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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.

Author information

1
Manchester Adult Cystic Fibrosis Centre, North West Lung Centre, Wythenshawe Hospital, University Hospital of South Manchester, Southmoor Road, Manchester, M23 9LT, United Kingdom. rowland.brightthomas@uhsm.nhs.uk

Abstract

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 (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.

CONCLUSIONS:

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.

PMID:
22633126
DOI:
10.1016/j.jcf.2012.04.012
[Indexed for MEDLINE]
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