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J Cyst Fibros. 2012 Dec;11(6):532-8. doi: 10.1016/j.jcf.2012.04.012. Epub 2012 May 25.

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

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Manchester Adult Cystic Fibrosis Centre, North West Lung Centre, Wythenshawe Hospital, University Hospital of South Manchester, Southmoor Road, Manchester, M23 9LT, United Kingdom.



We examined pulmonary artery pressure (PAP) characteristics of CF adults, studied clinical correlates and long-term survival.


Comprehensive clinical data were collected and Doppler echocardiography was used to estimate PAP in 109 stable CF adults and 50 healthy controls.


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 (PaO(2), r=-0.673, p<0.001), and FEV(1) percentage predicted (FEV(1)%, 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 FEV(1)% and PaO(2) were both stronger predictors of death.


PAP is raised in CF adults and correlates with pulmonary disease severity. Unlike PaO(2) and FEV(1)%, it does not appear to be an independent prognostic marker.

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