The Registry to EValuate Early And Long-term pulmonary arterial hypertension (PAH) Disease Management (REVEAL) is a 54-center, observational, US-based study provides information about demographics, course and management of patients with WHO Group I PAH. Fenfluramine/dexflenfluramine (fen/dex) use has been causally associated with PAH, though whether time course or disease severity differed compared with idiopathic PAH (IPAH) or PAH patients overall has not been determined.
Compare the characteristics, hemodynamics, medications, presenting PAH symptoms, and 1-year survival among REVEAL PAH patients with a history of fen/dex exposure, non-fen/non-dex anorexigen exposure (non-fen/dex), and no anorexigen use (non-anorexigen). Limited to patients with PAH symptom onset between 1998-present.
Median age at diagnosis was 50 years; 70%, 72%, and 59% of fen/dex, non-fen/dex, and non-anorexigen patients were functional class III at diagnosis; females represented 95% (n = 79), 96% (n = 283) and 74% (n = 1775), respectively. BMI was greatest in fen/dex patients (34.7kg/m2) vs 31.3 and 26.8kg/m2, respectively. At diagnosis, median RAPm (11, 9.5, and 8.0mmHg, respectively) was highest in fen/dex patients; however, there were no differences in median PAPm, PVRI, or CI. There were group differences in reporting of dyspnea, fatigue, chest pain, and syncope as initial PAH symptoms. PAH treatments including prostacyclin analogues (47%, 40%, and 41%), phosphodiesterase inhibitors (48%, 54%, and 51%), endothelin receptor antagonists (60%, 48%, and 45%) and calcium channel blockers (13%, 9%, and 7%) varied among groups, respectively. Among female patients only, one-year survival from enrollment was 89%, 91%, and 91% in fen/dex, non-fen/dex, and non-anorexigen patients, respectively (P = 0.86). These characteristics will be compared in patients with pre-1998 PAH symptom onset and with anorexigen exposure ≥ 3 months.
There is remarkably little difference in hemodynamics, presenting symptoms, and one-year survival from enrollment between PAH patients with and without a history of anorexigen exposure.
PAH patients with fen/dex exposure share similar clinical courses and prognosis as IPAH patients consistent with fen/dex an independent risk factor for PAH as opposed to an exacerbating comorbidity.
Robyn Barst, Grant monies (from industry related sources) Robyn Barst has received grant research from Actelion, Eli Lilly, Gilead, Novartis, and Pfizer.; Consultant fee, speaker bureau, advisory committee, etc. Adaani Frost has served as a steering committee member, an advisory board member, and/or a paid consultant to the following entities in the area of pulmonary hypertension: Actelion, Pfizer, Gilead, United Therapeutics/Lung Rx. Robyn Barst serves as a consultant to Actelion, Eli Lilly, Gilead, Novartis, and Pfizer.; Other Michelle Turner is an employee of ICON Clinical Research, a company which receives research funding from Actelion and other pharmaceutical companies.; No Product/Research Disclosure Information