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Introduction Recent studies have suggested 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 scored on high resolution computed tomography scans. Demographic data, lung function tests and right heart catheterisation were also performed. Results Patients with pulmonary hypertension associated with combined pulmonary fibrosis and emphysema syndrome had lower right ventricular ejection fraction when compared to both patients with PH-COPD and PH –ILD (p < 0.05). At Kaplan-Meier analysis, patients with PH-CPFE patients had significantly worse outcome than those with PH-COPD (p = 0.015), and borderline worse outcome than patients with PH-ILD (p = 0.050), [Figure][1] . 48 of 94 patients were diagnosed with severe PH-RESP, defined at mPAP≥40 mmHg. WHO functional class (p = 0.036), TLCO (p = 0.019), RVEF (p = 0.033) were significant independent predictors of outcome in patients with severe PH-RESP. Conclusion Patients with severe PH-RESP 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 patients with pulmonary hypertension associated with lung disease, particularly given the unreliable performance of echocardiography in patients with advanced lung disease. ![Abstract S120 Figure 1][2] Abstract S120 Figure 1 Kaplan Meier plot showing survival of patients with pulmonary hypertension associated with combined fibrosis and emphysema (PH-CPFE) in comparison to pulmonary hypertension interstitial lung disease (PH-ILD) and pulmonary hypertension associated with COPD (PH-COPD) [1]: #F1 [2]: pending:yes

Purpose The purpose of our study was to assess the diagnostic accuracy and reliability of contrast enhanced MR lung perfusion compared to perfusion scintigraphy in patients with CTEPH. Methods and Materials Retrospective analysis of patients investigated for CTEPH who had MR lung perfusion, perfusion scintigraphy and CTPA performed within a time interval of 5 days. The MR images were acquired using a time resolved 3D spoiled gradient echo sequence. The sequence parameters: TE 1.1 ms, TR 2.5 ms, flip angle of 30°, FOV=48 cm 2 , slice thickness of 5 mm, average of 32 slices and frame rate was 2 acquisitions per second. This was a breathhold sequence obtained after 0.05 ml/kg of Gadovist injection at 5 ml/s followed by a 20 ml saline flush. The subtraction images and positive enhancement dataset were analysed in the coronal plane. Four static views perfusion scintigraphy views were obtained. Final diagnosis of CTEPH or non-CTEPH was made at a multidisciplinary meeting following detailed multi-modality assessment. Results 27 patients had a diagnosis of CTEPH and 55 patients were diagnosed as non-CTEPH. In the CTEPH group, MRI lung perfusion diagnosed 26 patients as CTEPH and 1 patient with distal surgically inaccessible disease as non-CTEPH. In the non-CTEPH group, four patients were diagnosed as CTEPH and 48 patients were identified as non-CTEPH and three MRI were considered non-diagnostic. MR lung perfusion was not performed in 11 patients due to impaired renal function or patient unable to tolerate breathhold. None of the surgically accessible disease was missed with either modality. The MR lung perfusion had a sensitivity of 96%, specificity of 92%, PPV of 87%, NPV of 98% and accuracy of 94% for diagnosing CTEPH. Perfusion scintigraphy had a sensitivity of 93%, specificity of 90%, PPV of 83%, NPV of 96% and accuracy of 91%. Conclusion Our results show that MR lung perfusion compared to 4-view static perfusion scintigraphy has an overall higher accuracy compared to perfusion scintigraphy and has a role in assessment of patients with suspected CTEPH.

Introduction and Objectives The aim of this study was to assess the clinical utility of quantitative MR indices of cardiac morphology and function in a large cohort of patients with pulmonary hypertension (PH). Methods We retrospectively studied 233 consecutive patients with suspected PH who underwent cardiac MRI and right heart catheterisation (RHC) within 48 h. Four chamber and short axis (SA) CINE images were acquired using cardiac gated multi-slice imaging with a steady state free precession sequence at 1.5T. The diagnostic and prognostic significance of quantitative measurements of right ventricular morphology and function were assessed. Results Right ventricular end-diastolic mass index was the measurement with the strongest correlation with mPAP (r=0.74) and the highest diagnostic accuracy for the detection of PH (area under the receiver operator curve of 0.91). During the mean follow-up of 18 months (0–36 months), 36 patients with PH died. Right ventricular ejection fraction (p=0.003), right ventricular stroke volume index (p=0.03) and IVC size (p=0.01) were the MR predictors of mortality across the subgroups of PH. MR measurements of right ventricular ejection fraction (p=0.004), right ventricular stroke volume index (p=0.02), and left ventricular diastolic eccentricity index (p=0.005), all predicted mortality in patients with pulmonary arterial hypertension, Abstract S70 figure 1 below shows the Kaplan–Meier plots the MR predictors of mortality in PAH. IVC size (p=0.018) was an independent predictor of mortality in the full cohort of patients with PH, and diastolic eccentricity index (p=0.037) was an independent predictor of adverse outcome in patients with PAH. Conclusion Cardiac MRI provides a comprehensive assessment of right ventricular morphology and function in patients with PH. This study confirms the diagnostic and prognostic applicability of MRI in unselected patients with PH of varied aetiologies in a practical clinical setting.