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Further understanding of when to initiate therapies in pulmonary arterial hypertension (PAH) is important to improve long-term outcomes.Post hocanalyses of GRIPHON (NCT01106014) and exploratory analyses of TRITON (NCT02558231) suggested benefit of early selexipag initiation on long-term outcomes, despite no additional benefitversusinitial double combination on haemodynamic and functional parameters in TRITON.Post hocanalyses investigated the effect of early selexipag initiation on disease progression and survival in a large, pooled PAH cohort. Data from newly diagnosed (≤6 months) PAH patients from GRIPHON and TRITON were pooled. Patients on active therapy with selexipag (pooled selexipag group) were compared with those on control therapy with placebo (pooled control group). Disease progression end-points were defined as per the individual studies. Hazard ratios (HR) and 95% CI for time to first disease progression event up to end of double-blind treatment (selexipag/placebo) +7 days and time to all-cause death up to end of study were estimated using Cox regression models. The pooled dataset comprised 649 patients, with 44% on double background therapy. Selexipag reduced the risk of disease progression by 52%versuscontrol (HR: 0.48; 95% CI: 0.35–0.66). HR for risk of all-cause death was 0.70 (95% CI: 0.46–1.10) for the pooled selexipagversuscontrol group. Sensitivity analyses accounting for the impact of PAH background therapy showed consistent results, confirming the appropriateness of data pooling. Thesepost hoc, pooled analyses build on previous insights, further supporting selexipag use within 6 months of diagnosis, including as part of triple therapy, to delay disease progression.

This study evaluated the impact of the Salisbury Protocol for Assessment of Cauda Equina Syndrome (SPACES) on the waiting time for MRI in patients presenting with suspected Cauda Equina Syndrome (sCES) within a UK district general hospital.All consecutive patients undergoing an MRI scan in our hospital, for sCES, over a 12 month period, prior to and following the introduction of SPACES, were identified. Patient's gender, age, MRI diagnosis, time from MRI request to imaging and outcome were recorded.In the year prior to the introduction of SPACES, 66 patients underwent MRI for sCES, out of which 10.6% had cauda equina compression (CEC), 63.5% had other spinal pathology and 25% had a normal scan. In the year after introduction of SPACES, 160 patients underwent MRI for sCES out of which 6.2% had CEC, 70.7% had other spinal pathology and 23% had a normal scan. Despite the referrals for sCES increasing by more than 2-fold following the introduction of SPACES, the median time from MRI request to scan decreased from 9.1 to 4.2 hours (Implementation of SPACES for patients with sCES resulted in a substantial reduction in waiting time for MRI and decreased the number of transfers to the regional hub hospital. Based on our early experience, we encourage other centres within the UK to introduce such a pathway locally, to improve the management of patients with sCES.

Introduction Objective: To evaluate the clinical outcomes of F/BEVAR among octogenarians. Methods Databases were systematically searched for studies that compared outcomes following F/BEVAR between octogenarians and non-octogenarians. The primary outcome measures were peri-operative mortality, survival, re-intervention, aortic rupture, and aneurysm-related deaths. Secondary outcomes were peri-operative outcomes. Results Seven studies published between 2017 and 2022 were included reporting on a total of 7,552 patients (>80yrs, 1536; 3 (OR 0.72 [0.52-0.98], p=.04), hypercholesterolaemia (OR 0.56, [0.42-0.73], p Conclusion Survival is lower in octogenarians but there was no significant difference in aneurysm-related deaths or re-interventions, despite the increased incidence of aortic rupture and endoleaks during follow-up.

Introduction Objective: to identify and evaluate all patient reported outcome measures (PROMS) using the established Consensus-based Standard for the selection of health Measurement Instruments (COSMIN) guidelines. Methods A scoping systematic review was conducted for studies that used a PROM in aortic dissection (AD) patients according to PRISMA-Scr, using electronic databases and grey literature. Results 45 studies were found, encompassing 38 PROMs. The most common PROM utilised was the 36-Item Short-Form Survey (SF-36), used in 23 studies. The majority of the remainder PROMs however (30/38) were only utilised once in their respective studies, with no disease-specific tool identified. 6 studies further evaluated a psychometric property of 4 PROMs (PROMIS-29, SF-36, SWL and SWED-QUAL). Only 1 of these studies was a PROM validation study, with the remaining 5 only reporting on internal consistency. None of the studies assessed content validity. The methodological quality for all 6 papers was judged to be adequate or very good, however, the quality of evidence was judged to be low, with none of the PROMs having undergone a rigorous psychometric evaluation within the AD population. Conclusion This review identified severe heterogeneity in the PROMs used to assess the QOL of AD patients. These instruments are generic and lack any formal content validity evaluation. A validated dissection-specific tool for use in clinical practice is therefore required to better understand and improve QOL in this patient cohort. PROMIS-29 – Patient Reported Outcome Measure Information System SWL – Satisfaction With Life scale SWED-QUAL – Swedish Health-Related Quality of Life Scale

Introduction Objective To assess the quality of information provided by open access websites regarding aortic dissections on the World Wide Web. Methods The first 50 results from Yahoo, Bing and Google were collected using the search term ‘aortic dissection’ and duplicates removed. Website quality was scored using the University of Michigan Consumer Health Website tool (maximum score 80). Website readability was assessed using the Flesch Reading Ease score (maximum score 100) and Simple Measure of Gobbledygook (SMOG) score (years of education needed). Subjective assessment was undertaken to assess the quality of website content. Results 39 of 75 websites were included for analysis after applying the exclusion criteria. 94.9% of websites did not specify the type of aortic dissection discussed. Most websites were weak, with an average Michigan score of 48.89 (SD ±8.09). Sources published by non-profit organizations were of the lowest quality (mean score = 44.69 SD ± 5.85). Most websites were rated as ‘fairly difficult’ to read, with an overall mean FRE score of 58.92 (SD ± 28.03). Websites aimed at medical trainees had lowest readability (mean = 38.87, SD ±15.36). Websites published by non-profit organisations required the least years of education to read, with mean SMOG score of 10.93 (SD ±3.12). Conclusion Current online websites providing education to the public and professionals regarding aortic dissections are difficult to read, with a poor consistency and reliability between publishing organizations. Efforts must be made to produce high-quality, standardised information, to facilitate patient awareness and decision-making autonomy.

Introduction Objective - To assess the quality of information provided by open access websites regarding aortic aneurysms (AA) on the World Wide Web. Methods The first 50 results from Yahoo, Bing and Google were collected using the search term ‘aortic aneurysm’ and duplicates removed. Website quality was scored using the University of Michigan Consumer Health Website tool (maximum score 80). Website readability was assessed using the Flesch Reading Ease (FRE)score (maximum score 100) and Simple Measure of Gobbledygook (SMOG) score (years of education needed). Subjective assessment was undertaken to assess the quality of website content. Results 59 of 81 websites were included for analysis after applying the exclusion criteria. 59.3% of websites were targeted at abdominal aortic aneurysms, and only 3.4% specific to thoracic aneurysms. The mean Michigan score for all sources was 48.6 (SD ±5.8), indicating sources were weak globally irrespective of publisher (public, private, independent or non-profit organization). Most sources were rated as requiring standard reading level, with a global average FRE of 62.72 (SD ±10.8). Mean SMOG scores showed that websites published by private organisations required the least educational level to read (mean = 10.5, SD ± 0.1). Conclusion Current online websites providing education to the public and professionals regarding aortic aneuryms are consistently low quality and lack comprehensiveness, with a paucity of information available for thoracic aortic aneurysms specifically. Efforts must be made to improve the quality of sources as well as expanding the scope of content to facilitate patient awareness and decision-making autonomy.

The abundance of Lp(a) protein holds significant implications for the risk of cardiovascular disease (CVD), which is directly impacted by the copy number (CN) of KIV-2, a 5.5 kbp sub-region. KIV-2 is highly polymorphic in the population and accurate analysis is challenging. In this study, we present the DRAGEN KIV-2 CN caller, which utilizes short reads. Data across 166 WGS show that the caller has high accuracy, compared to optical mapping and can further phase ∼50% of the samples. We compared KIV-2 CN numbers to 24 previously postulated KIV-2 relevant SNVs, revealing that many are ineffective predictors of KIV-2 copy number. Population studies, including USA-based cohorts, showed distinct KIV-2 CN, distributions for European-, African-, and Hispanic-American populations and further underscored the limitations of SNV predictors. We demonstrate that the CN estimates correlate significantly with the available Lp(a) protein levels and that phasing is highly important.

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