Treatment of patients with pulmonary arterial hypertension at the time of death or deterioration to functional class IV: Insights from the REVEAL Registry
Section snippets
Study design
Study design and baseline characteristics of patients enrolled in REVEAL have been described previously.27, 28 Briefly, REVEAL is a 55-center (university-affiliated and community hospitals) observational, United States-based study.29 Patients are monitored for 5 years from the time of enrollment. Each participating center obtained Institutional Review Board approval before patient enrollment, and all patients provided informed consent. “Enrollment” was defined as the date consent was given.
Study population
Patient characteristics
At the data collection end point on September 15, 2011, 3,515 adult (aged ≥18 years) patients with PAH had been enrolled in REVEAL. Of these, 29% were newly diagnosed and 71% were previously diagnosed. Demographic characteristics of the PAH-related death, all-cause death, and FC worsening cohorts are reported in Table 1.
Discussion
These observations from REVEAL demonstrate that, despite current guideline recommendations, a substantial number of patients were not being treated as aggressively as guidelines suggest with intravenous prostacyclin and/or combination therapy at time of death and before or after being assessed as worsening to FC IV. A substantial number of patients were receiving only monotherapy at time of PAH-related death, and only 56% were receiving intravenous prostacyclin. The age-adjusted estimated
Disclosure statement
Assistance in manuscript development was provided by Jacqueline Lanoix, PhD, and Kathryn Leonard, of inScience Communications, Springer Healthcare. Ginny Lai, of ICON Late Phase & Outcomes Research, provided statistical programming support.
Funding and support for the REVEAL Registry were provided by Cotherix Inc, and its affiliate, Actelion Pharmaceuticals US Inc. Funding for preparation of this manuscript was provided by Actelion Pharmaceuticals US Inc.
H.W.F. serves as a consultant and is on
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2022, Archives of Medical ResearchCitation Excerpt :When initial therapy does not include a prostanoid and the patient's risk has not improved at follow-up assessment, the addition of a prostanoid to the combination is indicated. Nevertherless, the addition of prostanoids in general, and epoprostenol in particular, faces several barriers, as was shown in the REVEAL registry, in which intravenous prostacyclin was not used consistently in the more seriously ill patients enrolled in this registry: among patients who died, 56% in the PAH-related death cohort and and 43% in the all-cause death cohort, were receiving intravenous prostaciclin before death (20). Oral and inhaled prostanoids (treprostinil, selexipag, and iloprost) are also currently available.
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