Pulmonary artery pressure in cystic fibrosis adults: Characteristics, clinical correlates and long-term follow-up

Background: We examined pulmonary artery pressure (PAP) characteristics of CF adults, studied clinical correlates and long-term survival. Methods: Comprehensive clinical data were collected and Doppler echocardiography was used to estimate PAP in 109 stable CF adults and 50 healthy controls. Results: CF patients had lower day and night-time oxygen status, elevated CRP and BNP, and elevated PAP (27.7(13.2, 62.8) mmHg patients v 17.9(11.3, 30.9) mmHg controls, p<0.001). Even patients with mild pulmonary disease had raised PAP. PAP measurements strongly correlated with arterial partial pressure of oxygen (PaO2, r=-0.673, p<0.001), and FENT, percentage predicted (FEV1%, r=-0.642, p<0.001) which were both independent predictors of PAP. At 10 year follow up PAP measurements were related to survival but FEV1% and PaO2, were both stronger predictors of death. Conclusions: PAP is raised in CF adults and correlates with pulmonary disease severity. Unlike PaO2 and FEV1%, it does not appear to be an independent prognostic marker. (C) 2012 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.