Rationale: Chronic thromboembolic pulmonary disease (CTEPD) is defined by chronic organized thrombi in the pulmonary circulation without or with pulmonary hypertension. The current definition of chronic thromboembolic pulmonary hypertension (CTEPH) has adopted lower mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) thresholds. Objectives: Our aim was to identify its impact on the characterization of patients with CTEPD. Methods: All consecutive patients with CTEPD referred for cardiopulmonary exercise testing (CPET) in a pulmonary hypertension center were divided into four groups on the basis of pulmonary hemodynamics: group A, mPAP ≤ 20 mm Hg; group B, mPAP > 20 mm Hg with PVR > 2 and ≤3 Wood units (WU); group C, mPAP > 20 mm Hg with PVR > 3 WU; and group D, mPAP > 20 mm Hg with PVR < 2 WU ("unclassified"). We compared CPET, computed tomography pulmonary angiography, and cardiac magnetic resonance imaging data across the groups. Results: There was mild aerobic capacity impairment, mild/moderate ventilatory inefficiency, and no significant cardiac limitation on CPET in all groups. However, patients in groups A and D had better ventilatory efficiency and less oxygen desaturation on exercise because of lower dead-space ventilation. There was no difference in chronic pulmonary embolus burden and distribution or resting right ventricular function among the groups. Seventeen patients were reclassified as having "CTEPH" on the basis of the current definition. No functional deterioration was noted within a median period of 13 months on repeat CPET. Conclusions: CTEPD patients with similar clot burden and right ventricular function without or with mild/moderate pulmonary hypertension displayed a similar pattern of cardiopulmonary limitation, except for ventilatory efficiency. The current definition of CTEPH may lead to the reclassification of CTEPH in a considerable number of patients.

BACKGROUND AND OBJECTIVES:Brain natriuretic peptide (BNP) and N-terminal prohormone of BNP (NT-proBNP) are important biomarkers in pulmonary arterial hypertension (PAH). However, results are rarely available at the time of clinical assessment. The reliability of NT-proBNP/BNP point-of-care tests (POCT) in PAH patients and the stability of NT-proBNP in posted blood samples, to simulate remote monitoring, was investigated. METHODS:Group 1 PAH patients were prospectively recruited. A sample of 40 was required to demonstrate an intraclass correlation coefficient (ICC) of 0.94 with a 95% confidence interval width of < 0.1 for agreement between POCT and the laboratory standard. Blood samples were taken at two time-points for laboratory and POCT NT-proBNP/BNP. Separate samples were returned to the laboratory by post and some samples were assessed pre- and post-exercise assessing the impact of exercise. RESULTS:Forty-one patients were enrolled with 56 study visits. NT-proBNP laboratory and POCT (n = 50) provided equivalent test results (Passing-Bablok slope = 1.08, CI = 0.97-1.19, intercept = 18.22, CI = -41.6 to 4.5) and ICC = 0.97. However, laboratory and POCT BNP (n = 49), showed non-equivalence (Passing-Bablok slope = 1.24, CI 1.11-1.31, intercept = -5.11, CI = -9.4 to -0.46), ICC = 0.96. POCT NT-proBNP/BNP correctly classified 92% and 86% of cases, respectively against COMPERA 2.0 4-risk-strata thresholds. NT-proBNP postal laboratory samples and immediately processed NT-proBNP laboratory samples showed good agreement and exercise had no clinically significant effect on NT-proBNP/BNP results. Laboratory BNP identified fewer patients as high risk compared to NT-proBNP. BNP and NT-proBNP risk status agreed at only 57% of visits (p < 0.0009). CONCLUSIONS:These data support the use of POCT NT-proBNP as a rapidly accessible and reliable alternative in clinical settings and highlight the potential of NT-proBNP for remote monitoring via posted samples. TRIAL REGISTRATION:ClinicalTrials.gov registration: NCT05421949.

There are limited data comparing parameters reflecting gas transfer used to assess the likelihood of pulmonary hypertension (PH) in patients with systemic sclerosis (SSc) and regarding the impact of transitioning to Global Lung Initiative (GLI)-predicted values. 632 patients with suspected SSc associated PH were identified from the ASPIRE registry. Spirometry and CT reports were reviewed to identify significant lung disease. ROC curve analysis and correlations of the 3 markers of gas transfer with pulmonary arterial pressure were performed. Correlations of GLI-derived values with mean pulmonary arterial pressure were: DLco% r=−0.45, Kco% r=−0.42 and FVC%/DLco% r=0.37. Correlations in patients without lung disease were: DLco% r=−0.51, Kco% r=−0.44, FVC%/DLco% r=0.38, compared to patients with lung disease: DLco% r=−0.41, Kco% r=−0.39, FVC%/DLco% r=0.39. Area under the curve for the presence of PH in the overall study cohort was significantly superior for DLco% at 0.84 (optimal threshold 53%), compared with Kco% 0.74 (60%) and FVC%/DLco% was 0.74 (1.91), p both <0.001. Compared with European Coal and Steel Community-derived data, GLI-derived percent-predicted lung volumes were lower, DLco% and Kco% were higher and consequently FVC%/DLco% lower (p all <0.001). DLco performed as least as strongly as Kco or FVC%/DLco% in terms of correlations with mPAP and diagnostic utility, regardless of the presence or absence of lung disease. Transitioning to GLI equations led to lower predicted spirometric volumes and higher DLco%. This should be considered when interpreting changes in values over time and when using screening algorithms.

Background Tricuspid regurgitation jet velocity (TRJV) on echocardiography is used for screening patients with suspected pulmonary hypertension (PH). Artificial intelligence (AI) tools, such as the US2.AI, have been developed for automated evaluation of echocardiograms and can yield measurements that aid PH detection. This study evaluated the performance and utility of the US2.AI in a consecutive cohort of patients with suspected PH. Methods 1031 patients who had been investigated for suspected PH between 2009-2021 were retrospectively identified from the ASPIRE registry. All patients had undergone echocardiography and right heart catheterisation (RHC). Based on RHC results, 771 (75%) patients with a mean pulmonary arterial pressure >20 mmHg were classified as having a diagnosis of PH (as per the 2022 European guidelines). Echocardiograms were evaluated manually and by the US2.AI tool to yield TRJV measurements. Results The AI tool demonstrated high interpretation yield, successfully measuring TRJV in 87% of echocardiograms. Manually and automatically derived TRJV values showed excellent agreement (intraclass correlation coefficient 0.94, 95% CI 0.94-0.95) with minimal bias (Bland-Altman analysis). Automated TRJV measurements showed equally high diagnostic accuracy for PH as manual measurements (area under the curve 0.88, 95% CI 0.84-0.90 versus 0.88, 95% CI 0.86-0.91). Conclusion Automated TRJV measurements on echocardiography were similar to manual measurements, with similarly high and noninferior diagnostic accuracy for PH. These findings demonstrate that automated measurement of TRJV on echocardiography is feasible, accurate and reliable and support the implementation of AI-based approaches to echocardiogram evaluation and diagnostic imaging for PH.

Introduction Remote assessment of real-world physical activity is a potentially important addition in evaluating the effect of therapies on how a patient "feels, functions and survives". Actigraphy, the most used measure of physical activity, is an accepted clinical trial endpoint in pulmonary arterial hypertension (PAH), with use in clinical settings becoming increasingly common. Remote monitoring of actigraphy provides high dimensional, real-world data. Despite this, there are challenges in adopting actigraphy as a clinical endpoint. One potential under-recognised issue is the impact of seasonal variation on data interpretation. This study analysed physical activity and physiological measurements of cardiopulmonary function to assess stability and variability throughout the year. Methods 87 patients with a confirmed diagnosis of PAH were enrolled into the UK National Cohort Study of Idiopathic and Heritable Pulmonary Arterial Hypertension (13/EE/0203), and an insertable cardiac monitor (LinQ, Medtronic) implanted using standard techniques. Remote data was collected via a regulatory approved online portal between November 2018 and October 2023. Physical activity and heart rate parameters were measured from the LinQ device which contains an embedded single axis accelerometer. Results Mean age of the 87 patients was 52.5 (14.9 SD) years and 78% were female. Two (2.5%) patients were World Health Organization functional class 1 and 32 (40%), 40 (50%), 6 (7.5%) were classes 2, 3 and 4 respectively. Average daily physical activity varied during the year ranging from 129.5 min/day to 155.0 min/day (figure 1A). Variation in physical activity was cyclical over multiple years and significantly altered by season with physical activity most limited in winter (136 min), increasing in autumn (141 min) and spring (149 min), and greatest in summer (151 min) (figure 1B). There were no seasonal changes in day or night heart rate and heart rate variability (figure 1A). Conclusions In patients with PAH physical activity varies by season through the calendar year measured by actigraphy, however, physiological measures of cardiopulmonary function are unchanged. This finding has implications for the use of actigraphy as a clinical trial endpoint and when assessing longitudinal activity in clinical practice. Seasonal effects may render data challenging to interpret in both contexts and may require adjustment. Conflict of Interest No conflict of interest

Introduction Heart failure (HF) incidence is increasing in older adults with high hospitalisation and mortality rates. Treatment is complicated by side effects and comorbidities. We investigated the clinical characteristics of octogenarians presenting to the HF clinic.Methods Data were collected on octogenarians (80–89 years) referred to the HF clinic in two periods. The data included demographics, HF phenotype, comorbidities, symptoms and treatment. We investigate the temporal changes in clinical characteristics using χ2 test. We aimed to determine the clinical characteristics which were associated with optimisation of HF pharmacological intervention in the clinic, conducting multivariate regression analysis. Statistical significance is determined at p<0.05.Results Data were collected in April 2012 to January 2014 and in June 2021 to December 2022. In this cross-sectional study of temporal data, 571 octogenarians were referred to the clinic in the latter period, in whom the prevalence of HF was 68.48% (391 patients). HF with preserved ejection fraction (HFpEF) was the most common phenotype and increased significantly compared with the first period (46.3% and 29.2%, p<0.001). Frailty, chronic kidney disease and ischaemic heart disease increased significantly versus the first period (p<0.001). During the second period, and following the consultation, of the patients with HF with reduced ejection fraction (HFrEF), 86.4% and 82.7% were on a beta blocker and on an ACE inhibitor/angiotensin receptor blocker/angiotensin receptor-neprilysin inhibitor, respectively. Clinical characteristics associated with further optimisations of HF pharmacological therapy in the HF clinic were: New York Heart Association (NYHA) functional class III and the presence of HFrEF phenotypeConclusions With a prevalence of HF at 68% among the octogenarians referred to the HF clinic, HFpEF incidence is rising. The decision to optimise HF pharmacological treatment in octogenarians is driven by NYHA functional class III and the presence of HFrEF phenotype.

Background Diagnostic rates and risk factors for the subsequent development of chronic thromboembolic pulmonary hypertension (CTEPH) following pulmonary embolism (PE) are not well defined. Methods Over a 10-year period (2010–2020), consecutive patients attending a PE follow-up clinic in Sheffield, UK (population 554 600) and all patients diagnosed with CTEPH at a pulmonary hypertension (PH) referral centre in Sheffield (referral population estimated 15–20 million) were included. Results Of 1956 patients attending the Sheffield PE clinic 3 months following a diagnosis of acute PE, 41 were diagnosed with CTEPH with a cumulative incidence of 2.10%, with 1.89% diagnosed within 2 years. Of 809 patients presenting with pulmonary hypertension (PH) and diagnosed with CTEPH, 32 were Sheffield residents and 777 were non-Sheffield residents. Patients diagnosed with CTEPH at the PE follow-up clinic had shorter symptom duration (p<0.01), better exercise capacity (p<0.05) and less severe pulmonary haemodynamics (p<0.01) compared with patients referred with suspected PH. Patients with no major transient risk factors present at the time of acute PE had a significantly higher risk of CTEPH compared with patients with major transient risk factors (OR 3.6, 95% CI 1.11–11.91; p=0.03). The presence of three computed tomography (CT) features of PH in combination with two or more out of four features of chronic thromboembolic pulmonary disease at the index PE was found in 19% of patients who developed CTEPH and in 0% of patients who did not. Diagnostic rates and pulmonary endarterectomy (PEA) rates were higher at 13.2 and 3.6 per million per year, respectively, for Sheffield residents compared with 3.9–5.2 and 1.7–2.3 per million per year, respectively, for non-Sheffield residents. Conclusions In the real-world setting a dedicated PE follow-up pathway identifies patients with less severe CTEPH and increases population-based CTEPH diagnostic and PEA rates. At the time of acute PE diagnosis the absence of major transient risk factors, CT features of PH and chronic thromboembolism are risk factors for a subsequent diagnosis of CTEPH.

BACKGROUND:There are limited data assessing the spectrum of systemic sclerosis-associated pulmonary hypertension (PH). METHODS:Data for 912 systemic sclerosis patients assessed between 2000 and 2020 were retrieved from the Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre (ASPIRE) registry and classified based on 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines and multimodality investigations. RESULTS:Reduction in pulmonary vascular resistance (PVR) diagnostic threshold to >2WU resulted in a 19% increase in precapillary PH diagnoses. Patients with PVR ≤2WU had superior survival to PVR >2-3WU which was similar to PVR >3-4WU. Survival in pulmonary arterial hypertension (PAH) was superior to PH associated with lung disease. However, patients with mild parenchymal disease on CT had similar characteristics and outcomes to patients without lung disease. Combined pre- and postcapillary PH had significantly poorer survival than isolated postcapillary PH. Patients with mean pulmonary arterial wedge pressure (PAWP) 13-15 mm Hg had similar haemodynamics and left atrial volumes to those with PAWP >15 mm Hg. Unclassified-PH had more frequently dilated left atria and higher PAWP than PAH. Although Unclassified-PH had a similar survival to No-PH, 36% were subsequently diagnosed with PAH or PH associated with left heart disease. The presence of 2-3 radiological signs of pulmonary veno-occlusive disease was noted in 7% of PAH patients and was associated with worse survival. Improvement in incremental shuttle walking distance of ≥30 m following initiation of PAH therapy was associated with superior survival. PAH patients diagnosed after 2011 had greater use of combination therapy and superior survival. CONCLUSION:A number of systemic sclerosis PH phenotypes can be recognized and characterized using haemodynamics, lung function and multimodality imaging.

Purpose: Tricuspid regurgitation jet velocity (TRJV) on echocardiography is used for the screening of patients with suspected pulmonary hypertension (PH). Artificial intelligence (AI) tools have been developed for automated evaluation of echocardiograms and can yield measurements that aid PH detection. This study evaluated the performance and utility of an existing tool (US2.AI) in a consecutive cohort of patients with suspected PH.

Background International guidelines recommend regular, hospital-based risk stratification to aid assessment and management of patients with pulmonary arterial hypertension. Technological advances enable daily, remote measurement of cardiopulmonary physiology and physical activity that have the potential to provide early evaluation of therapeutic efficacy and facilitate early intervention based on the physiological changes that precede clinical events. We sought to investigate the relationship between remote-monitored parameters and the COMPERA 2.0 4-strata risk score and evaluate physiological changes following therapeutic escalation and prior to clinical worsening events. Methods Eighty-seven patients with pulmonary arterial hypertension were implanted with insertable cardiac monitors including a nested set of twenty-eight patients also implanted with a pulmonary artery pressure monitor. Hospital measured and remote monitored physiological parameters were evaluated by 4-strata COMPERA 2.0 risk score. A time stratified bidirectional case-crossover study was undertaken to evaluate physiological changes at the time of therapy escalation and clinical worsening events in the nested group with insertable cardiac and pulmonary artery pressure monitors. A summary measure of remote physiological risk was calculated as the sum of the z-score of physical activity, heart rate reserve and total pulmonary resistance and applied to remote monitoring data. Results Insertable cardiac monitor-measured physical activity, heart rate variability and heart rate reserve were decreased and night heart rate increased in patients with increasing COMPERA 2.0 score (p<0.0001). Daily physical activity was related to incremental shuttle walk distance (p<0.0001) but not six-minute walk distance. Following therapeutic escalation mean pulmonary artery pressure and total pulmonary resistance were reduced and cardiac output, and physical activity increased at 7, 4, 22, and 42 days, respectively (p<0.05). Clinical worsening events were preceded by increased mean pulmonary artery pressure and total pulmonary resistance, reduced cardiac output and physical activity (p<0.05). Applying a remote physiological risk score to remote-monitored data demonstrated that following a clinically indicated increase in therapy, a reduction in physiological risk was identifiable at day three, and preceding a clinical worsening event, an increase in adverse physiology was observable at day - 16. Conclusion Approved devices accurately identify change in physiology in patients with pulmonary arterial hypertension following therapeutic intensification and before clinical worsening. A remote assessment of haemodynamic and cardiac monitoring may facilitate personalised, proactive medicine and innovative clinical study designs. Condensed Abstract Technological advances provide the capacity to remotely measure cardiopulmonary physiology. In 87 patients with insertable cardiac monitors and a nested group 28 patients with pulmonary arterial hypertension implanted with pulmonary artery pressure monitors, significant improvements in cardiopulmonary function and physical activity were observed following therapeutic escalation and preceding clinical worsening events. The study highlights the potential of remote monitoring for personalised management, early therapeutic evaluation, and innovative clinical trial designs in patients with pulmonary hypertension. Twitter (X) post #PHPEEPS Remote monitoring shows improved cardiopulmonary function just 7 days after therapy adjustments, and adverse changes 12 days before a worsening event. The future of personalised care? Learning points Pulmonary artery pressure monitor and insertable cardiac monitors offer safe and reliable data capture of physiological risk markers that change in response to therapy and preceding clinical worsening events. Remote monitored measures of physiology differ between patients with low, int-low, int-high and high risk of one-year mortality stratified by COMPERA 2.0 4-strata risk model. Remote risk evaluation may facilitate personalised medicine and proactive management for early evaluation of therapeutic efficacy and detection of clinical worsening. Plain Language Summary This study was undertaken in 87 patients diagnosed with pulmonary arterial hypertension (PAH). Treatments in PAH are based on a risk classification system with the aim of achieving a low-risk group. The usual in-hospital method uses the COMPERA 2.0 risk model which combines a field walk test, NT-proBNP (blood test) and World Health Organisation Functional Classification (WHO FC) which categorises level of breathlessness during everyday activity. The evidence for this is linked to risk of death, classified into four groups: low, intermediate-low, intermediate-high, and high risk. The aim of this study was to see whether newer medical technologies could grade risk in a remote setting. The two technologies used in this study are safe and approved for use. The first is a pulmonary artery pressure monitor (CardioMEMS) that measures the pressure in the lungs. It is implanted during right heart catheterisation (RHC). Measurements can be taken at home and sent securely to a medical database for the healthcare team to view. Please see the plain graphical summary figure for more information on the CardioMEMS device. The second technology is an insertable cardiac monitor (ICM), which is implanted under the skin using local anaesthetic, and sends remote readings such as physical activity and heart rate. Both technologies were implanted into a subgroup of patients to investigate whether these technologies could help classify risk from home, and whether they could detect response to new treatments, or signs that a condition may be getting worse. 28 patients with both these devices took part in the study and a further 59 had an ICM only. A remote risk score was calculated using 3 things: physical activity, heart rate reserve (HRR: difference between maximum heart rate for age and resting heart rate) from the ICM and total pulmonary resistance (TPR: a measure of the pressure and flow through the lungs) from the CardioMEMS. The results showed that these measures could classify risk as well as the in-hospital COMPERA 2.0 model. The remote risk score detected response to treatment as early as 6 days and clinical worsening as early as 12 days before an event (e.g. hospitalisation) in the group observed. Patient and Public Involvement and Engagement (PPIE) The study was developed following the 2017 Pulmonary Hypertension Association UK (PHA UK) survey in which 39% of patients reported difficulties attending hospital for appointments.[1][1] A subsequent remote monitoring survey (2021) was positively received, with key themes highlighting benefits of ‘improving [disease] understanding’, ‘personalising treatment’, and ‘reducing interruptions or unnecessary visits’.[2][2] Patients from the study and volunteers from PHA UK provided feedback on the results of the research. Amendments were made to the lay summary and a graphical summary was introduced following this feedback. There was universal agreement that participation in the study was beneficial to patients and future research. Participants involved in the study agreed the devices offer enhanced accessibility to non-invasive risk stratification and improvements in home-based care with minimal personal effort. Furthermore, the minimally invasive devices offered empowerment, confidence, and reassurance, with “opportunity to play an active role in [their] health and personal wellbeing” and “greater confidence with day-to-day living”. No incentives were offered for the PPIE in this study. ![Plain Graphical Summary:][3] Plain Graphical Summary: CardioMEMS implantation covering frequently asked questions (FAQ). Created with BioRender.com ### Competing Interest Statement Disclosures: AMKR: Research funding: Wellcome Trust Clinical Research Career Development Fellowship (206632/Z/17/Z), Medical Research Council (UK) Experimental Medicine Award (MR/W026279/1), NIHR Biomedical Research Center Sheffield, Contribution in kind: Medtronic, Abbott, Endotronix, Novartis, Janssen. Research support and consulting: NXT Biomedical, Endotronix, SoniVie, Neptune, Gradient. MT: Research funding: NIHR Biomedical Research Center Cambridge, NIHR HTA. Personal support: GCK and Jansen. DSMB: ComCov, FluCov. PDM: personal support: Abbott. JTM, SB, HZ, JP, CA, JT, DNN, CB, CP, CR, SR, JA, AR, LW, JD, RL, FV, CD, NH, IA, KD, AJS, JH, AH, AC, TB, SKWH, JMSW, AART, RC, CE, DGK: none. ### Funding Statement Wellcome Trust Clinical Research Career Development Fellowship (AR: 206632/Z/17/Z; AS: 205188/Z/16/Z, PDM: 214567/Z/18/Z), BHF Intermediate Fellowship (AART: FS/18/13/33281), MRC Confidence in Concepts (AR), Medtronic External Research Program Award (AR), Donald Heath Research Fellowship (JM), MRC Experimental Medicine grant (MR/W026279/1) (AR/JM/MT/DGK), NIHR Applied Research Collaboration North East and North Cumbria (TB: NIHR2001), National Institute for Health Research (SKWH: NIHR302746; JMSW: NIHR301614). The research was carried out at the National Institute for Health and Care Research (NIHR) Sheffield and Cambridge cardiorespiratory Biomedical Research Centres. AR is grateful to Richard Hughes, whose generous philanthropic support has helped to make this work possible. ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: Ethical approval was provided by NHS research ethics committees (FIT-PH: 19/YH/0354; National Cohort Study of Idiopathic and Heritable Pulmonary Arterial Hypertension: 13/EE/0203). I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable. Yes All data produced in the present study are available upon reasonable request to the authors [1]: #ref-1 [2]: #ref-2 [3]: pending:yes

Background: The development of atrial flutter and fibrillation (AFL/AF) in patients with pre-capillary pulmonary hypertension has been associated with an increased risk of morbidity and mortality. Rate and rhythm control strategies have not been directly compared. Methods: Eighty-four patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmo-nary hypertension (CTEPH) with new-onset AFL/AF were identified in the ASPIRE registry. First, baseline characteristics and rates of sinus rhythm (SR) restoration of 3 arrhythmia management strategies (rate control, medical rhythm control and DC cardioversion, DCCV) in an early (2009-13) and later (2014-19) cohort were compared. Longer-term outcomes in patients who achieved SR versus those who did not were then explored. Results: Sixty (71%) patients had AFL and 24 (29%) AF. Eighteen (22%) patients underwent rate control, 22 (26%) medical rhythm control and 44 (52%) DCCV. SR was restored in 33% treated by rate control, 59% medical rhythm control and 95% DCCV (p < 0.001). Restoration of SR was associated with greater improvement in functional class (FC) and Incremental Shuttle Walk Distance (p both <0.05). It also independently predicted superior survival (3-year survival 62% vs 23% in those remaining in AFL/AF, p < 0.0001). In addition, FC III/IV independently predicted higher mortality (HR 2.86, p = 0.007). Right atrial area independently predicted AFL/ AF recurrence (OR 1.08, p = 0.01). DCCV was generally well tolerated with no immediate major complications.Conclusions: Restoration of SR is associated with superior functional improvement and survival in PAH/CTEPH compared with rate control. DCCV is generally safe and is more effective than medical therapy at achieving SR.

Introduction European guidelines for pulmonary hypertension (PH) recommend comprehensive risk stratification to optimise therapy and achieve/maintain a low risk profile (table 1) using repeated hospital-based investigations undertaken in specialist centres. Resting heart rate (RHR), total pulmonary resistance (TPR) and physical activity (min/day) are associated with mortality in patients with PAH and may be combined to provide a remote physiological risk score. In patients with implanted cardiac monitors and pulmonary artery pressure monitors we sought to observe the change in physiology following clinically indicated escalation of therapy and around the time of clinical worsening events. Methods 28 patients with pulmonary arterial hypertension (PAH) were enrolled into (NAIAD – 13/EE/0203) and FIT-PH (19/YH/0354) and implanted with a pulmonary artery pressure monitor (CardioMEMs, Abbott) and insertable cardiac monitors (ICM, LinQ, Medtronic) to measure daily physical activity, cardiac output, mean pulmonary artery pressure (mPAP), total pulmonary resistance (TPR), night heart rate and day heart rate. Results Following therapeutic escalation (n=18), remote mPAP, TPR, cardiac output and physical activity all improved at days 7, 4, 22 and 42 respectively (p<0.05) compared to control (n=28). Clinical worsening events (CWE, n=13) were preceded by an increase in remote monitored mPAP, TPR (figure 2) and reduction in cardiac output and physical activity (p<0.05) compared to the control group (n=24). Changes in physiological risk score (formula) were detectable 6 days after therapeutic escalation and 10 days prior to CWE (p<0.05). Conclusion Daily data provided by implanted, regulatory approved devices accurately provides an early evaluation of clinical efficacy and clinical worsening in patients with pulmonary arterial hypertension. Remote risk evaluation may facilitate personalised therapy and proactive management. Such data can be applied to a newly derived remote physiological risk score, and has valuable utility to clinical practice, and clinical trials. Conflict of Interest None

Introduction European guidelines for pulmonary hypertension (PH) recommend comprehensive risk stratification to optimise therapy and achieve/maintain a low risk profile1,3 using repeated hospital-based investigations undertaken in specialist centres. To facilitate frequent risk evaluation we sought to identify mortality-associated parameters and develop a risk score that may be implemented using approved remote monitoring technology. Methods Consecutive patients (n=5820) with pulmonary hypertension (PH) were identified from the ASPIRE registry (6/YH/0352) and mortality-associated parameters identified using stepwise Cox regression and the Kaplan-Meier method. PH mortality-associated measures identified from ASPIRE were applied to the PAH population group (n=1771) and physiological risk markers identified. Z-scores stratified by age and physiological risk markers were then internally validated using the COMPERA 2.0 simplified four-strata risk model. Kaplan-Meier survival rates were analysed for low, intermediate-low, intermediate-high and high risk categories for 1, 3 and 5 years. Results Multivariate Cox regression from the ASPIRE registry identified age, male sex, PH aetiology, WHO functional class, incremental shuttle walk distance (ISWD), resting heart rate (RHR), total pulmonary resistance (TPR) and pulmonary vascular resistance (PVR) as independent indicators of mortality. Physiological components capable of remote measurement selected were RHR, TPR and ISWD (as exercise component). Mortality was increased with each decile of baseline risk (figure 1). This was stratified by physiological scores comprising z-scores of age, ISWT, TPR and RHR (p<0.001) (Figure 1). LOESS-derived thresholds of physiological risk analysed a sub-group of patients with PAH (n=1771); classifying into low-, intermediate-low, intermediate-high, and high-risk for one-year mortality. These were well matched to COMPERA 2.0 score-stratified groups (Cohen’s weighted Kappa 0.61, p<0.001 between risk strata) (Figure 2, Table 1) demonstrating a valid remote comparator for risk stratification. The physiological risk score = mort.zAge – mort.zTPR + mort.zHRR + mort.zISWT= 1.0 (Table 2). Nationalised data collections ensured no patient was lost to follow-up. All parameters were recorded prior to initiation of any PAH therapy and compared as baseline measures. Conclusion A physiological risk score, comprising age, haemodynamics (TPR, RHR) and ISWD when applied at baseline, accurately stratified patients with PAH for mortality at 1, 3 and 5 years. These parameters have the potential for remote assessment, enabling ease of clinical use and offering potential for further research. Remote application of the physiological risk score requires further validation. Conflict of Interest none