PURPOSE: Historically, there has been a significant delay between the onset of symptoms and the diagnosis of pulmonary arterial hypertension (PAH). The REVEAL Registry is a multicenter, observational, U.S.-based study that is designed to meet the need for current and reliable information about the clinical course and management of PAH. One objective of the registry is to characterize the medical histories of enrolled patients.
METHODS: All consenting patients with PAH diagnosed according to specific hemodynamic criteria are being enrolled at 50 sites in the US. Patients will be followed for a minimum of five years from the time of enrollment, and various demographic data are being gathered at the time of enrollment, including medical history, co-morbidities, and time to diagnosis of PAH.
RESULTS: Over half of the 1226 patients (60%) enrolled in the study have 2 or more comorbid conditions (hypertension, 32%; hypothyroidism, 16%; scleroderma, 15%; clinical depression, 14%; and diabetes, 13%). The most common initial symptoms attributable to PAH include dyspnea on exertion (81%), fatigue (28%), chest pain/discomfort (20%), and edema (20%). The following median times (25 percentile, 75 percentile) are reported for the time from the initial symptoms to: presentation to physician for evaluation, 1 (0, 9) months; patient informed they had PAH, 7 (1, 23) months; diagnostic right heart catheterization (RHC), 13 (4, 36) months; and first visit to PAH clinic, 15 (6, 41) months.
CONCLUSION: For the patients enrolled in this registry, there was still a significant delay in the time between first symptoms to the diagnosis of PAH (RHC), with most patients experiencing a one-year delay. However, a quarter of the patients experienced an even longer delay that approaches 3 years. This result is only generalizable to patients currently being treated for PAH.
CLINICAL IMPLICATIONS: With the number of FDA-approved agents for treating PAH increasing yearly, shortening the time to start of treatment might have a significant impact on patient outcomes.
DISCLOSURE: C. Gregory Elliott, No Product/Research Disclosure Information; Grant monies (from industry related sources) Adaani Frost has received research grants and unrestricted educational grants from Actelion and Cotherix; Consultant fee, speaker bureau, advisory committee, etc. Greg Elliott has served as a consultant for Actelion. Harrison Farber has served on a speaker bureau and as a consultant for Actelion. Adaani Frost serves as a Steering Committee member for Actelion and has received honoraria and served on a speaker bureau for Actelion and Cotherix; Other Michelle Turner has received payment from Actelion through a Master Services Agreement.