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Expert Insights in PAH Care
Expert Insights in PAH Care: Answers to Your Frequently Asked Questions

Released: December 26, 2025

Brian P. Rose
Brian P. Rose, PA-C, MPH, AACC
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Key Takeaways
  • Treatment must consider patients’ comorbidities to ensure PAH-directed therapy is not adversely affected.
  • Nonvasoreactive refers to patients with PAH who have a lack of response to acute vasoreactivity testing during right heart catheterization; these patients should not be treated with calcium channel blockers.
  • Older adults with PAH may remain highly functional and benefit meaningfully from oral PAH therapy.

One of the most rewarding parts of participating in a live symposium about modern pulmonary arterial hypertension (PAH) care is hearing the questions that surface when healthcare professionals (HCPs) actively translate the latest guidelines into their real-world practice. In the recent session titled “Leading With Impact: NPs and PAs at the Center of PAH Care Transformation,” which was held during the 2025 Cardiology Intensive for Advance Practice Providers, HCP questions reflected a growing sophistication in how PAH care is approached today. It is not as a single decision point but a longitudinal process that requires early suspicion, accurate classification, frequent reassessment, and thoughtful escalation. In this commentary, I address 6 frequently asked questions that highlight common areas of uncertainty in PAH care that, when clarified, can meaningfully improve patient outcomes.

Are there specific comorbidities that change how HCPs should treat patients with PAH?
Although PAH is considered a rare disease, it does, however, have a higher prevalence among patients with connective tissue disorders. The most common type seen in practice is idiopathic PAH.

It is important to note that patients with PAH have other comorbidities, such as coronary artery disease, chronic obstructive pulmonary disease, valvular heart disease, atrial fibrillation, sleep apnea, and/or diabetes, among many others. Although these comorbidities may not be the primary driver of patients’ PAH, failing to address them can blunt the benefit of their PAH-directed therapy. In parallel with guideline-directed treatment, HCPs must optimize any contributing conditions by treating comorbidities like sleep apnea, managing volume status, controlling ischemia, and coordinating care with rheumatology or hepatology when appropriate. Treating PAH in isolation rarely succeeds.

What does “nonvasoreactive” mean and why does it matter?
Nonvasoreactivity refers to the response—that is, a lack of response specifically—with acute vasoreactivity testing during right heart catheterization (RHC). Only a small subset of patients with PAH demonstrate true vasoreactivity and, therefore, qualify for treatment with calcium channel blockers. In practice, most patients are nonvasoreactive, meaning they do not meet criteria for favorable hemodynamic response to agents like inhaled nitric oxide, epoprostenol, or adenosine. For these patients, calcium channel blockers are not an appropriate therapy, and their treatment should follow guideline-directed combination therapy pathways. Understanding this distinction prevents patients from taking ineffective and potentially harmful therapy.

Is there an echocardiographic cutoff for right ventricular dysfunction that mandates RHC?
There is no single echocardiographic cutoff that universally applies to all patients with PAH; rather, their clinical context matters. If an echocardiogram was obtained because a patient is symptomatic, in particular with unexplained dyspnea, any evidence of right ventricular enlargement or dysfunction should raise concern and prompt further evaluation. In high-risk populations, such as those with connective tissue disease, I use a much lower threshold for RHC, even if echocardiographic findings are subtle. In converse, new or disproportionate right ventricular dysfunction in patients with known left-sided heart disease should prompt reconsideration of their underlying diagnosis. RHC remains essential for defining hemodynamics and avoiding misclassification.

How do you approach PAH treatment in older adults, including those in their 80s or 90s?
Age alone should not exclude patients from receiving necessary PAH treatment. What matters more is their functional status, symptom burden, and overall goals of care. I often say, “There’s 90, and then there’s 90.” Some patients in their 90s remain highly functional and may benefit meaningfully from oral PAH therapy. That said, treatment in older adults requires careful attention to polypharmacy, management of adverse events, and close follow-up. Treatment escalation decisions should be individualized, and expectations should be discussed openly. Transplant may not be a consideration for this patient population but symptom improvement and quality of life are.

When should pulmonary rehabilitation be started in patients with PAH?
Pulmonary rehabilitation is 1 of the most underutilized—yet valuable—components of PAH care. I have never regretted referring patients too early, but I have regretted waiting too long to do so. Pulmonary rehabilitation provides supervised exercise, education, and confidence building to patients who are often afraid to exert themselves. In most cases, a referral should occur early in patients’ treatment course, even as medical therapy is initiated or optimized. Furthermore, pulmonary rehabilitation is not a substitute for pharmacotherapy; it is a complementary treatment that improves patients’ functional capacity and quality of life.

How do you define treatment success once patients are on advanced or 4-pathway therapy?
At advanced stages of PAH, treatment success should no longer be defined by a single metric. Objective measures, such as 6-minute walk distance, NT-proBNP levels, and risk scores remain important, but they must be interpreted in context of how patients feel and function day to day. For those on 4-pathway therapy, disease stability can be a meaningful win. In addition, mild adverse events may be managed rather than prompting immediate treatment de-escalation, provided the overall benefit outweighs the burden. At this stage, shared decision-making is even more critical and should include honest conversations about expectations, long-term goals, and when to consider transplant evaluation or palliative care approaches.

Your Thoughts
PAH management is no longer centered on just choosing a single therapy for treatment; rather, it now requires continuously reassessing risk, responding early to change, and partnering closely with patients and multidisciplinary teams. The questions raised during the live symposium reflect how far pulmonology has come and how much opportunity remains to improve patient outcomes through thoughtful, individualized care.

Do you refer your patients with PAH to pulmonary rehabilitation early? You can get involved in the conversation by answering the poll question and posting a comment below.

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Do you refer your patients with PAH to pulmonary rehabilitation early?

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