Abstract Pulmonary arterial hypertension (PAH), particularly group 1, is managed with prostacyclin analogs like epoprostenol, delivered intravenously (IV). However, complications of IV therapy, including infections, catheter issues, and the risk of rapid decompensation during interruptions, highlight the need for oral alternatives. Treprostinil, a prostacyclin analog with a longer half-life and more stable formulation, offers oral (PO) and inhaled options. While manufacturer guidelines exist for IV-to-IV or IV-to-PO treprostinil transitions, no specific protocol for converting from IV epoprostenol to PO treprostinil is available. A 48-year-old female with PAH and systemic lupus erythematosus with thrombocytopenia presented for outpatient consultation due to complications related to her Hickman line, used for IV epoprostenol. Diagnosed with PAH in 2004, her treatment history included multiple transitions between IV epoprostenol and oral agents like sildenafil, tadalafil, and selexipag due to worsening hemodynamics. Initial treatment around the time of her diagnosis was limited due to her inability tolerate up titration of PO treprostinil due to significant headache and diarrhea. At the time of consultation, the patient was on IV epoprostenol, sildenafil, and ambrisentan. A dislodged Hickman line prompted hospitalization for conversion from IV epoprostenol to PO treprostinil. Upon admission, a peripherally inserted central catheter (PICC) was placed due to extensive venous thrombosis. The transition plan was initiated with careful monitoring by her pulmonologist and a pulmonary hypertension pharmacy specialist. The conversion followed a titration process over four days, starting with a decrease in IV epoprostenol and gradual increases in PO treprostinil. The final PO dose was 2.5 mg TID, with sildenafil and macitentan resumed. The patient tolerated the transition well and was discharged on PO treprostinil, sildenafil, and macitentan. Six months post-conversion, her clinical status improved, including a significant increase in her six-minute walk test from 100m to 450m, a drop in NT-proBNP from 387 pg/mL to 51, and reduced right atrial and ventricular dilation. The patient did develop headaches with reinitiation of PO medication however this was resolved with conservative management. The patient may also benefit from sotatercept, however continues to have thrombocytopenia secondary to lupus. This case contributes to the limited literature on IV epoprostenol to PO treprostinil conversion, offering a longer, more cautious titration approach than previously reported. Transitioning from IV to PO therapy in PAH patients can provide a safer, more convenient treatment option, eliminating risks associated with continuous infusion. Further studies are needed to develop standardized protocols for such transitions to optimize patient outcomes. This abstract is funded by: None
Shah et al. (Fri,) studied this question.
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