Serial development of pulmonary hypertension in patients with idiopathic pulmonary fibrosis

Background: Idiopathic pulmonary fibrosis ( IPF) is a disease with very high mortality. Objective: We sought to characterize serial changes in pulmonary artery pressures ( PAP) in patients with advanced IPF who survive to transplant. Methods: Retrospective analysis of IPF patients comparing mean PAP at the time of initial evaluation for transplantation ( mPAP baseline) with mPAP at the time of transplant ( mPAP follow-up). The measurements were correlated with New York Heart Association ( NYHA) functional class and oxygen requirements. Results: The final cohort consisted of 44 patients with serial right heart catheterization data. The mean mPAP baseline and mPAP follow-up were 22.5 and 32.7 mm Hg, respectively. 38.6% ( 17/44) of the patients had pulmonary hypertension ( PH) at baseline. The majority of the non-PH patients developed PH during the serial time interval with a subsequent incidence of 77.8%. At the time of transplant, 86.4% of the patients had PH. There was a significant association between transplant NYHA class, severity of PH and oxygen requirements. Transplant NYHA class IV patients had a higher rate of mPAP change. The severity of PH at the time of transplant did not affect transplant outcomes. Conclusion: PH is common and progressive in patients with advanced IPF who are transplant candidates. Serial change and severity of PAP elevations have a significant association with oxygen requirements and functional status, but not transplant outcomes. Whether or not progressive PH has a significant impact on outcomes without transplantation requires further study. Copyright (C) 2008 S. Karger AG, Basel.