Important Clinical Considerations for Pulmonary Arterial Hypertension

EP: 1.Pathophysiology of Pulmonary Arterial Hypertension

EP: 2.Differential Diagnosis of Pulmonary Arterial Hypertension

EP: 3.Importance of Early Detection and Monitoring for Disease Progression

EP: 4.Risk Stratification and the Importance of Patient-Centered Care

EP: 5.Strengths and Limitations of Current Therapeutic Options

EP: 6.Overview of Different Therapies for Patients with Pulmonary Arterial Hypertension

EP: 7.Sotatercept for Patients with Pulmonary Arterial Hypertension

EP: 8. The Role of the Prostacyclin Pathway

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EP: 9.Important Clinical Considerations for Pulmonary Arterial Hypertension

EP: 10.Economic Impact of Pulmonary Arterial Hypertension

Clinical Trial Evidence and Treatment Evolution

The STELLAR study demonstrated sotatercept's efficacy in a mature patient population with an average disease duration of nine years, heavily pretreated with 2-3 agents and 40% receiving infusion therapy. Despite this advanced disease state, sotatercept addition showed dramatic improvements in exercise capacity, symptoms, NT-proBNP levels, and hemodynamics, with mean pressure reductions not seen since early prostacyclin analog trials. Clinical experience has largely recapitulated these trial results with generally good tolerability.

The HYPERION trial examined early sotatercept use in newly diagnosed PAH patients within the first year, but was stopped early due to overwhelmingly positive results from another trial that eliminated equipoise for continuing placebo-controlled studies in this fatal disease. The ZENITH trial specifically targeted high-risk patients with REVEAL scores ≥9, a population typically excluded from clinical trials due to severe illness and potential inability to tolerate investigational agents.

ZENITH results showed sotatercept reduced clinical worsening by 76% in high-risk patients, using a high bar endpoint of death, transplantation, or hospitalization rather than softer disease progression measures used in other studies. This evidence supports sotatercept use across the PAH risk spectrum, from newly diagnosed patients to those with advanced disease, potentially changing treatment paradigms and timing of therapeutic interventions.