TY - JOUR T1 - Right ventricular dysfunction in pulmonary hypertension with combined pulmonary fibrosis and emphysema syndrome JF - European Respiratory Journal JO - Eur Respir J VL - 42 IS - Suppl 57 SP - P4081 AU - Andrew Swift AU - Smitha Rajaram AU - Dave Capener AU - Catherine Hill AU - Christine Davies AU - Judith Hurdman AU - Robin Condliffe AU - Charlie Elliot AU - David Kiely AU - Jim Wild Y1 - 2013/09/01 UR - http://erj.ersjournals.com/content/42/Suppl_57/P4081.abstract N2 - Introduction:Recent studies suggest that the coexistence of emphysema and fibrosis alters clinical outcome. The aim of this study was to investigate the comparative clinical characteristics, pulmonary function, haemodynamics and right ventricular (RV) function and outcome in patients with pulmonary hypertension associated with combined pulmonary fibrosis and emphysema (PH-CPFE), chronic obstructive pulmonary disease (PH-COPD) and interstitial lung disease (PH-ILD).Methods:In 79, incident patients with pulmonary hypertension associated with respiratory disease, cardiovascular magnetic resonance imaging was performed at 1.5T. Emphysema and fibrosis were qualitatively assessed on high resolution computed tomography scans as previously described. Pulmonary function tests and right heart catheterisation were also performed.Results:Patients with pulmonary hypertension associated with combined pulmonary fibrosis and emphysema syndrome had a significantly lower right ventricular ejection fraction (RVEF) and a larger right ventricular end-systolic volume index (RVESVI), when compared to both patients with PH-COPD and PH –ILD (p<0.05). A weak, yet statistically significant association between right ventricular dysfunction and the combined emphysema fibrosis score was identified (RVEF; r= -0.25, p=0.038). Whereas, pulmonary function test data were not significantly associated with RV dysfunction. At Kaplan Meier analysis, patients with PH-CPFE patients had worse outcome than those with PH-COPD (p=0.024) and borderline worse outcome than PH-ILD (p=0.067).Conclusion:Patients with PH-CPFE have poor outcome with worse RV function in comparison to patients with PH-COPD and PH-ILD. ER -