~40% of adult patients with functional class I PAH are at risk for death within 5 years of diagnosis.*
*Data are from the Pulmonary Hypertension Connection Registry, which evaluated 1,360 patients prospectively from 1982-2004 and prospectively from 2004-2006 at a single USA practice over time at three different university hospitals (University of Illinois, Rush University Medical Center, and University of Chicago, all Chicago, IL, USA). The survival estimates are from the cohort of n=578 incident/prevalent patients. Limitations include that this was an observational study with a large number of patients entered retrospectively which can lead to lost data or inconclusive data for analysis.
Reference: Thenappan T, Shah SJ, Rich S, Gomberg-Maitland M. A USA-based registry for pulmonary arterial hypertension: 1982-2006. Eur Respir J. 2007;30(6):1103- 1110.