Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious complication of pulmonary embolism, leading to high pulmonary arterial pressures. Pulmonary endarterectomy (PEA) surgery is the gold standard treatment for CTEPH, significantly improving patient outcomes. Post-operative cardiac remodelling is an important consideration, highlighting the need to balance early exercise with potential impacts on cardiac recovery. The objective of the present study was to investigate the safety and feasibility of early exercise interventions following PEA surgery for CTEPH, and to review the existing literature on early exercise interventions post-PEA. Methods A systematic review was conducted using the AMED, CINAHL, Embase and MEDLINE databases. Search terms included “pulmonary endarterectomy”, “chronic thromboembolic pulmonary hypertension”, “exercise”, “exercise training”, “mobility” and “rehabilitation”. Studies were included if they involved adult patients with CTEPH who underwent PEA and participated in post-operative exercise interventions. Results The review identified studies with small, heterogeneous patient cohorts engaging in early post-operative exercise following PEA. Interventions included walking, cycling, dumbbell exercises and respiratory training. Safety measures, such as continuous monitoring of oxygen levels and heart rate, were implemented and no adverse events were reported. Improvements in exercise capacity and right heart measurements were observed, suggesting that early exercise is both safe and beneficial. Conclusion Early exercise interventions following PEA surgery appear safe and feasible, with potential benefits for exercise capacity and cardiac remodelling. Future research should include randomised controlled trials and qualitative studies to confirm these findings and assess impacts on patient quality of life.

ABSTRACT Pulmonary hypertension (PH) is a progressive condition associated with reduced physical activity and impaired quality of life. While exercise training is now recognised as a safe and beneficial adjunct to PH management, opportunities for supervised programmes remain limited. There is a growing need for person‐centred, acceptable interventions that enable patients to engage safely and meaningfully in physical activity within their own environment. The aim of this study was to explore the lived experiences of individuals with PH who participated in a 10‐week, home‐based exercise programme, and to evaluate its acceptability, utility, and perceived impact. Semi‐structured interviews were conducted with participants (N = 13) diagnosed with precapillary PH, specifically pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), who completed the intervention. Data was analysed thematically to identify core themes reflecting patient experiences and perceived changes. Thematic analysis revealed four key themes: convenience and accessibility, development of exercise self‐regulation skills, support and accountability, and perceived improvements in physical fitness and well‐being. Person‐centred, behaviourally informed home‐based exercise interventions can help individuals with PH exercise safely, overcome fear, enhance self‐efficacy, and re‐engage with physical activity. These findings provide patient‐driven insights to guide the design and implementation of scalable exercise models for the PH population.

ABSTRACT This study explored attitudes towards exercise, recollections of physical activity advice received, and the dimensions that influence physical activity (PA) engagement in individuals diagnosed with pulmonary hypertension. Virtual semi‐structured interviews (n = 21) were conducted with those diagnosed with pulmonary hypertension. Participants (mean age 57.4 (SD ± 13.1) were recruited through the Pulmonary Hypertension Association of the UK. Interviews were transcribed verbatim and analysed using reflexive thematic analysis. Four main themes were generated: (i) Fear of breathlessness and overdoing it; (ii) lack of motivation and desire for monitoring and targets; (iii) self‐presentation: keeping up appearances, and (iv) Little PA advice: patient‐driven communication. A lack of motivation was a common barrier to PA engagement amongst PH patients. Other dimensions included fear and anxiety related to dyspnoea and overexertion and protective self‐presentation. The influence of self‐presentation and identity in exercise‐avoidance in PH is a novel finding. The study also found recollections of little PA advice from treating clinicians and a tendency for patient driven PA information. Future PA interventions that alleviate fear and anxiety through cognitive restructuring, provide reassurance on the safety and benefits of exercise, and include clear instruction on behaviour and graded exposure would be worthwhile to increase PA in PH. Interventions that broaden the conception of PA and promote lifestyle‐based PA alongside fostering patient motivation for PA through goal setting, self‐monitoring and review of behaviour are also likely to be valuable.

Pulmonary arterial hypertension is a rare diagnosis and, when present with pulmonary arteriovenous malformations, a diagnosis of hereditary haemorrhagic telangiectasia should be considered. This case highlights the difficulties in this form of PH. https://bit.ly/4tAmW9R.

Purpose: Reflective of a need for inclusive public health guidance that acknowledges capability, complexity, and the need for equity in physical activity promotion, the aim of this work was to address gaps in national guidance and develop tailored national physical activity and sedentary behaviour guidelines for specific populations in Ireland (pregnant and postpartum women, and people living with chronic conditions). These guidelines accompany the updated National Physical Activity and Sedentary Behaviour Guidelines for Ireland (“Every Move Counts”) which were launched in 2024. The first of their kind in Ireland, they represent an innovative step towards delivering inclusive, evidence-informed recommendations that recognise the unique needs, risks, and opportunities of these populations, supporting a more equitable approach to physical activity promotion across the life course. Policy Description: The guidelines were developed using a structured evidence-informed approach, incorporating international best practice, a review of the evidence following the WHO’s 2020 guidelines expansion, stakeholder consultation, and iterative expert input. The same methodology was applied to both guideline sets through separate but parallel development processes to ensure population-specific relevance while maintaining consistency in tone, structure, and messaging. Stakeholder engagement was central, with consultation involving healthcare professionals, public health stakeholders, academics, advocacy groups, and individuals with lived experience. Development included the drafting of guidelines and accompanying health messaging, followed by surveys to assess perceived clarity, relevance, inclusivity, and feasibility with healthcare professionals and target populations. Feedback was synthesised through stakeholder consultation meetings and expert panels, enabling refinement while preserving public health scope and safety considerations. Ireland’s Health Service Executive (HSE) commissioned this work, and dissemination will be led by the HSE through healthcare, community, and policy channels, with messaging tailored for healthcare and exercise professionals, policy makers, and the public. Conclusion: These guidelines provide a critical addition to national policy infrastructure by promoting the benefits of physical activity in a safe, inclusive, and empowering way for groups historically underrepresented in physical activity policy. The process demonstrates a model for inclusive, evidence-informed guideline development and contributes to global efforts to enhance physical activity guidance for diverse populations through practical, stakeholder-informed approaches.

INTRODUCTION:Health-related quality of life (HRQoL) in pulmonary arterial hypertension (PAH) is valued as an outcome measure by patients, clinicians and regulators. The selection of patient-reported outcome measures (PROMs) for measurement of HRQoL in PAH clinical trials lacks systematic evaluation of their suitability, accuracy and reliability. METHODS:We report a systematic review (PROSPERO ID: CRD42024484021) following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines of PROMs selected in PAH clinical trials. PROM measurement properties were then evaluated according to the 10-step COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) checklist and graded by recommendation for use. Finally, HRQoL was modelled into a conceptual framework using patient interviews and surveys. RESULTS:Screening of 896 records identified 90 randomised controlled trials. 43 trials selected PROMs, of which 20 were sufficiently validated to detect meaningful change. Of these, eight trials were adequately powered, using either EuroQol-five dimensions-five levels (EQ-5D-5L), Short-Form-36 (SF-36) or the Living with Pulmonary Hypertension Questionnaire (LPHQ). The COSMIN evaluation recommended EmPHasis-10 and the LPHQ for use (grade A); whereas, SF-36 and EQ-5D-5L require further study (grade B). A conceptual framework of HRQoL was developed from literature comprising 8045 patients. This framework can be used to visualise the different HRQoL concepts measured by different PROMs. CONCLUSION:To improve patient-centred research, greater consistency in PROM selection is required. Three of 90 randomised controlled trials have selected COSMIN-recommended PROMs. Whilst the PROMs evaluated require development across the 10 areas of psychometric property measurement, EmPHasis-10 and the LPHQ can be recommended for use. The ratified conceptual framework can further support PROM selection by identifying the HRQoL concepts they are likely to capture.

Exercise training is recommended for pulmonary hypertension (PH). Post hoc analysis of the PH and Home-Based (PHAHB) trial stratified patients into two groups based on median diffusing capacity of the lungs for carbon monoxide (DLCO). Patients with higher DLCO had a greater improvement in physical activity performance in response to exercise training, compared to those with lower DLCO. DLCO may be an important consideration in prescribing exercise in PH.

Background Pulmonary hypertension (PH) is a heterogeneous condition, associated with a high symptom burden and a substantial loss of exercise capacity. Despite prior safety concerns regarding physical exertion, exercise training as a supportive therapy is now recommended for PH patients. Currently, most programmes are hospital-based, which limits accessibility. There is a need to provide alternative approaches for physical activity engagement for PH patients. The aim of this research was to develop, implement and evaluate the safety, feasibility and effectiveness of home-based physical activity intervention for PH. Methods An entirely remotely delivered home-based physical activity intervention underpinned by behaviour change theory and informed by end-users, was assessed using a single-arm feasibility study design. Participants (n=19; 80% female) with a mean± sd age of 49.9±15.9 years with a diagnosis of PH undertook a 10-week, home-based physical activity intervention with induction training, support materials, telecommunication support, health coaching, exercise training and assessments, all remotely delivered. Training involved respiratory training along with a combination of aerobic and resistance exercises. Results The intervention was deemed safe as no adverse events were reported. A high level of feasibility was demonstrated as the protocol was implemented as intended, sustained a high level of engagement and adherence and was well accepted by participants in terms of enjoyment and utility. There was a significant improvement in functional capacity, physical activity, exercise self-efficacy and quality of life, between baseline and post-training. Conclusion The study demonstrates that an entirely remotely delivered home-based physical activity programme is safe, feasible and effective in improving functional capacity, physical activity and quality of life in PH patients.

There is considerable interest in the role of platelets and blood coagulation in the pathobiology of pulmonary arterial hypertension (PAH). The aim of this study is to characterise thrombin generation in PAH using calibrated automated thrombography (CAT). Institutional ethical approval was granted. Thrombin generation using CAT was performed using a Fluoroskan Ascent® Plate Reader and ThrombinoscopeTM software. In the period between July 2020 and July 2022, 20 individuals with PAH and 20 healthy volunteers were recruited to this study. Platelet counts were significantly lower in individuals with PAH relative to healthy controls (p=0.0053). Mean platelet volume was significantly higher in PAH subjects at 10.8 fL versus 8.2 fL (p=0.0001) respectively. CAT parameters also differed between groups, as individuals with PAH had significantly lower endogenous thrombin potential, peak thrombin generation and thrombin generation velocity index in both platelet-rich and platelet-poor plasma, suggesting a hypocoagulable profile (Table 1). This data demonstrated a hypocoagulable profile in subjects with PAH. This may represent an 'exhaustion effect' of coagulation factors due to sustained and prolonged activation of the procoagulant pathways in the pulmonary circulation. The clinical implications of these results are immensely relevant and support the shift away from empiric anticoagulation in this population.

Objective Physical activity (PA) is an established adjunct therapy for pulmonary hypertension (PH) patients to mitigate PH symptoms and improve quality of life. However, PA engagement within this population remains low. This study investigated PH patients’ knowledge of PA, recalled advice, exercise preferences and PA support needs. Methods Semi-structured interviews were conducted with 19 adults (mean age 50 years; SD ±12 years) diagnosed with PH, living in Ireland. Interview scripts were digitally recorded and transcribed verbatim. Thematic analysis was used to analyse the data. Results Four key themes were identified: Lack of PA knowledge; exercise setting preference; accountability and monitoring; and clinician delivered PA information and guidance. Conclusion This study found that PH clinicians provide suboptimal PA advice, yet patients desired clinician-delivered PA guidance. Home-based exercise was preferred with monitoring and external accountability deemed as important to facilitate sustained engagement. Practice implications PH clinicians are well positioned to play a critical role in assisting and empowering PH patients to engage in PA. Providing training and education to PH clinicians regarding exercise prescription may be beneficial. Further research is needed to evaluate the feasibility and efficacy of home-based exercise interventions to improve quality of life and physical activity in PH.

Introduction: Exercise training is now recommended as an adjunct to medical therapy for stable pulmonary hypertension (PH) patients. To date, most PH exercise training studies have utilized an in-hospital based model which limits accessibility and availability. A home-based exercise program (HBEP) offer an alternative mode of delivery with the potential to reach more patients. Aim: The aim of this study was to explore the acceptability and utility a remotely delivered HBEP from PH patients' perspectives. Methods: Nineteen participants undertook a 10-wk HBEP with induction training, support materials, telecommunication support, health coaching, exercise training and fitness assessments all remotely delivered. Semi-structured, exit interview were conducted. All interviews were audio recorded, transcribed verbatim and analysed with Braun and Clarke's thematic analysis. Results: Thirteen exit interviews lasting 30-55 min were conducted (85% F, mean(SD) age 49.9±15.9 yr). Results identified four main themes; i) convenience and accessibility of the exercise program, ii) development of self-regulation skills, iii) support and accountability and iv) improvements in physical fitness. Participants reported a high level of satisfaction and enthusiasm towards the HBEP. The HBEP allowed patients to engage in exercise in a familiar setting, at a time that was convenient for them and removed the travel burden associated with facility-based programs. Conclusion: There was a high level of acceptability among PH patients for a 100% remotely delivered HBEP. It was deemed to be beneficial, practical, accessible and, appropriate to their condition.

Introduction: Hospital-based exercise programs although beneficial in the treatment and management of pulmonary hypertension (PH) are resource intensive, rarely available and are unlikely to be scalable. Home-based exercise program (HBEP) offer an alternative mode of delivery with the potential to reach more patients. Aims: This study investigated the feasibility, acceptability, safety and efficacy of a remotely delivered HBEP for PH patients. Functional capacity, physical activity (PA), quality of life (QoL) and exercise self-efficacy were also assessed. Methods: The study used a single-arm intervention with pre-post-training comparisons. Eligible adult PH patients were recruited. Participants undertook a 10-wk HBEP with induction training, support materials, telecommunication support, health coaching, exercise training and fitness assessments all remotely delivered. Training involved a combination of cycling, walking and resistance exercises. Results: Nineteen PAH adults (80% F) with a mean (SD) age 49.9±15.9 yr participated. No adverse events were reported and the majority of participants (79%) found the program to be “extremely enjoyable”. Time spent walking (p=.01) and cycling (p=.002) and the number of sets and repetitions of resistance exercises (P=.000) increased between pre-post training. There was a significant improvement in 6-min walk distance (p=.000), moderate (p=.000) and light intensity (p=.008) PA, steps (p=.000), and lower body strength (p=.000). Exercise self-efficacy (p=.000) and QoL (p=.01) also improved. Conclusion: A 100% remotely delivered HBEP is safe, feasible and effective in improving, QoL, functional capacity and PA levels in PH patients.

IntroductionNovel therapies for pulmonary hypertension (PH) have improved survival and slowed disease progression. However, patients still present with symptoms of exertional dyspnoea and fatigue, which impacts their ability to perform activities of daily living, reduces exercise tolerance and impairs their quality of life (QoL). Exercise training has shown to be safe and effective at enhancing QoL and physical function in PH patients, yet it remains an underused adjunct therapy. Most exercise training for PH patients has been offered through hospital-based programmes. Home-based exercise programmes provide an alternative model that has the potential to increase the availability and accessibility of exercise training as an adjunct therapy in PH. The purpose of this study is to investigate the feasibility, acceptability, utility and safety of a novel remotely supervised home-based PH exercise programme.MethodsSingle arm intervention with a pre/post comparisons design and a follow-up maintenance phase will be employed. Eligible participants (n=25) will be recruited from the Mater Misericordiae University Hospital PH Unit. Participants will undergo a 10-week home-based exercise programme, with induction training, support materials, telecommunication support and health coaching sessions followed by a 10-week maintenance phase. The primary outcomes are feasibility, acceptability, utility and safety of the intervention. Secondary outcomes will include the impact of the intervention on exercise capacity, physical activity, strength, health-related QoL and exercise self-efficacy.Ethics and disseminationEthics approval has been obtained from the Mater Misericordiae Institutional Review Board REF:1/378/2032 and Dublin City University Research Ethics DCUREC/2018/246. A manuscript of the results will be submitted to a peer-reviewed journal and results will be presented at conferences, community and consumer forums and hospital research conferences.Trial registration numberISRCTN83783446; Pre-results.

A 25-year old man was admitted to hospital with a 12-month history of progressive dyspnoea and fatigue, with an acute deterioration over the preceding 2 weeks. He denied any significant past medical history, was a lifelong non-smoker and had moved to Ireland from Brazil with his family 1 year prior. On presentation, he was hypoxic, with peripheral oxygen saturations of 79% on room air and tachycardic at 110 beats·min−1. Examination revealed a loud pulmonary component of the second heart sound, but was otherwise unremarkable, as he was clinically euvolaemic, with clear lung fields and there were no audible cardiac murmurs. Chest radiograph demonstrated prominent hilar vasculature (figure 1). Mediastinal adenopathy, septal line thickening, centrilobular ground glass opacities on CT and a markedly reduced TLCO in a young patient with pulmonary hypertension, should alert the clinician to this potential diagnosis https://bit.ly/3cfe9pX

Pulmonary hypertension is a progressive cardiorespiratory disease that is characterized by considerable morbidity and mortality. While physical activity can improve symptoms and quality of life, engagement in this population is suboptimal. The aim of this study was to explore attitudes towards exercise and the dimensions that influence physical activity participation in individuals with pulmonary hypertension. Virtual, semi-structured interviews were conducted with individuals, with a formal diagnosis of pulmonary hypertension. Participants were recruited through the Pulmonary Hypertension Association of Ireland. Interviews were transcribed and analysed using thematic analysis. Nineteen patients were interviewed (n = 19). There was a female preponderance (n = 13) and the mean age was 50 ± 12 years. Three themes were identified and included fear, perceived value of exercise and environmental factors. Fear was the primary theme and included three sub-themes of fear of (i) over-exertion, (ii) physical damage and (iii) breathlessness. The perceived value of exercise encompassed two distinct sub-themes of perceived (i) exercise importance and (ii) benefits of exercise. Environmental factors included the terrain, weather conditions and location. Fear of overexertion, harm and dyspnoea strongly influenced attitudes to and engagement in physical activity. This study revealed heterogenous patient perspectives regarding the importance of physical activity and exercise. Future interventions that mitigate fear and promote the value of physical activity for individuals with pulmonary hypertension may have considerable benefits in promoting physical activity engagement. Such interventions require multidisciplinary involvement, including specialised pulmonary hypertension clinicians and exercise and behaviour change specialists.

Cardiac rehabilitation (CR) is highly effective as secondary prevention for cardiovascular diseases (CVD). Uptake of CR remains suboptimal (30% of eligible patients) and long-term adherence to a physically active (PA) lifestyle is even lower. Innovative strategies are needed to counteract this phenomenon. We developed the PATHway system (Physical Activity Towards Health) to provide a comprehensive, remotely monitored home-based CR program for CVD patients. The PATHway-I study aimed to investigate its feasibility and clinical efficacy during phase III CR. Participants were randomized on a 1:1 basis to the PATHway intervention group (PW) or usual care control group (UC) in a Single-blind, multicenter, randomized controlled pilot trial. Outcomes were assessed at completion of phase II CR and six months follow-up. The primary outcome was PA (Actigraph GT9X link). Secondary outcomes included measures of physical fitness, modifiable cardiovascular risk factors, endothelial function, intima-media thickness of the common carotid artery and quality of life. System usability and patients' experiences were evaluated only in PW. A mixed-model ANOVA with Bonferroni adjustment was used to analyze between-group effects over time. Missing values were handled by means of an intention-to-treat analysis. Statistical significance was set at a two-sided alpha level of 0.05. Data are reported as mean ± SD. A convenience sample of 120 CVD patients (61.4 ± 13.5 years, 22 women) was included. The PATHway system was deployed in the homes of 60 participants. System use decreased over time and system usability was average with a score of 65.7 ± 19.7 (range 5-100). Moderate to vigorous intensity PA increased in PW (PW: 127 ± 58 min to 141 ± 69 min, UC: 146 ± 66 min to 143 ± 71 min; p-interaction=0.039), while diastolic blood pressure (PW: 79 ± 11 to 79 ± 10 mmHg, UC: 78 ± 9 to 83 ± 10 mmHg; p-interaction=0.004) and cardiovascular risk score remained constant, but deteriorated in UC (PW: 15.9 ± 10.4 to 15.5 ± 10.5%, UC: 14.5 ± 9.7 to 15.7 ± 10.9%; p-interaction=0.004). This pilot study demonstrated the feasibility and acceptability of a technology-enabled, remotely monitored, home-based CR program. Although clinical effectiveness was demonstrated, several challenges were identified that could influence adoption of PATHway. This trial was registered at www.clinicaltrials.gov: NCT02717806