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PAH Management
What Healthcare Professionals Need to Know About Diagnosing and Treating PAH

Released: March 27, 2024

Expiration: March 26, 2025

Corinne Young
Corinne Young, MSN, FNP-C, FCCP
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Key Takeaways
  • Diagnosing and treating PAH requires thorough attention and screening practices.
  • Risk assessment is crucial in determining initial treatment regimens and when to advance therapy.
  • Sotatercept shows promise in improving exercise tolerance for patients with PAH.

Pulmonary arterial hypertension (PAH) poses significant challenges in diagnosis and treatment, necessitating familiarity among all healthcare professionals (HCPs). Patients often present with nonspecific symptoms, such as dyspnea with exertion and fatigue, making diagnosis complex and often delayed. This ClinicalThought commentary, I focus on how HCPs can provide timely diagnosis and treatment of PAH. 

Screening and Diagnosis
Echocardiograms offer initial insights into PAH diagnosis, encompassing various parameters beyond estimated right ventricular systolic pressure. This preliminary examination is crucial in the diagnosis of PAH and other pulmonary diseases, but systematic evaluations including assessment of connective tissue disorders, left heart issues, pulmonary conditions, and sleep apnea are also used to identify possible underlying causes. Chronic thromboembolic pulmonary hypertension warrants special attention, and lung ventilation–perfusion scans are often recommended for screening, especially in those patients with history of thrombosis. Ultimately, right heart catheterization remains indispensable for diagnosing PAH, providing essential hemodynamic parameters and differentiation between pre– and post–capillary disease.

Risk Assessment in PAH
A diagnosis of group 1 PAH involves assessing patients’ risk through various parameters, including functional class, exercise tolerance, biomarkers, and hemodynamics. Risk scores, such as the 3-stratum or 4-stratum approach and the REVEAL risk calculator, aid in objective risk assessment. Many other risk scores exist, such as SPAHR, COMPERA, and French, but HCPs should be consistent in using the same calculator for each visit with their patients. Treatment decisions are guided by risk levels, with aggressive therapies for high-risk patients and dual oral combination therapy for intermediate-risk patients.

Management of PAH Treatment
Treatment aims to achieve a low-risk status and induce a favorable prognosis. Regular follow-up and reassessment of patients are crucial, involving biomarker tests, exercise tolerance assessments, and imaging studies. Escalation of therapy may be necessary for patients who do not respond adequately to initial treatment. Traditional first-line treatments include sildenafil or tadalafil, which are PDE5 inhibitors and are preferred as they are cost-effective options. Other therapies, such as macitentan—an endothelin receptor agonist—have been approved in previous years. On March 22, 2024, the FDA approved a single-drug combination therapy of macitentan and tadalafil for the treatment of chronic PAH. This approval was based on the phase III A DUE study, where the combination demonstrated a greater reduction in pulmonary vascular resistance than each therapy alone. Other guideline-recommended pathways for PAH treatment include nitric oxide and prostacyclin.

A Novel Treatment
On March 26, 2024, the FDA approved sotatercept, for treatment of PAH. Sotatercept is a first-in-class activin receptor type IIA-Fc fusion protein that acts as a ligand trap for certain TGF-β superfamily members. It is hypothesized that the inhibition of these ligands by sotatercept rebalances pulmonary vascular homeostasis in favor of proapoptotic and growth-inhibition signaling. Clinical trials, such as STELLAR (NCT04576988), have demonstrated the potential of sotatercept in improving exercise tolerance, suggesting its inclusion in the treatment arsenal for PAH as an add-on therapy.

Your thoughts?
How many patients do you treat with PAH per week? Are you aware of these new FDA approvals? Let us know in a comment below

 

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