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We evaluated the prevalence and prognostic value of CT-pulmonary angiographic (CTPA) measures in 292 treatment naive patients with pulmonary arterial hypertension (PAH). Pulmonary artery calcification (13%) and thrombus (10%) were exclusively seen in PAH-congenital heart disease. Oesophageal dilation (46%) was most frequent in PAH-systemic sclerosis. Ground glass opacification (GGO) (41%), pericardial effusion (38%), lymphadenopathy (19%) and pleural effusion (11%) were common. On multivariate analysis, inferior vena caval area, the presence of pleural effusion and septal lines predicted outcome. In PAH, CTPA provides diagnostic and prognostic information. In addition, the presence of GGO on a CT performed for unexplained breathlessness should alert the physician to the possibility of PAH.

A 73-year-old retired miner, with 36 years ‘at the coal face’, presented with a 5-year history of exertional dyspnoea on climbing steep inclines. His medical history was limited to quiescent chronic lymphocytic lymphoma, for which he required no chemotherapy or radiotherapy and had annual haematology follow-up. He was a smoker, with a 15 pack-year history, and took no regular medications. The patient had a widely split second heart sound, with accentuation of the pulmonary component, and bilateral intercostal bruits. Echocardiography demonstrated normal left sided chambers, moderate mitral and tricuspid regurgitation, and a raised estimated systolic pulmonary artery pressure of 80 mm Hg. Pulmonary function tests showed an obstructive pattern (FEV1 1.91 L (70% predicted), FVC 3.62 L (101%), FEV1/FVC ratio 53%), with a mildly reduced diffusion capacity (transfer coefficient (KCO) 0.86 mmol/min/KPa/L, 70% predicted). On the incremental shuttle walking test, his oxygen saturations dropped from …

We thank Marloes et al for their comments on our recently published article and their interest in pulmonary artery (PA) size as a potential prognostic marker and also their comments regarding adjusting the measurement for body surface area (BSA).1 First, we do recognise the diagnostic value of PA size and PA to aortic (PA:Ao) ratio in selected subgroups of patients, and we have previously shown that a PA:Ao ratio ≥1 in patients with systemic sclerosis and suspected pulmonary arterial hypertension (PAH) is highly suggestive of …

Background Previous studies have demonstrated survival in systemic sclerosis (SSc)-associated pulmonary arterial hypertension (SSc-PAH) to be worse than in idiopathic pulmonary arterial hypertension (IPAH). Possible explanations include age, differences in the underlying pulmonary vasculopathy and the ability of the right ventricle to compensate for the increased afterload.1 We investigated differences between these conditions by comparing demographic, haemodynamic and cardiac magnetic resonance imaging (MRI) characteristics and outcomes in a large cohort of incident, treatment-naive patients. Methods 183 patients with IPAH and 192 patients with SSc-PAH were identified from departmental databases including the ASPIRE registry.2 Sub-group analysis in 83 patients who had undergone cardiac MRI within 14 days of right heart catheterisation was performed. Results Median survival in IPAH was 7.8 years and in SSc-PAH was 3 years (p Conclusion The reasons for poorer outcomes in SSc-PAH are likely to be multifactorial including, but not limited to, older age, increased pulmonary arterial stiffness and reduced gas transfer. References Condliffe R, Howard LS. Connective tissue disease-associated pulmonary arterial hypertension. F1000 Prime Rep. 2015;7:06. Hurdman J, Condliffe R, Elliot CA, et al. ASPIRE registry: assessing the spectrum of pulmonary hypertension Identified at a REferral centre. Eur Respir J. 2012;39(4):945–955.

Background Collateral ventilation has been proposed as a mechanism of compensation of respiratory function in obstructive lung diseases but observations of it in vivo are limited. The assessment of collateral ventilation with an imaging technique might help to gain insight into lung physiology and assist the planning of new bronchoscopic techniques for treating emphysema. Objective To obtain images of delayed ventilation that might be related to collateral ventilation over the period of a single breath-hold in patients with chronic obstructive pulmonary disease (COPD). Methods Time-resolved breath-hold hyperpolarised 3 He MRI was used to obtain images of the progressive influx of polarised gas into initially non-ventilated defects. Results A time-series of images showed that 3 He moves into lung regions which were initially non-ventilated. Ventilation defects with delayed filling were observed in 8 of the 10 patients scanned. Conclusions A method for direct imaging of delayed ventilation within a single breath-hold has been demonstrated in patients with COPD. Images of what is believed to be collateral ventilation and slow filling of peripheral airspaces due to increased flow resistance are presented. The technique provides 3D whole-lung coverage with sensitivity to regional information, and is non-invasive and non-ionising.

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

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism potentially curable by surgery. Perfusion scintigraphy is currently advocated as the imaging modality of choice to exclude CTEPH due to its high sensitivity. We have evaluated the diagnostic utility of lung perfusion MRI. Methods Consecutive patients attending a pulmonary hypertension referral centre undergoing lung perfusion MRI, perfusion scintigraphy, CT pulmonary angiography (CTPA) and right heart catheterisation within 14 days were identified. Results Of 132 patients, 78 were diagnosed as having CTEPH. Lung perfusion MRI correctly identified 76 patients as having CTEPH with an overall sensitivity of 97%, specificity 92%, positive predictive value 95% and negative predictive value 96% compared with perfusion scintigraphy (sensitivity 96%, specificity 90%) and CTPA (sensitivity 94%, specificity 98%). No cases of surgically accessible CTEPH were missed with either modality. Conclusions Lung perfusion MRI has high sensitivity equivalent to perfusion scintigraphy in diagnosing CTEPH but does not require ionising radiation, making it an attractive initial imaging modality to assess patients with suspected CTEPH.

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.