PT  - JOURNAL ARTICLE
AU  - Melanie Brewis
AU  - Alistair Church
AU  - Andrew Peacock
AU  - Martin Johnson
TI  - Severe pulmonary hypertension in patients with emphysema but preserved FEV1: Prognosis and response to treatment
DP  - 2013 Sep 01
TA  - European Respiratory Journal
PG  - P2620
VI  - 42
IP  - Suppl 57
4099  - http://erj.ersjournals.com/content/42/Suppl_57/P2620.short
4100  - http://erj.ersjournals.com/content/42/Suppl_57/P2620.full
SO  - Eur Respir J2013 Sep 01; 42
AB  - Mild to moderate pulmonary hypertension (PH) is a common complication of chronic obstructive pulmonary disease. A smaller proportion present with severe PH with mean pulmonary artery pressures (mPAP) of >35mmHg. The role of PH therapies in this group is unknown.Methods:34 patients with CT evidence of emphysema but preserved FEV1(standardised residuals >-1.645) and mPAP >35mmHg at right heart catheterisation were diagnosed between 2000-2012. 54 idiopathic PAH (IPAH) patients from the same period were used for comparison. Six minute walk distance (6MWD), NTproBNP and functional class (Fc) were assessed at baseline and following 3 months of PH therapy.Results:In comparison to IPAH patients, there was no significant improvement in outcomes. (table1.) 1 and 3 year survival were worse in the emphysema group (Fig1). The strongest predictor of survival in the emphysema group was right atrial pressure (P<0.001).Conclusion. Compared with IPAH patients, patients with emphysema, preserved FEV1 and severe PH have worse survival and appear to respond poorly to PH targeted therapy.