How PAH Risk Status and Treatment Guidelines Influence Your Treatment Plan Sponsored Post

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Two important components in determining a treatment plan for pulmonary arterial hypertension (PAH) are PAH risk status and PAH treatment guidelines. Whether you desire a more active lifestyle or a lessening of symptoms, your risk status can play an important role in achieving your goals. Knowing your risk status and understanding how it is used within treatment guidelines allows you to be more empowered when discussing your goals with your healthcare team, whether they be living longer, feeling better, and doing more.

What Is PAH Risk Assessment and Risk Status?

A PAH risk assessment gives your PAH specialist a comprehensive view of your PAH and helps your healthcare team assess how you are doing now and how you may do in the future. Your PAH specialist performs a risk assessment by using your test results from your visit, which are then scored and used to calculate your risk status.

Risk status can be low, intermediate, or high risk. Low risk means that there is a better chance of survival over the next five years, while intermediate and high risk means that there is a lower chance of survival over the next five years. Your risk status also helps your healthcare team determine how well your treatment plan is working and whether changes are needed.

To learn more about risk status, refer to our previous article, “PAH Risk Status – It May Change How You Think About the Future.”

How Do Risk Status and Treatment Guidelines Influence Treatment Options?

PAH treatment guidelines are used by cardiologists and pulmonologists who specialize in PAH when diagnosing and treating this rare condition. These guidelines are based on a culmination of research and issued by the World Symposium on Pulmonary Hypertension (WSPH), the European Society of Cardiology, the European Respiratory Society, and the American College of Chest Physicians.

The WSPH treatment guidelines use risk status to recommend how doctors should treat PAH patients. These guidelines recommend that newly diagnosed PAH patients at low or intermediate risk status should be treated with a combination of oral medications. For newly diagnosed PAH patients at high-risk status, an infused medication should be prescribed in their initial combination treatment plan. Initial oral monotherapy may be appropriate for some patients in specific situations, but guidelines recommend most patients should be treated with multiple medications. 1

The guidelines also recommend that a risk assessment be performed every three to six months to assess whether risk status has changed and how well a patient’s current treatment plan is working. If risk status is not at low risk after three to six months, treatment guidelines recommend the physician adjust their treatment plan, which often includes adding a medication.

Notably, treatment guidelines are focused on treating to low-risk status, not stability. Stability, although important, focuses only on how you have been doing in the past. Today, PAH experts focus on risk status because it goes further—it looks at how you’re doing now, how you can feel better today, and how you may be doing in the future.

Why Do Treatment Guidelines Recommend Multiple Medications or Adding a Therapy?

Research has shown that taking multiple PAH medications can keep patients healthier longer. One recent analysis of 17 studies involving more than 4,000 PAH patients found that patients taking multiple PAH medications had a 35% lower chance of getting worse than patients only taking one medication. The analysis also found that taking multiple medications improved patients’ 6-minute walk distance and WHO Functional Class, and reduced the risk of PAH-related hospital admissions.2

Another recent study further validated why many patients may need to add a medication or adjust their treatment plan over time. In the study of 181 newly diagnosed/untreated patients at PH centers, between 57-65% of these patients did not achieve or maintain low-risk status after six months on a combination of two oral medications.3 Studies such as these show that many PAH patients are candidates for treatment plan adjustments.

Risk Status and Treatment Guidelines Enable Deeper Conversations With Your Healthcare Team

Knowing your risk status and understanding how risk status and PAH treatment guidelines influence your treatment plan enables you to work more closely with your healthcare team to help get to low-risk status and determine if your current treatment plan is working.

To learn more about PAH, 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.


  1. Galiè N, McLaughlin VV, Rubin LJ, et al. An overview of the 6th World Symposium on Pulmonary Hypertension. Eur Respir J 2019; 53: 1802148 [].
  2. Lajoie AC, Lauzière G, Lega JC, et al. Combination therapy versus monotherapy for pulmonary arterial hypertension: a meta-analysis [published correction appears in Lancet Respir Med. 2016 Jun;4(6):e34]. Lancet Respir Med. 2016;4(4):291-305. doi:10.1016/S2213-2600(16)00027-8
  3. Badagliacca R, D’Alto M, Ghio S, et al. Risk Reduction and Hemodynamics with Initial Combination Therapy in Pulmonary Arterial Hypertension. Am J Respir Crit Care Med. 2021;203(4):484-492. doi:10.1164/rccm.202004-1006OC

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