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Background and Objective Pulmonary hypertension is a life-limiting complication of interstitial lung disease (ILD-PH). We investigated whether treatment with phosphodiesterase 5 inhibitors (PDE5i) in patients with ILD-PH was associated with improved survival. Methods Consecutive incident patients with ILD-PH and right heart catheterisation, echocardiography and spirometry data were followed from diagnosis to death, transplantation or censoring with all follow-up and survival data modelled by Bayesian methods. Results The diagnoses in 128 patients were idiopathic pulmonary fibrosis (n = 74, 58%), hypersensitivity pneumonitis (n = 17, 13%), non-specific interstitial pneumonia (n = 12, 9%), undifferentiated ILD (n = 8, 6%) and other lung diseases (n = 17, 13%). Final outcomes were death (n = 106, 83%), transplantation (n = 9, 7%) and censoring (n = 13, 10%). Patients treated with PDE5i (n = 50, 39%) had higher mean pulmonary artery pressure (median 38 mm Hg [interquartile range, IQR: 34, 43] vs. 35 mm Hg [IQR: 31, 38], p = 0.07) and percentage predicted forced vital capacity (FVC; median 57% [IQR: 51, 73] vs. 52% [IQR: 45, 66], p=0.08) though differences did not reach significance. Patients treated with PDE5i survived longer than untreated patients (median 2.18 years [95% CI: 1.43, 3.04] vs. 0.94 years [0.69, 1.51], p = 0.003) independent of all other prognostic markers by Bayesian joint-modelling (HR 0.39, 95% CI: 0.23, 0.59, p

AIMS: To develop a suite of quality indicators (QIs) for the evaluation of the care and outcomes for adults with pulmonary arterial hypertension (PAH). METHODS AND RESULTS: We followed the European Society of Cardiology (ESC) methodology for the development of QIs. This included (i) the identification of key domains of care for the management of PAH, (ii) the proposal of candidate QIs following systematic review of the literature, and (iii) the selection of a set of QIs using a modified Delphi method. The process was undertaken in parallel with the writing of the 2022 ESC/European Respiratory Society (ERS) guidelines for the diagnosis and treatment of pulmonary hypertension and involved the Task Force chairs, experts in PAH, Heart Failure Association (HFA) members and patient representatives. We identified five domains of care for patients with PAH: structural framework, diagnosis and risk stratification, initial treatment, follow-up, and outcomes. In total, 23 main and one secondary QIs for PAH were selected. CONCLUSION: This document presents the ESC QIs for PAH, describes their development process and offers scientific rationale for their selection. The indicators may be used to quantify and improve adherence to guideline-recommended clinical practice and improve patient outcomes. ispartof: EUROPEAN JOURNAL OF HEART FAILURE vol:25 issue:4 pages:469-477 ispartof: location:England status: published

Sarcoidosis associated pulmonary hypertension (SAPH) is a well-recognised complication, associated with a seven-fold increase in mortality. This comprehensive review will summarise these recent developments and proposes the use of a phenotype-based management approach in SAPH. Recent registry-based studies have highlighted the adverse outcomes associated with SAPH and shown that reduced 6-min walk distance and diffusion capacity for carbon monoxide are predictive of poor prognosis. There is increasing interest in methods for early detection of SAPH, although whether early diagnosis impacts on survival remains uncertain. The pathophysiology underpinning SAPH is complex and often incorporates multiple mechanisms. Once the diagnosis is confirmed, understanding the underlying phenotypes of SAPH is key to providing the most effective management plan. There is some evidence that treating patients with precapillary PH with pulmonary vasodilators may improve some haemodynamic and quality life measures. However, more work is needed to determine whether mortality is affected. SAPH is associated with worsened survival. A range of phenotypes are recognised in SAPH. Multimodality risk assessment in patients with SAPH is likely to be important and is an area that requires further work. Published evidence for pulmonary vasodilator therapies in SAPH with a Pulmonary arterial hypertension-like phenotype is encouraging so far, but multiple confounding factors affects the quality of the evidence. The role of immunosuppressive agents for improving pulmonary pressures is unclear. Urgent controlled trials are needed.

AIMS: Comprehensive echocardiography assessment of right ventricular (RV) impairment has not been reported in critically ill patients with COVID-19. We detail the specific phenotype and clinical associations of RV impairment in COVID-19 acute respiratory distress syndrome (ARDS). METHODS: Transthoracic echocardiography (TTE) measures of RV function were collected in critically unwell patients for associations with clinical, ventilatory and laboratory data. RESULTS: Ninety patients (25.6% female), mean age 52.0 ± 10.8 years, veno-venous extracorporeal membrane oxygenation (VVECMO) (42.2%) were studied. A significantly higher proportion of patients were identified as having RV dysfunction by RV fractional area change (FAC) (72.0%,95% confidence interval (CI) 61.0-81.0) and RV velocity time integral (VTI) (86.4%, 95 CI 77.3-93.2) than by tricuspid annular plane systolic excursion (TAPSE) (23.8%, 95 CI 16.0-33.9), RVS' (11.9%, 95% CI 6.6-20.5) or RV free wall strain (FWS) (35.3%, 95% CI 23.6-49.0). RV VTI correlated strongly with RV FAC (p ≤ 0.01). Multivariate regression demonstrated independent associations of RV FAC with NTpro-BNP and PVR. RV-PA coupling correlated with PVR (univariate p

The acute respiratory distress syndrome (ARDS) is major clinical problem in respiratory medicine carrying a high mortality and morbidity burden. This review summarizes the diagnosis and pathophysiology behind pulmonary hypertension and right ventricular dysfunction in ARDS as well as current recommended treatment approaches.

P142 Table 1Baseline characteristics and exercise measurements Post COVID-19 BPD (N = 20) Non-COVID BPD (N = 20) Healthy controls (N = 15) BASELINE CHARACTERISTICS Age/years 41 (10) 49 (14) 50 (18) Gender M:F 6:14 6:14 9:6 BMI (kg/m2) 25 (4) 26 (5) 25 (4) Nijmegen score (/64) 23 (12–44) 23 (14–41) - FEV1 (% pred) 111 (13) 107 (16) 96 (6) FVC (% pred) 118 (14) 114 (16) 107 (12) FEV1/FVC Ratio 80 (6) 78 (6) 75 (12) Resting SpO2 (%) 98 (95–100) 99 (94–100) 97 (96–99) Resting HCO3 − (earlobe) (mmol/L) 24 (5) 22 (3) 24 (2) Resting PaCO2 (kPa) 4.4 (0.8) 4.3 (0.7) 4.7 (0.5) Resting BORG CR-10 dyspnoea (/10) 0.7 (0.8) 1.4 (1.3) 0.2 (0.6) PEAK exercise CPET Variables Duration of test (minutes) 10 (4) 9 (2) 15 (3) Main reason cited for exercise cessation Legs = 6 Legs = 8 Legs = 6 Breathing = 14 Breathing = 12 Breathing = 4 BORG CR-10 dyspnoea (/10) End=5.3 (2.3) End=4.2 (1.5) End=4.1 (1.7) Peak VO2 (L/min) 2.18 (0.87) 1.52 (0.62) 2.77 (1.22) Peak VO2 (% predicted) 106.5 (33.1) 79.8 (17.5) 124.8 (27.3) Peak VO2 (mL/min/kg) 29.6 (7.6) 20.7 (7.1) 37.8 (14.8) Peak Heart Rate (beats/min) 170 (12.6) 141 (26) 167 (15) Heart Rate Reserve (beats/min) 20 (19) 30 (20) 2 (13) Peak VE (L/min) 89 (26) 60 (27) 96 (35) Peak Tidal Volume (L) 2.6 (1.3) 1.86 (0.88) 2.37 (0.71) Peak Breathing Frequency (/min) 43 (23) 31 (9) 33 (8) Peak SpO2 (%) 97 (93–100) 99 (94–100) 95 (73–98) PEAK exercise gas exchange values PaO2 (kPa) 13.3 (3.2) 13.8 (1.2) 13.7 (1.2) PaCO2 (kPa) 4.4 (1.1) 4.2 (0.7) 4.1 (0.7) PETCO2 (kPa) 4.4 (0.6) 4.3 (0.5) 4.8 (0.8) P(A-a)O2 difference (kPa) 2.8 (1.2) 2.1 (0.9) 2.6 (0.9) P(a-ET)CO2 difference (kPa) - 0.10 (0.25) −0.09 (0.37) −0.35 (0.53) Approximate entropy (ApEn) of ventilatory variables during incremental exercise ApEn Tidal Volume 1.61 (0.05) 1.28 (0.23) 1.02 (0.29) ApEn Breathing Frequency 1.40 (0.10) 1.41 (0.20) 1.32 (0.21) ApEn Minute Ventilation 1.22 (0.11) 0.97 (0.30) 0.65 (0.23) Data shown as mean (SD) or median (range);M:F: Male:Female;BMI: body mass index;FEV1: forced expiratory volume in the first second;FVC: forced vital capacity;SpO2;oxygen saturation;CPET: cardiopulmonary exercise test;VO2: oxygen consumption;VE:ConclusionsPost COVID BPD can be characterised by application of non-linear statistical modelling of exercise ventilatory data. This approach now needs further validation to facilitate application in automated CPET equipment, to identify and highlight this important differential diagnosis.

Background Pulmonary arterial hypertension (PAH), is a rare and progressive disease with a high morbidity and mortality. Prostanoid pulmonary vasodilators are the most effective treatment for idiopathic and connective tissue associated PAH. Nonetheless, data examining their safety and efficacy in patients with Eisenmenger syndrome the most severe form of PAH, that is, related to cyanotic congenital heart disease (CHD-PAH) remains limited. Aim To evaluate safety and the clinical efficacy of nebulised iloprost in patients with Eisenmenger syndrome who are on maximum background oral PAH therapy. Methods This pilot study was a randomised, double-blind, placebo-controlled, cross-over study. Patients were randomised to receive nebulised placebo or iloprost for 12 weeks and were then crossed over, with a 7–14-day washout. The primary endpoint was a change in 6-minute walk distance (6MWD). Results Sixteen patients (11 females, aged 47.3 ± 9.8 year) were recruited, twelve completed the study. All were in WHO-FC III, with a resting oxygen saturation of 84 [81–87] % and a median 6MWD of 290 [260–300] m. There was no significant difference in the primary endpoint between nebulised iloprost (0[−4–9]m) and placebo (10 [−15–51]m), p = 0.58. There were no safety concerns with nebulised iloprost. Conclusions Our pilot study provides preliminary evidence that the addition of nebulised iloprost to maximum oral PAH therapy did not improve the primary endpoint of 6MWD. Nebulised iloprost was well tolerated with no significant safety concerns in CHD-PAH.

Background The systolic to diastolic (SD) duration ratio reflects global RV performance in pulmonary arterial hypertension (PAH) yet limited data exists on its application to adult non-congenital PAH. We measured SD ratios on echocardiogram in idiopathic PAH (IPAH) to establish its response to pulmonary vasodilator therapy and prognostic value at diagnosis and follow up. Methods Incident patients with IPAH undergoing echocardiogram, haemodynamic and exercise assessments were identified within our centre between 2005 and 2018. SD ratios were adjusted for heart rate at diagnosis and follow up. Results In 98 patients at diagnosis, the mean SD ratio was 1.03 ± 0.37 decreasing to 0.85 ± 0.25, p Conclusions The SD ratio carries prognostic value at diagnosis and follow up in IPAH. Its further evaluation alongside current PAH risk stratification parameters should be considered.

Background: Breathlessness has significant impact on quality-of-life in patients with pulmonary arterial hypertension (PAH) yet few data assess ventilatory responses during day to day activities. In Eisenmenger syndrome (EIS), the additional impact of cyanosis on symptom profiles is even less well described. Method: Fifteen adult patients with idiopathic PAH (IPAH), six with EIS and fifteen age, sex and BMI-matched controls underwent assessment. Participants completed spirometry and four self-paced stair flights wearing portable CPET equipment. BORG dyspnoea scores were collected at rest and completion. Results: Both IPAH and EIS groups had amplified ventilatory responses. Minute ventilation at stair-climb completion was 44±12, 37±15 and 27±9L/min in EIS, IPAH and Controls respectively (p = 0.01). Peak Tv, RR, Tv:FVC ratio, VE/VCO2 slope and ascent time were higher in EIS and IPAH despite similar spirometry and O2 uptake. EIS patients showed immediate decline in end-tidal CO2 and O2 saturations at stair-climb onset. Peak BORG score correlated with stair-climb time (R=0.73, p=0.002), peak end-tidal CO2 (R=-0.73, p=0.001), peak VE (R=0.53, p=0.008), peak RR (R=0.42, p=0.011) and VE/VCO2 (R=0.54, p=0.001) but not with resting or exercise O2 saturations. Conclusion: Increased ventilatory response to stairclimbing in EIS and IPAH drives higher dyspnoea perception relevant to exercise training and recovery times.

International audience; Rationale: Pulmonary hypertension (PH) has been described in patients treated with leflunomide. Objectives: To assess the association between leflunomide and PH. Methods: We identified incident cases of PH in patients treated with leflunomide from the French PH Registry and through the pharmacoVIGIlAnce in Pulmonary ArTerial Hypertension (VIGIAPATH) program between September 1999 to December 2019. PH etiology, clinical, functional, radiologic, and hemodynamic characteristics were reviewed at baseline and follow-up. A pharmacovigilance disproportionality analysis using the World Health Organization's global database was conducted. We then investigated the effect of leflunomide on human pulmonary endothelial cells. Data are expressed as median (min-max). Results: Twenty-eight patients treated with leflunomide before PH diagnosis was identified. A total of 21 (75%) had another risk factor for PH and 2 had two risk factors. The median time between leflunomide initiation and PH diagnosis was 32 months (1-120). Right heart catheterization confirmed precapillary PH with a cardiac index of 2.37 L⋅min-1 ⋅m-2 (1.19-3.1) and elevated pulmonary vascular resistance at 9.63 Wood Units (3.6-22.1) without nitric oxide reversibility. Five patients (17.9%) had no other risk factor for PH besides exposure to leflunomide. No significant hemodynamic improvement was observed after leflunomide withdrawal. The pharmacovigilance disproportionality analysis using the World Health Organization's database revealed a significant overrepresentation of leflunomide among reported pulmonary arterial hypertension-adverse drug reactions. In vitro studies showed the dose-dependent toxicity of leflunomide on human pulmonary endothelial cells. Conclusions: PH associated with leflunomide is rare and usually associated with other risk factors. The pharmacovigilance analysis suggests an association reinforced by experimental data.

Background: Right ventricular (RV) coupling to the pulmonary circulation influences the response of the RV to the increased afterload caused by pulmonary hypertension (PH), which ultimately determines prognosis. A methodology that accounts for pulsatile flow is required when assessing ventriculo-arterial coupling. We applied wave intensity analysis (WIA) methods to assess the compliance of the main pulmonary artery (PA) in patients with or without PH and compared this to PA distensibility, RV function and clinical outcomes. Methods: High-fidelity blood pressure and Doppler flow velocity tracings were obtained simultaneously during cardiac catheterisation for suspected PH. RV volumes, main PA distensibility and ventriculo-arterial coupling (Emax/Ea) were analysed using cardiovascular magnetic resonance. Results: The study included 17 ​PH patients and 6 controls. Wave speed, reservoir and excess pressure were higher in PH patients compared to controls (p ​

Pulmonary veno-occlusive disease (PVOD) is classified into idiopathic, heritable and associated with other conditions, most often connective tissue disease (CTD) and sarcoidosis. In patients with PVOD with a CTD, immunosuppressive therapies can improve symptoms, gas transfer, imaging and haemodynamics [1, 2]. Despite evidence for inflammation in PVOD pathophysiology [3, 4], including capillary immune complex and complement deposition [5], the role for immunosuppression in idiopathic or heritable forms of PVOD is uncertain. We present three patients with heritable and idiopathic PVOD who received immunosuppression for suspected immune dysregulation. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Dr. Lindner has nothing to disclose. Conflict of interest: Dr. Bergbaum has nothing to disclose. Conflict of interest: Dr. Samaranayake has nothing to disclose. Conflict of interest: Dr. Pitcher reports personal fees from Janssen, during the conduct of the study. Conflict of interest: Dr. Weingart has nothing to disclose. Conflict of interest: Dr. Semple has nothing to disclose. Conflict of interest: Dr. Kokosi has nothing to disclose. Conflict of interest: Dr. Wells has nothing to disclose. Conflict of interest: Dr. Montani reports grants and personal fees from Janssen, grants and personal fees from Bayer, personal fees from GSK, personal fees from Pfizer, grants, personal fees and non-financial support from MSD, personal fees from Chiesi, personal fees from Boehrigher, non-financial support from Accerleron, outside the submitted work. Conflict of interest: Dr. Dimopoulos reports grants, personal fees and other from Janssen, during the conduct of the study. Conflict of interest: Dr. McCabe reports personal fees and other from Janssen, during the conduct of the study. Conflict of interest: Dr. Kempny has nothing to disclose. Conflict of interest: Dr. Harries has nothing to disclose. Conflict of interest: Dr. Orchard has nothing to disclose. Conflict of interest: Dr. Wort reports grants, personal fees from Janssen, grants and personal fees from MSD, grants from GSK, reseach grants from Janssen and Bayer, during the conduct of the study. Conflict of interest: Dr. Price reports personal fees from Janssen, during the conduct of the study.

Background The risk of complications, including death, is substantially increased in patients with pulmonary hypertension (PH) undergoing anaesthesia for surgical procedures, especially in those with pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH). Sedation also poses a risk to patients with PH. Physiological changes including tachycardia, hypotension, fluid shifts, and an increase in pulmonary vascular resistance (PH crisis) can precipitate acute right ventricular decompensation and death. Methods A systematic literature review was performed of studies in patients with PH undergoing non-cardiac and non-obstetric surgery. The management of patients with PH requiring sedation for endoscopy was also reviewed. Using a framework of relevant clinical questions, we review the available evidence guiding operative risk, risk assessment, preoperative optimisation, and perioperative management, and identifying areas for future research. Results Reported 30 day mortality after non-cardiac and non-obstetric surgery ranges between 2% and 18% in patients with PH undergoing elective procedures, and increases to 15–50% for emergency surgery, with complications and death usually relating to acute right ventricular failure. Risk factors for mortality include procedure-specific and patient-related factors, especially markers of PH severity (e.g. pulmonary haemodynamics, poor exercise performance, and right ventricular dysfunction). Most studies highlight the importance of individualised preoperative risk assessment and optimisation and advanced perioperative planning. Conclusions With an increasing number of patients requiring surgery in specialist and non-specialist PH centres, a systematic, evidence-based, multidisciplinary approach is required to minimise complications. Adequate risk stratification and a tailored-individualised perioperative plan is paramount.

Background: The risk of complications, including death, is markedly increased in patients with pulmonary hypertension (PH) undergoing anaesthesia for surgical procedures. We performed a systematic literature review of available studies in patients with PAH or chronic thromboembolic PH (CTEPH) undergoing non-cardiac, non-obstetric surgery, finding a mortality ranging between 2-18% in elective and 15-50% in emergency surgery. Methods/Results: We devised relevant clinical questions based on expert consensus opinion in order to provide recommendations of best practice, using a Delphi scoring method. Based on findings from our literature review and expert opinion, we provide 10 recommendations for best clinical practice involving (1) preoperative planning and the use of a perioperative MDT, (2) management of procedural sedation, (3) decision making where surgery is performed, (4) patient-related risk assessment, (5) procedure-related risk assessment, (6) perioperative optimization of PH therapies, (7) perioperative parenteral PAH therapy, (8) perioperative monitoring and anaesthetic management, (9) postoperative management, and (1) management of perioperative pulmonary hypertensive crises, including consideration of ECMO and lung transplantation. Areas for future research are also suggested. Conclusion: This was a collaborative project by UK PH services. We provide 10 recommendations for good clinical practice in this common clinical scenario, where risk assessment is crucial, as mortality remains high.

Introduction: Patients with pulmonary hypertension (PH) require repeat investigation to allow risk stratification and guide optimal therapy. These tests include CT and catheter studies utilising ionising radiation and contrast. Oxygen-enhanced magnetic resonance imaging (OE-MRI) is an alternative technique that provides measures related to regional ventilation, diffusion and perfusion and may provide novel biomarkers of PH severity. Aims: Assess correlation of OE-MRI measures with standard clinical measures of PH severity. Methods: Newly diagnosed group 1 or 4 PH patients underwent OE-MRI, with resultant measures compared with standard of care clinical outputs via Spearman’s correlation. Results: 12 patients were scanned (mean age 54.9±16.6 years old, mean pulmonary artery pressure 41.3±13.9mmHg). Significant correlation was seen between right ventricular (RV) end diastolic volume and median OE-MRI oxygen wash in time (WIT) (rs= 0.571, p= 0.048) and WIT interquartile range – a marker of spatial disease heterogeneity (rs= 0.857, p= 0.003). The WIT interquartile range also showed a moderate, negative association with RV ejection fraction (rs= -0.618, p=0.034). Interquartile range of native lung T1 relaxation time negatively correlated with TLCO (rs= -0.786, p= 0.021). Conclusions: Significant correlations have been demonstrated between OE-MRI measures of gas delivery and established markers of PH severity.

Background Improvements in availability and accuracy of diagnostic testing in pulmonary embolism (PE) in the last 20 years have more recently been paralleled by the introduction of additional anticoagulation agents and treatment strategies. These developments are likely to shape potentially important changes in PE incidence, associated mortality and treatment complications. Methods We investigated trends in PE incidence, PE-related mortality and bleeding risk by analysing Hospital Episodes Statistics for England. Results Between 1997 and 2015, 464,046 patients (53.9% female) were hospitalized with PE in England. The annual number of hospitalizations with an associated diagnosis of PE more than doubled over this period (24,366 in 1998 vs. 53,108 in 2014), with a corresponding increase in PE hospitalization rate (50.2 to 97.8 per 100,000 population/year), evident in all age categories. Mortality at 1 and 3 months decreased over the study period and was significantly associated with age, treatment era and comorbidities. The risk of bleeding resulting in hospitalization or death within 3 and 12 months after the index PE admission increased over the study period (4.3%/5.1% for 1998–2004 versus 6.1%/7.2% for 2010–2014, p Conclusions The incidence of PE doubled in England between 1997 and 2015, likely attributable to raised awareness and ability to diagnose less severe cases. While PE-associated mortality decreased, there was an increase in bleeding risk. Renewed efforts directed at reducing the incidence of bleeding, including consideration of anticoagulation regimens and investigation of anticoagulation requirement in patients with low-risk features, are needed.

Introduction: Sarcoidosis associated pulmonary hypertension (SAPH) is a leading contributor to sarcoidosis-related mortality. The 6-min walk test (6MWT) is widely used in assessment of cardiorespiratory conditions. A reduced 6-min walk distance (6MWD) has been associated with increased mortality in SAPH. We examined patients from the Registry of Sarcoidosis Associated Pulmonary Hypertension (ReSAPH) who had performed 6MWT at enrollment to identify variables that affect 6MWD, and the prognostic value of 6MWT variables regarding death or lung transplantation. Material and methods: ReSAPH patients with available 6MWT were included. Variables analyzed using pre-defined cutoffs included 6MWD, initial and end of test Borg dyspnea score, oxygen saturation, and heart rate at beginning, end, and after 1-min recovery, absolute change in oxygen saturation, modified distance-saturation product (mDSP), and the heart rate recovery at 1-min (HRR). Findings: 174 patients met inclusion criteria; 48 patients died and 8 underwent lung transplantation. Patients with 6MWD300 m (p = 0.012). No associations with outcome were observed with mDSP cutoff 200 m%, desaturation≥5% and oxygen saturation

ObjectivesApproximately 5%–10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain.MethodsAll adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Clinical variables associated with arrhythmia and their relation to mortality were assessed using Cox analysis.ResultsA total of 310 patients (mean age 34.9±12.3 years, 36.8% male) were enrolled. The majority had Eisenmenger syndrome (58.4%), 15.2% had a prior defect repair and a third had Down syndrome. At baseline, 14.2% had a prior history of arrhythmia, mostly supraventricular arrhythmia (86.4%). During a median follow-up of 6.1 years, 64 patients developed at least one new arrhythmic episode (incidence 3.47% per year), mostly supraventricular tachycardia or atrial fibrillation (78.1% of patients). Arrhythmia was associated with symptoms in 75.0% of cases. The type of PAH-CHD, markers of disease severity and prior arrhythmia were associated with arrhythmia during follow-up. Arrhythmia was a strong predictor of death, even after adjusting for demographic and clinical variables (HR 3.41, 95% CI 2.10 to 5.53, pConclusionsArrhythmia is common in PAH-CHD and is associated with an adverse long-term outcome, even when managed in a specialist centre.

BACKGROUND AND OBJECTIVE: In interstitial lung disease (ILD), pulmonary hypertension (PH) is a major adverse prognostic determinant. Transthoracic echocardiography (TTE) is the most widely used tool when screening for PH, although discordance between TTE and right heart catheter (RHC) measured pulmonary haemodynamics is increasingly recognized. We evaluated the predictive utility of the updated European Society of Cardiology/European Respiratory Society (ESC/ERS) TTE screening recommendations against RHC testing in a large, well-characterized ILD cohort. METHODS: Two hundred and sixty-five consecutive patients with ILD and suspected PH underwent comprehensive assessment, including RHC, between 2006 and 2012. ESC/ERS recommended tricuspid regurgitation (TR) velocity thresholds for assigning high (>3.4 m/s), intermediate (2.9-3.4 m/s) and low ( 3.4 m/s, and excluded PH in 60% of ILD subjects with a TR velocity

IntroductionSarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients.MethodsReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrolment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest radiography, Scadding stage and 6-min walk distance (6MWD), among others. Cox regression models were used to identify independent predictors of transplant-free survival.ResultsData from 215 patients followed for a mean±sd 2.5±1.9 years were available for analysis. In the 159 precapillary patients, the Kaplan–Meier-adjusted 1-, 3- and 5-year transplant-free survival was 89.2%, 71.7% and 62.0%, respectively. Kaplan–Meier-adjusted 1-, 3- and 5-year transplant-free survival in the incident group was 83.5%, 70.3% and 58.3%, respectively, and in the prevalent group was 94.7%, 72.2% and 66.3%, respectively. Patients with reduced diffusing capacity of the lung for carbon monoxide (DLCO) (1)/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort.ConclusionReduced DLCO (1/FVC ratio was identified as an independent risk factor for worsened outcomes.