Panelists discuss how managing patients with comorbidities requires careful evaluation to distinguish true Group 1 PAH features from underlying cardiac or pulmonary disease, with a more measured treatment approach and close monitoring for complications.
Prostacyclin Therapy Role and Challenges
Prostacyclin pathway utilization varies significantly between regions, with European practice differing substantially from US approaches due to different available formulations and clinical experience. European centers primarily use parenteral prostacyclins through fully implantable pumps rather than external infusion systems, while prostacyclin receptor agonist use remains limited compared to US practice. Clinical experience suggests some patients may be successfully transitioned from prostacyclins to sotatercept, though this requires careful monitoring.
Evidence supporting sotatercept efficacy compared to prostacyclin therapy creates potential for paradigm shifts in treatment approaches, with some centers beginning to use sotatercept before prostacyclin therapy or attempting to wean patients from prostacyclins after sotatercept initiation. However, concerns remain about long-term sotatercept safety data, and the strong historical evidence for prostacyclin therapy maintains its important role, particularly in right heart failure situations.
Prostacyclin therapy presents significant challenges including substantial side effects such as headache, nausea, diarrhea, flushing, and jaw pain, along with administration complexity for parenteral formulations. Continuous infusion therapy requires central venous access with associated infection risks, daily medication preparation, and lifestyle limitations. Patients often live with chronic symptoms like diarrhea and headaches, necessitating individualized risk-benefit assessments considering mortality risk, quality of life impact, and patient preferences when making treatment decisions.
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