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Risk in PAH

PAH Patients Can Be at Greater Risk Than They Present1-3

Findings From the REVEAL Registry*

REVEAL was a US-based, observational registry involving 55 academic- and community-based treatment centers. 3515 patients enrolled between March 2006 and December 2009.4

An analysis of 3001 patients with PAH showed clinical worsening of PAH in 1 year in 1†:

43%

of FC II patients (401/925)

An anaylsis of first-time hospitalizations in 862 newly diagnosed patients with PAH showed 2‡:

of PAH-related hospitalizations occured in FC II patients(24% [58/244])

An analysis of 3001 patients with PAH showed clinical worsening of PAH in 1 year in 1†:

of FC III patients(625/1399)

An analysis of first-time hospitalizations in 862 newly diagnosed patients with PAH showed2:

og PAH-related hospitalizations occured in FC III patients (136/244)

*REVEAL (Registry to Evaluate Early and Long-term PAH Disease Management) was funded and sponsored by Actelion Pharmaceuticals US, Inc.4

†Analysis included 3001 patients. The analysis criteria included patients age >18 years with PCWP ≤15 mmHg at diagnostic RHC. Clinical worsening was defined as worsening NYHA FC, a ≥15% reduction in 6MWD, all-cause hospitalization, or the introduction of a parenteral prostacyclin analog for any reason. Excludes patients who died or had a major event without a worsening event.

‡Newly diagnosed defined as within 90 days of registry enrollment. Analysis evaluated 862 newly diagnosed patients for first-time hospitalization. Hospitalizations were categorized as PAH related or PAH unrelated based on case report forms. Categories were defined prior to independent review. Of the 862 patients, 718 had available FC assessments (167 were in FC II and 424 were in FC III) and 257 were hospitalized for PAH. Of the 257 patients hospitalized for PAH, 244 had available FC assessments. Data shown represent patients with a PAH-related hospitalization and FC assessment.

6MWD=6-minute walk distance; FC=Functional Class; NYHA=New York Heart Association; PCWP=pulmonary capillary wedge pressure; RHC=right heart catheterization.

References: 1. Frost AE, Badesch DB, Miller DP, et al. Evaluation of the predictive value of a clinical worsening definition using 2-year outcomes in patients with pulmonary arterial hypertension: a REVEAL Registry analysis. Chest. 2013;144(5):1521-1529. 2. Burger CD, Long PK, Shah MR, et al. Characterization of first-time hospitalizations in patients with newly diagnosed pulmonary arterial hypertension in the REVEAL Registry. Chest. 2014;146(5):1263-1273. 3. Humbert M, Kovacs G, Hoeper MM, et al; ESC‍/‍ERS Scientific Document Group. 2022 ESC‍/‍ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Heart J. 2022‍;‍43(‍38‍)‍:‍3618‍-‍3731. 4. McGoon MD, Miller DP. REVEAL: a contemporary US pulmonary arterial hypertension registry. Eur Respir Rev. 2012;21(123):8-18.