The Registry to EValuate Early And Long-term pulmonary arterial hypertension (PAH) Disease Management (REVEAL) is a 54-center, observational, US-based study that provides current information about demographics, course and management of patients with WHO Group I PAH. Here we present 2-year follow-up outcomes for both newly and previously diagnosed PAH patients.
The objectives were to estimate survival, freedom from all-cause hospitalization, freedom from major events and freedom from clinical worsening in newly and previously diagnosed PAH patients enrolled in REVEAL. Major events were defined as having ≥1 of the following: death, transplant, or atrial septostomy. Clinical worsening was defined as experiencing ≥1 of the following: death, transplant, atrial septostomy, NYHA functional class (FC) worsening, and medication addition due to PAH progression. Kaplan-Meier estimates are based on times of events relative to REVEAL enrollment.
Overall, 2527 patients were enrolled as of March 27, 2009. Mean age at enrollment was 53 years, 80% were female; mean±SD 6-minute walk test distance (6-MWD) was 366±126 m; 8%, 37%, 50% and 6% were functional class I, II, III and IV, respectively; and 46%, 3% and 51% had IPAH, FPAH, and APAH, respectively. Mean pulmonary arterial pressure was 49±14 mmHg and pulmonary vascular resistance index was 19±11 Wood units. Two-year survival was 80.3±2.8% and 82.8±0.9% in newly diagnosed and previously diagnosed patients, respectively (P=0.054). Two-year freedom from hospitalization was 55.2±3.2% and 59.3±1.2%, respectively (P=0.29). Two-year freedom from major events was 78.1±2.9% and 81.5±0.9%, respectively (P=0.046). Two-year freedom from clinical worsening was 40.2±3.2% and 50.5±1.2%, respectively (P<0.001).
Though previously diagnosed patients had consistently better two-year outcomes, outcomes appear to stabilize in newly diagnosed patients after the initial months, perhaps due to initiation of treatment.
Although PAH remains a fatal progressive disease, medical treatment and careful follow-up in the current era can achieve freedom from major events in most patients.
Michael McGoon, Consultant fee, speaker bureau, advisory committee, etc. Michael McGoon serves on a Data Safety and Monitoring Board/Clinical End-point Committee and as a consultant to Actelion. David Badesch 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, Lilly/ICOS, GSK, and MondoBiotech/mondoGEN. Raymond Benza has received honoraria from Actelion, Encysive, and Pfizer.; Other Dave Miller is an employee of ICON Clinical Research, a company which receives research funding from Actelion and other pharmaceutical companies.; No Product/Research Disclosure Information