Prognostic value of right ventricular function in patients with severe pulmonary hypertension associated with respiratory disease

Introduction The presence of pulmonary hypertension in patients with respiratory disease is an indicator of poor clinical outcome. The aim of this study was to assess the association between right ventricular (RV) function and survival in patients with severe pulmonary hypertension ‘out of proportion’ to respiratory disease (PH-RESP). Methods In 94 patients with pulmonary hypertension associated with respiratory disease, pulmonary function tests, high resolution computed tomography, cardiac magnetic resonance (CMR) imaging and right heart catheter were performed. RV measurements were analysed as predictors of mortality in patients with severe PH-RESP (mean pulmonary artery pressure ≥ 40 mmHg) Results 48 of 94 patients were diagnosed with severe PH-RESP. During the follow-up period of 2.5 years (+/-8months), of the 48 patients with severe PH-RESP, 31 patients died. WHO functional class (p=0.002), TLCO (%) (p=0.029) RVEF (p=0.024), PVRI (p=0.015) were univariate predictors of adverse outcome at Cox proportional hazards regression analysis in severe PH-RESP. At multivariate analysis, WHO functional class (p=0.036), TLCO (p=0.019), RVEF (p=0.033) remained significant predictors of outcome. Conclusion Patients with severe pulmonary hypertension associated with respiratory disease have a dire clinical outcome. RVEF is an independent predictor of adverse outcome in these patients and may be a powerful biomarker for use in clinical trials of targeted therapy in this population.