easily than patients who are at higher risk.

It’s your move
With effective treatment and lifestyle changes lowering PAH risk is a goal for many PAH patients. People with PAH who improve their risk status enjoy better outcomes in terms of survival. They also tend to feel better and are more capable of carrying out activities they enjoy. For these reasons, a primary goal of PAH treatment is often lowering risk status.

By working with your doctor to understand your risk status, you can decide if low-risk is the right goal for you and determine the best strategies for achieving or maintaining a low-risk status. Ask your doctor about your risk status at your next appointment.

To learn more about risk status and how it could change your future, visit

This article was sponsored by the PAH Initiative, where knowledge meets inspiration. The PAH Initiative, sponsored by United Therapeutics, is dedicated to advancing patient care in pulmonary arterial hypertension.


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