Transitioning from continuous IV to inhaled prostacyclin using a cross-taper protocol was successful in 1 of 2 patients, with the other requiring protocol abortion due to worsening dyspnea.
Case Report (n=2)
Is it safe and feasible to transition clinically stable PAH patients from continuous intravenous to inhaled prostacyclin?
Transitioning stable PAH patients from continuous intravenous to inhaled prostacyclin using a cross-taper protocol is feasible but requires close monitoring as clinical worsening may necessitate protocol abortion.
Abstract Rationale Continuous intravenous (IV) prostacyclin therapy remains the cornerstone of treatment for advanced pulmonary arterial hypertension (PAH) but is limited by catheter-related complications, systemic side effects, and lifestyle restrictions. For patients with stable hemodynamics, transitioning from IV to inhaled prostacyclin in the outpatient setting, while maintained on a stable background regimen, may preserve clinical stability and reduce treatment burden. We describe early experience applying a structured transition protocol in patients with stable PAH. Methods Case 1: A 31 year old woman developed severe postpartum PAH following pre-eclampsia, complicated by right ventricular (RV) failure requiring extra-corporal membrane oxygenation (ECMO). She was initiated on IV epoprostenol, sildenafil, and ambrisentan. After stabilization and successful ECMO weaning, her course was complicated by catheter-related bacteremia, abscess, and osteomyelitis requiring line replacement. She was discharged on triple therapy. Follow-up echocardiography demonstrated improved RV function. Sotatercept was added, then a transition using a cross-taper protocol, reducing IV treprostinil from 20 to 3 ng/kg/min while titrating Yutrepia to 132.5 µg four times daily, was completed over one month. The final taper from 3 ng/kg/min IV epoprostenol to discontinuation was completed under invasive hemodynamic monitoring. Case 2: A 70-year-old woman with WHO Group 1 PAH secondary to fenfluramine-phentermine exposure, on chronic IV treprostinil, riociguat, and macitentan, was clinically stable after adding subcutaneous sotatercept. Transition from IV to inhaled treprostinil was initiated using a cross-taper protocol, reducing IV treprostinil from 34 to 18 ng/kg/min while titrating Yutrepia from 106 to 132.5 µg four times daily. She developed progressive dyspnea following a COVID-19 vaccination, prompting protocol abortion and re-escalation of IV treprostinil to 20 ng/kg/min, which restored symptomatic stability. Results In Case 1, transition was completed while maintaining symptomatic stability. Post-transition studies showed normalized pulmonary artery pressures and stable cardiac output, and normal RV function. In Case 2, symptoms worsened during tapering, necessitating protocol discontinuation, though no lasting hemodynamic compromise occurred. Both patients experienced transient prostacyclin-related side effects, including jaw pain, headache, and mild cough post-inhalation, which were self-limited and tolerable. Conclusions These cases demonstrate the potential success and challenges of transitioning clinically stable PAH patients from continuous IV prostacyclin to inhaled formulations. A structured, closely monitored taper can be safe in select patients under expert management but requires readiness to reverse course if symptoms or hemodynamics worsen. This pilot protocol will be refined and applied prospectively to additional patients, with outcomes and safety data reported in future analyses. This abstract is funded by: none
Hron et al. (Fri,) conducted a case report in Pulmonary Arterial Hypertension (n=2). Transition from intravenous to inhaled prostacyclin (Yutrepia) was evaluated on Clinical stability and successful transition. Transitioning from continuous IV to inhaled prostacyclin using a cross-taper protocol was successful in 1 of 2 patients, with the other requiring protocol abortion due to worsening dyspnea.