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We aimed to describe the clinical characteristics, healthcare resource utilization (HCRU) and costs, health‐related quality of life (HRQoL), and survival for patients with pulmonary arterial hypertension (PAH), stratified by 1‐year mortality risk at diagnosis. Adults diagnosed with PAH at the Sheffield Pulmonary Vascular Disease Unit between 2012 and 2019 were included. Patients were categorized as low, intermediate, or high risk for 1‐year mortality at diagnosis. Demographics, clinical characteristics, comorbidities, HCRU, costs, HRQoL, and survival were analyzed. Overall, 1717 patients were included: 72 (5%) at low risk, 941 (62%) at intermediate risk, and 496 (33%) at high risk. Low‐risk patients had lower HCRU prediagnosis and 1‐year postdiagnosis than intermediate‐ or high‐risk patients. Postdiagnosis, there were significant changes in HCRU, particularly inpatient hospitalizations and accident and emergency (A&E) visits among high‐risk patients. At 3 years postdiagnosis, HCRU for all measures was similar across risk groups. Low‐risk patients had lower EmPHasis‐10 scores (indicating better HRQoL) at diagnosis and at 1‐year follow‐up compared with intermediate‐ and high‐risk patients; only the score in the high‐risk group improved. Median overall survival decreased as risk category increased in analyzed subgroups. Low‐risk status was associated with better 1‐year survival and HRQoL compared with intermediate‐ and high‐risk patients. HCRU decreased in high‐risk patients postdiagnosis, with the most marked reduction in A&E admissions. The pattern of decreased per‐patient inpatient hospitalizations and A&E visits at 3 years postdiagnosis suggests that a diagnosis of PAH helps to decrease HCRU in areas that are key drivers of costs. [ABSTRACT FROM AUTHOR]

What is the psychosocial burden of COVID-19 on people with pulmonary hypertension? Fear of COVID-19, anxiety and depression in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension during the pandemic. PH CARE COVID survey: an international patient survey on the care for pulmonary hypertension patients during the early phase of the COVID-19 pandemic. [Extracted from the article]

Patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (PH) are at increased risk when undergoing anesthesia and major surgery. Data on outcomes for elective orthopedic surgery in patients with PH are limited. A patient pathway was established to provide access to elective lower limb arthroplasty. This included assessment of orthopedic needs, fitness for anesthesia, preoperative optimization, and intra‐ and postoperative management. Patient data were retrospectively retrieved using patient's hospital records. Between 2012 and 2020, 29 operations (21 total hip replacements [THRs], 7 total knee replacements [TKRs], 1 total hip revision) were performed in 25 patients (mean age: 67 years). Perioperatively, 72% were treated with low‐dose intravenous prostanoid. All had arterial lines, and central access and perioperative lithium dilution cardiac output monitoring was used in 86% of cases. Four patients underwent GA, 21 spinal anesthesia, and 4 CSE anesthesia. Supplemental nerve blocks were performed in all patients undergoing general, and 12 of 21 undergoing spinal anesthesia. All were managed in high dependency postoperatively. Hospital length of stay and complication rates were higher than reported in non‐PH patients. Perioperative complications included hypotension requiring vasopressors (n = 10), blood transfusion (n = 7), nonorthopedic infection (n = 4), and decompensated right heart failure (n = 1). There was no associated mortality. All implants were functioning well at 6 weeks and subsequent follow‐up. EmPHasis‐10 quality of score decreased by 5.5 (±2.1) (p = 0.04). A dedicated multiprofessional pathway can be used to safely select and manage patients with PH through elective lower limb arthroplasty. [ABSTRACT FROM AUTHOR]

Abstract Background There is variation in the decisions made by telephone assessment nurses using computerised decision support software (CDSS). Variation in nurses' attitudes to risk has been identified as a possible explanatory factor. This study was undertaken to explore the effect of nurses' attitudes to risk on the decisions they make when using CDSS. The setting was NHS 24 which is a nationwide telephone assessment service in Scotland in which nurses assess health problems, mainly on behalf of out-of-hours general practice, and triage calls to self care, a service at a later date, or immediate contact with a service. Methods All NHS 24 nurses were asked to complete a questionnaire about their background and attitudes to risk. Routine data on the decisions made by these nurses was obtained for a six month period in 2005. Multilevel modelling was used to measure the effect of nurses' risk attitudes on the proportion of calls they sent to self care rather than to services. Results The response rate to the questionnaire was 57% (265/464). 231,112 calls were matched to 211 of these nurses. 16% (36,342/231,112) of calls were sent to self care, varying three fold between the top and bottom deciles of nurses. Fifteen risk attitude variables were tested, including items on attitudes to risk in clinical decision-making. Attitudes to risk varied greatly between nurses, for example 27% (71/262) of nurses strongly agreed that an NHS 24 nurse "must not take any risks with physical illness" while 17% (45/262) disagreed. After case-mix adjustment, there was some evidence that nurses' attitudes to risk affected decisions but this was inconsistent and unconvincing. Conclusion Much of the variation in decision-making by nurses using CDSS remained unexplained. There was no convincing evidence that nurses' attitudes to risk affected the decisions made. This may have been due to the limitations of the instrument used to measure risk attitude.

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.)

The aim of this study was to develop a measure of the impact of pulmonary hypertension (PH) on health-related quality of life (HRQoL) as there is a need for a short, validated instrument that can be used in routine clinical practice. Interviews were conducted with 30 PH patients to derive 32 statements, which were presented as a semantic differential six-point scale (0-5), with contrasting adjectives at each end. This item list was completed by patients attending PH clinics across the UK and Ireland. Rasch analysis was applied to identify items fitting a uni-dimensional model. 226 patients (mean age 55.6±14 years; 70% female) with PH (82% had pulmonary arterial hypertension) completed the study questionnaires. 10 of the 32 items demonstrated fit to the Rasch model (Chi-squared 16; p>0.05) and generated the emPHasis-10 questionnaire. Test-retest (intraclass correlation coefficient 0.95, n=33) and internal consistency (Chronbach's α=0.9) were strong. emPHasis-10 scores correlated consistently with other relevant measures and discriminated subgroups of patients stratified by World Health Organization functional class (ANOVA F=1.73; p<0.001). The emPHasis-10 is a short questionnaire for assessing HRQoL in pulmonary arterial hypertension. It has excellent measurement properties and is sensitive to differences in relevant clinical parameters. It is freely available for clinical and academic use.

Pulmonary arterial hypertension (PAH) is a rare, debilitating and rapidly progressive disease. Although there have been important medical advances in PAH management, the search for a cure continues. Despite an increased understanding of the disease, data on the wider effect of PAH on patients and carers, beyond the clinical symptoms, are still limited. In order to explore this, a large-scale international survey investigated four key areas affected by PAH (physical and practical, emotional, social, and information needs) and provides new insight into patients' and carers' experiences of living with the disease. The results from the survey highlight not only the limited ability of patients to carry out everyday tasks, but also the financial impact and social isolation experienced by both patients and carers. The study confirmed that a decline in a patient's World Health Organization functional class, which indicates an increase in clinical severity of the disease, is associated with greater limitations. Results from the survey demonstrate the need for multidisciplinary PAH management and a comprehensive standard of care to assess and improve all aspects of well-being for both patients and carers. In addition, they underline the need for updated PAH guidelines that address these needs.

The phenotype and outcome of severe pulmonary hypertension in chronic obstructive pulmonary disease (COPD) is described in small numbers, and predictors of survival are unknown. Data was retrieved for 101 consecutive, treatment-naïve cases of pulmonary hypertension in COPD. Mean ± SD follow-up was 2.3 ± 1.9 years. 59 patients with COPD and severe pulmonary hypertension, defined by catheter mean pulmonary artery pressure ≥40 mmHg, had significantly lower carbon monoxide diffusion, less severe airflow obstruction but not significantly different emphysema scores on computed tomography compared to 42 patients with mild-moderate pulmonary hypertension. 1- and 3-year survival for severe pulmonary hypertension, at 70% and 33%, respectively, was inferior to 83% and 55%, respectively, for mild-moderate pulmonary hypertension. Mixed venous oxygen saturation, carbon monoxide diffusion, World Health Organization functional class and age, but not severity of airflow obstruction, were independent predictors of outcome. Compassionate treatment with targeted therapies in 43 patients with severe pulmonary hypertension was not associated with a survival benefit, although improvement in functional class and/or fall in pulmonary vascular resistance >20% following treatment identified patients with improved survival. Standard prognostic markers in COPD have limited value in patients with pulmonary hypertension. This study identifies variables that predict outcome in this phenotype. Despite poor prognosis, our data suggest that further evaluation of targeted therapies is warranted.