PURPOSE: The Registry to EValuate Early And Long-term PAH Disease Management (REVEAL), a multicenter, observational, U.S.-based study, is designed to meet the need for current and reliable information about demographics, clinical course and management of patients diagnosed with WHO Group I pulmonary arterial hypertension (PAH).
METHODS: A broader than usual PAH definition permitted enrollment of patients with pulmonary artery occlusion pressures (PAOP) ≤;18mmHg, and pediatric patients. Data from REVEAL and from a subgroup (TRAD, 2321 patients) excluding pediatric patients and those with 18mmHg>PAOP≥15mmHg were compared to the original NIH registry, the French Registry (FrR) and a large single center US registry (Thenappan). Age, sex, diagnosis, duration of symptoms prior to diagnosis, 6 minute walk (6MW), diagnostic hemodynamics, and functional class (FC III+IV) were compared where possible between these registries.
RESULTS: Between 3/2006 and 9/2007, 2977 patients were enrolled in the REVEAL registry. The registry confirms that in the 21st century, the US PAH patient population is older (mean age 48), with a higher female preponderance (4:1) than reported previously. In spite of increased awareness of PAH, time from symptoms to diagnosis has increased by 10 months. Hemodynamic parameters are little changed at time of diagnostic heart catheterization compared to the NIH registry; however, only 55% of patients are presenting as FC III/IV compared to 75% in the NIH and FrR registries. These data are consistent in 6MW of FrR patients < REVEAL 6MW. The frequency of HIV as a cause of PAH appears less than that in the FrR, although higher than that reported by Thenappan, suggesting regional HIV differences in the US. The TRAD subgroup analysis has similar demographics as the complete REVEAL registry.
CONCLUSION: The huge REVEAL database clearly demonstrates changes in the PAH demographic characteristics since the original NIH registry, indicating older presentation and striking female preponderance. Diagnosis is not occurring earlier, in spite of increased awareness of the disease.
CLINICAL IMPLICATIONS: Efforts must be made to decrease delays between onset of symptoms and diagnosis of PAH.
DISCLOSURE: Adaani Frost, Grant monies (from industry related sources) Grant Support (Actelion); Consultant fee, speaker bureau, advisory committee, etc. REVEAL Registry Steering Committee (Actelion); No Product/Research Disclosure Information