Individualizing PAH Therapy in an Evolving Treatment Landscape - Episode 10
Stepping Down From Parenteral Therapy in PAH: Oral Prostacyclin Pathway Options
Patients want their pumps removed, but safety comes first. The panel discusses selection criteria, a rapid parenteral-to-oral transition experience, and practical dosing of oral prostacyclin pathway therapy.
"Stepping Down From Parenteral Therapy in PAH: Oral Prostacyclin Pathway Options" addresses a request many clinicians hear, from patients who want their pumps removed.
Dr. McLaughlin asks how to select patients on parenteral prostacyclin who ask to come off their pump after hearing about newer therapies. Dr. Vaidya says clinicians want to honor quality-of-life requests, such as a patient who misses swimming because of her subcutaneous pump, but must do so safely. She first ensures the patient is at the lowest risk status possible, compensated, free of recurrent right heart failure hospitalizations, and optimized in right heart function, jugular venous pressure, and BNP.
Dr. Vaidya wants a comprehensive objective assessment before any change, including invasive hemodynamics, echo, and biomarkers. In some cases he may uptitrate the parenteral dose and move the patient to an equivalent oral dose before switching. She also describes a recent University of Cincinnati publication of about 35 patients, roughly half with connective tissue disease. They moved from parenteral prostacyclin to oral selexipag over 36 hours during a three-day admission. Most were high risk by noninvasive parameters but had been catheterized beforehand. Most reached only modest selexipag doses, 91% were alive at one year, and pulmonary vascular resistance stayed in the 3-to-4 range. Dr. McLaughlin agrees these patients must be highly selected and supported by objective data.
She then asks whether oral or inhaled prostacyclin pathway agents might spare patients a pump. Dr. Kim cites the GRIPHON trial and its ten-year open-label follow-up, which shows preserved efficacy and tolerability. He cautions that oral agents need careful titration and some patients discontinue, but he emphasizes that patients need not reach the highest dose. Almost a quarter in GRIPHON were on the two lowest doses with an equivalent primary endpoint. He tells patients and specialty pharmacy that titration is not a race and should target the maximally tolerated dose.
Our next episode, "PAH Medication Side Effects: Tolerability, Titration, and Shared Decision-Making," covers side effects and how they shape shared decision-making.
