Conference: 2014 International PHA Conference and Scientific Sessions
Release Date: 06.22.2014
Presentation Type: Abstracts
File Download: 2014 Conference Abstract - Farber H
Download Adobe Acrobat
Purpose: Tailoring PAH therapy to match patient risk level may improve outcomes. Intensive therapy (ie, continuous intravenous prostacyclin) is recommended for high-risk patients. Epoprostenol for injection (Veletri®; Epo-A), a prostacyclin analog approved for World Health Organization Group 1 PAH, has established efficacy in patients in functional class (FC) III/IV. Here, we describe PROSPECT patients who transitioned from inhaled prostacyclin to Epo-A.
Methods: PROSPECT is a prospective, 50-center, observational US registry to assess Epo-A use in routine practice. Patient data were collected quarterly for ≤1 year. For this analysis, patients were grouped as “naïve” to all prostacyclin or “transitioned” from inhaled prostacyclin. Data were downloaded on September 23, 2013. Baseline demographics, disease characteristics, and clinical outcomes are reported descriptively.
Results: Of 97 patients in this analysis who were naïve to intravenous prostacyclins, 69 were naïve to all prostacyclin (but not to all PAH therapy) and 28 had transitioned from inhaled prostacyclin. At enrollment, 73.9% of patients naïve to prostacyclin (38 % IPAH, 23% CTD) and 96.4% of patients transitioned from inhaled prostacyclin (53% IPAH, 4% CTD) were in FC III/IV, although duration of PAH was shorter in the prostacyclin-naïve cohort. Other characteristic differences at enrollment between the naïve and transitioned cohorts were mean 6MWD (311 vs 274m), REVEAL risk score (9.1 vs 9.7) and mean BNP (511 vs 333 pg/mL), respectively. Mean mRAP, PVR and CI were similar between the two cohorts. During treatment, median Epo-A dose at 12 months was similar between the naïve and transitioned cohorts, 23 and 26 ng/kg/min, respectively. The hospitalization rate was higher (5.32 vs 3.16/1000 patient days) and the 12-month Kaplan-Meier survival estimate was lower (63.1% vs 83.2%) for patients transitioned from inhaled prostacyclin vs patients naïve to prostacyclin, respectively.
Conclusions: Patients in PROSPECT who transitioned from inhaled prostacyclin to Epo-A were high-risk patients based on REVEAL risk score, functional class, and 6MWD. During the one-year study period, they had exceptionally high morbidity and mortality, compared with a prostacyclin-naïve cohort. These findings suggest that patients on inhaled prostacyclins require close observation and should be transitioned to Epo-A before severe decompensation.