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Demographics, Disease Characteristics, and Outcomes of Patients With Non-Group 1 Pulmonary Hypertension (PH) Enrolled in the PROSPECT Registry

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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.22.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Robert Frantz

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Epoprostenol for injection (Veletri®; Epo-A) is a prostacyclin analog approved to treat World Health Organization Group 1 pulmonary arterial hypertension (PAH). Use of Epo-A as treatment for non-Group 1 PH has not been evaluated clinically.

RationaleEpoprostenol for injection (Veletri®; Epo-A) is a prostacyclin analog approved to treat World Health Organization Group 1 pulmonary arterial hypertension (PAH). Use of Epo-A as treatment for non-Group 1 PH has not been evaluated clinically. PROSPECT is a prospective, 50-center, observational US registry to evaluate Epo-A use in routine practice. A small number of non-Group 1 patients who were receiving Epo-A at enrollment were registered in PROSPECT. In this analysis, we describe this cohort to gather insight into the use of Epo-A in non-Group 1 patients.

METHODS: Patients currently receiving or recently initiated on Epo-A were enrolled in PROSPECT. Patient data were collected quarterly for up to 1 year. Data download for this analysis occurred on May 21, 2013. Baseline demographics, disease characteristics, and patient disposition are reported.

Methods: Eighteen patients in PROSPECT were diagnosed with non-Group 1 PH (6 with sarcoidosis, 5 with chronic thromboembolic PH, and 7 each with a distinct diagnosis); 336 patients were diagnosed with Group 1 PAH. Mean mRAP were 15.6 and 11.9 mmHg and mean PCWP were 16.6 and 11.5 mmHg for non-Group 1 and Group 1 patients , respectively. Mean PVR were slightly higher for the non-Group 1 patients (11.2 Wood units) vs Group 1 patients (10.9 Wood units). Patients from both cohorts, 61% of non-Group 1 and 69% of Group 1, were receiving additional PAH medications (PDE5i, ERA or both). Median Epo-A doses at 1 month were 10.0 and 16.0 ng/kg/min for non-Group 1 and Group 1 patients, respectively, and 23.9 and 29.5 ng/kg/min at 12 months, respectively. There were 12 hospitalizations in the non-Group 1 cohort and 294 hospitalizations in the Group 1 cohort; the respective rates of hospitalizations were 2.04 and 2.78 per 1000 patient-days. Two non-Group 1 patients and 51 Group 1 patients died during the study.

Conclusions: Relative to the Group 1 cohort, the non-Group 1 cohort appeared to have worse hemodynamics at enrollment. Median Epo-A dose was lower in the non-Group 1 cohort than in the Group 1 cohort at 1 and 12 months. The non-Group 1 cohort had hospitalization and mortality rates similar to those of the Group 1 cohort. Our analysis suggests that usage of Epo-A in non-Group 1 patients is uncommon and is reserved for patients with severely compromised hemodynamics and/or right-heart failure for whom Epo-A use appears to be driven by Group 1-like hemodynamics. Our findings show that Epo-A treatment in the non-Group 1 cohort in PROSPECT, which included patients with a diverse set of diagnoses, resulted in outcomes that were similar to those observed for the Group 1 cohort.

Type: Clinical Science