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Little is known about the impact of living with pulmonary hypertension. This paper reports data exploring the experience of living with pulmonary hypertension. Qualitative, semistructured, one-to-one interviews were conducted in participants' homes to understand their experiences of living with pulmonary hypertension. Thematic analysis was used to identify codes and generate themes from the interview data. The identification of initial codes was conducted independently by the first author, and checked by the second. Thirty patients recruited through the pulmonary hypertension descriptions of living with pulmonary hypertension are presented under five themes that center on the invisibility of pulmonary hypertension, and its complex treatment are presented: (i) living with a hidden illness; (ii) being on a symptomology rollercoaster; (iii) expectations from treatments; (iv) treatment burden; and (v) awareness of financial burden of treatments. Key findings included daily challenges of living with a rare condition that is largely 'hidden' and its related complex treatment regimes. People with pulmonary hypertension would benefit if more healthcare professionals, as well as family and friends, would validate their condition and provide them with appropriate support. [ABSTRACT FROM AUTHOR]

Background: Pulmonary Hypertension Association UK (PHA-UK) is the only charity in the UK especially for people affected by pulmonary hypertension (PH). To better understand the impact of PH on patients and carers beyond clinical symptoms, the PHA-UK carried out a cross-sectional survey on the effect of PH on daily living, along with a follow-up survey assessing the financial impact of PH.Methods: This is a descriptive cross-sectional survey of adult patients with PH in the UK. A quantitative survey of four key topics (time to diagnosis, quality of life [QoL], financial impact and specialist treatment), was made available to PHA-UK members and patients on PH therapy, with a follow-up financial impact survey sent to those responders who agreed to be contacted further. Data collection was carried out in January and February 2017 for the main survey, and November and December 2017 for the financial impact survey.Results: The main survey was completed by 567 individuals, and the financial follow-up survey by 171. Mean age of responders was 69 ± 17 years with 70% female. 60% of respondents said PH had a major impact on their QoL, with 45% reporting that treatment and management improves their QoL 'a lot'. The time between first experiencing symptoms and diagnosis was ≥1 year for 48% of patients, with 40% seeing 4+ doctors before diagnosis. 63% of patients reported financial worries. Patients in part-time and full-time work reported the greatest financial burden, with a 13 and 33% fall in monthly income respectively. Patients had positive experiences of treatment in specialist centres, with 62% rating their care 'excellent', and 92% saying they preferred travelling to a specialist centre rather than seeing a local non-specialist.Conclusions: This study reports the largest UK survey exploring issues affecting patients with PH. The study shows that despite the availability of new therapies, patients are still experiencing delays prior to diagnosis, and experiencing both emotional and financial impacts from the disease. By identifying the areas patients find most important in their treatment, this research can inform future care policies and long-term management to support patients living with PH and their families. [ABSTRACT FROM AUTHOR]

This final report of the Lancet Commission into liver disease in the UK stresses the continuing increase in burden of liver disease from excess alcohol consumption and obesity, with high levels of hospital admissions which are worsening in deprived areas. Only with comprehensive food and alcohol strategies based on fiscal and regulatory measures (including a minimum unit price for alcohol, the alcohol duty escalator, and an extension of the sugar levy on food content) can the disease burden be curtailed. Following introduction of minimum unit pricing in Scotland, alcohol sales fell by 3%, with the greatest effect on heavy drinkers of low-cost alcohol products. We also discuss the major contribution of obesity and alcohol to the ten most common cancers as well as measures outlined by the departing Chief Medical Officer to combat rising levels of obesity-the highest of any country in the west. Mortality of severely ill patients with liver disease in district general hospitals is unacceptably high, indicating the need to develop a masterplan for improving hospital care. We propose a plan based around specialist hospital centres that are linked to district general hospitals by operational delivery networks. This plan has received strong backing from the British Association for Study of the Liver and British Society of Gastroenterology, but is held up at NHS England. The value of so-called day-case care bundles to reduce high hospital readmission rates with greater care in the community is described, along with examples of locally derived schemes for the early detection of disease and, in particular, schemes to allow general practitioners to refer patients directly for elastography assessment. New funding arrangements for general practitioners will be required if these proposals are to be taken up more widely around the country. Understanding of the harm to health from lifestyle causes among the general population is low, with a poor knowledge of alcohol consumption and dietary guidelines. The Lancet Commission has serious doubts about whether the initiatives described in the Prevention Green Paper, with the onus placed on the individual based on the use of information technology and the latest in behavioural science, will be effective. We call for greater coordination between official and non-official bodies that have highlighted the unacceptable disease burden from liver disease in England in order to present a single, strong voice to the higher echelons of government. [ABSTRACT FROM AUTHOR]

Health-related quality of life (HRQoL) scores assess symptom burden in pulmonary arterial hypertension (PAH) but data regarding their role in prognostication and risk stratification are limited. We assessed these relationships using the emPHasis-10 HRQoL measure.1745 patients with idiopathic PAH (IPAH), drug-induced PAH (DPAH), heritable PAH (HPAH) (collectively "(I/D/H)PAH"), or connective tissue disease-associated PAH (CTD-PAH), who had completed emPHasis-10 questionnaires at one of six UK referral centres between 2014 and 2017, were identified. Correlations with exercise capacity and World Health Organization (WHO) functional class were assessed, and exploratory risk stratification thresholds were tested.Moderate correlations were seen between emPHasis-10 scores and 6-min walk distance (r=-0.546), incremental shuttle walk distance (r=-0.504) and WHO functional class (r=0.497) (all p<0.0001). Distribution of emPHasis-10 score differed significantly between each WHO functional class (all p<0.0001). On multivariate analysis, emPHasis-10 score, but not WHO functional class, was an independent predictor of mortality. In a risk stratification approach, scores of 0-16, 17-33 and 34-50 identified incident patients with 1-year mortality of 5%, 10% and 23%, respectively. Survival of patients in WHO functional class III could be further stratified using an emPHasis-10 score ≥34 (p<0.01). At follow-up, patients with improved emPHasis-10 scores had improved exercise capacity (p<0.0001) and patients who transitioned between risk groups demonstrated similar survival to patients originally in those risk groups.The emPHasis-10 score is an independent prognostic marker in patients with (I/D/H)PAH or CTD-PAH. It has utility in risk stratification in addition to currently used parameters. Improvement in emPHasis-10 score is associated with improved exercise capacity., Competing Interests: Conflict of interest: R.A. Lewis reports non-financial support for meeting attendance from Actelion Pharmaceuticals, outside the submitted work. Conflict of interest: I. Armstrong has nothing to disclose. Conflict of interest: C. Bergbaum has nothing to disclose. Conflict of interest: M.J. Brewis has nothing to disclose. Conflict of interest: J. Cannon has nothing to disclose. Conflict of interest: A. Charalampopoulos reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, personal fees and non-financial support from Novartis, and grants from Bayer and GSK, outside the submitted work. Conflict of interest: A.C. Church has nothing to disclose. Conflict of interest: J.G. Coghlan has nothing to disclose. Conflict of interest: R.J. Davies has nothing to disclose. Conflict of interest: K. Dimopoulos has nothing to disclose. Conflict of interest: C. Elliot reports personal fees for advisory board work and lectures from Actelion Pharmaceuticals, GlaxoSmithKline and Bayer, as well as grants from Pfizer, Actelion Pharmaceuticals and Bayer, outside the submitted work. Conflict of interest: J.S.R. Gibbs reports personal fees from Acceleron, Arena, Bayer, Complexa, Pfizer and GSK, as well as grants and personal fees from Actelion and United Therapeutics, outside the submitted work. Conflict of interest: W. Gin-Sing has nothing to disclose. Conflict of interest: G. Haji has nothing to disclose. Conflict of interest: A.G. Hameed has nothing to disclose. Conflict of interest: L.S. Howard has nothing to disclose. Conflict of interest: M.K. Johnson reports grants and personal fees for meeting attendance, lectures and advisory board work from Actelion and MSD, outside the submitted work. Conflict of interest: A. Kempny has nothing to disclose. Conflict of interest: D.G. Kiely reports grants, personal fees and non-financial support from Actelion, Bayer and GSK, as well as personal fees and non-financial support from MSD, outside the submitted work. Conflict of interest: F. Lo Giudice has nothing to disclose. Conflict of interest: C. McCabe has nothing to disclose. Conflict of interest: A.J. Peacock has nothing to disclose. Conflict of interest: O. Peleyeju has nothing to disclose. Conflict of interest: J. Pepke-Zaba has nothing to disclose. Conflict of interest: G. Polwarth has nothing to disclose. Conflict of interest: L. Price reports educational grants from Actelion J&J, during the conduct of the study. Conflict of interest: I. Sabroe reports grants and personal fees for advisory board work from AstraZeneca, as well as grants from GSK, outside the submitted work. Conflict of interest: B.E. Schreiber has nothing to disclose. Conflict of interest: K. Sheares reports educational support from Actelion, Bayer and GSK, as well as personal fees for lectures and consultancy from Actelion, outside the submitted work. Conflict of interest: D. Taboada reports fees for lectures and education and travel grants from Actelion, Bayer, GlaxoSmithKline, Lilly, MDS and Pfizer, outside the submitted work. Conflict of interest: A.A.R. Thompson reports grants from the British Heart Foundation (Intermediate Clinical Fellowship FS/18/13/3328), during the conduct of the study, as well as non-financial support for travel to meetings from Actelion Pharmaceuticals Ltd, outside the submitted work. Conflict of interest: M.R. Toshner has nothing to disclose. Conflict of interest: I. Wanjiku has nothing to disclose. Conflict of interest: S.J. Wort has nothing to disclose. Conflict of interest: J. Yorke has nothing to disclose. Conflict of interest: R. Condliffe reports grants, personal fees and non-financial support from Actelion Pharmaceuticals and Bayer, as well as grants from GSK, outside the submitted work., (Copyright ©ERS 2021.)

Pulmonary endarterectomy (PEA) is the gold standard treatment for operable chronic thromboembolic pulmonary hypertension (CTEPH). However, a proportion of patients with operable disease decline surgery. There are currently no published data on this patient group. The aim of this study was to identify outcomes and prognostic factors in a large cohort of consecutive patients with CTEPH.Data were collected for consecutive, treatment-naive CTEPH patients at the Pulmonary Vascular Disease Unit of the Royal Hallamshire Hospital (Sheffield, UK) between 2001 and 2014.Of 550 CTEPH patients (mean±sd age 63±15 years, follow-up 4±3 years), 49% underwent surgery, 32% had technically operable disease and did not undergo surgery (including patient choice n=72 and unfit for surgery n=63), and 19% had inoperable disease due to disease distribution. The 5-year survival was superior in patients undergoing PEA (83%) versus technically operable disease who did not undergo surgery (53%) and inoperable due to disease distribution (59%) (p<0.001). Survival was superior in patients following PEA compared with those offered but declining surgery (55%) (p<0.001). In patients offered PEA, independent prognostic factors included mixed venous oxygen saturation, gas transfer and patient decision to proceed to surgery.Outcomes in CTEPH following PEA are excellent and superior to patients declining surgery, and strongly favour consideration of a surgical intervention in eligible patients., Competing Interests: Conflict of interest: A.A.R. Thompson reports nonfinancial support (travel grants) from Actelion Pharmaceuticals Ltd, outside the submitted work. Conflict of interest: C.A. Elliot reports personal fees for lecturing and advisory board work from Actelion Pharmaceuticals, GlaxoSmithKline and Bayer, grants for research from Pfizer, Actelion Pharmaceuticals and Bayer, and grants for travel and attendance at conferences from Bayer and Actelion Pharmaceuticals, outside the submitted work. Conflict of interest: J. Hurdman was part funded as a clinical research fellow by an unrestricted educational grant from Actelion, and has received funding to attend conferences from Actelion, GSK and Pfizer. Conflict of interest: A. Charalampopoulos has received honoraria for lecturing and taking part in advisory boards, as well as support to attend scientific events/congresses from Actelion, GSK, Servier and MSD. Conflict of interest: N. Hamilton has and continues to receive honoraria for participation in advisory boards and educational meetings, and has and continues to receive funding to attend educational meetings from a number of pharmaceutical companies including Actelion, Bayer, GSK and MSD (these companies manufacture drug therapies for a variety of indications, but include areas of clinical interest in pulmonary hypertension). Conflict of interest: J. Pepke-Zaba, or her institution, has received research, educational grants and speaker's honoraria from Actelion, Bayer Pharma AG, Merck and GSK. Conflict of interest: D.P. Jenkins reports personal fees for lecturing from Bayer, and personal fees for lecturing and consultancy work for the MERIT study from Actelion, outside the submitted work. Conflict of interest: A. Lawrie reports research grants from the British Heart Foundation, Medical Research Council UK, Actelion Pharmaceuticals and GSK, and conference travel and scientific meeting support from Actelion Pharmaceuticals, outside the submitted work. Conflict of interest: R. Condliffe has received honoraria for lecturing and advisory boards from Actelion, Bayer and GSK, outside the submitted work. Conflict of interest: D.G. Kiely has received funding to attend educational meetings, fees for giving educational lectures and participating in advisory boards, and institutional funding for research from Actelion, Bayer and GSK, and has received funding to attend educational meetings, and fees for giving educational lectures and participating in advisory boards from MSD, outside the submitted work., (Copyright ©ERS 2018.)