Explore the vast range of titles available.

Pulmonary arterial hypertension (PAH) is a multifactorial and severe disease without curative therapies. PAH pathobiology involves altered pulmonary arterial tone, endothelial dysfunction, distal pulmonary vessel remodeling, and inflammation, which could all depend on ion channel activities (K+, Ca2+, Na+ and Cl−). This review focuses on ion channels in the pulmonary vasculature and discusses their pathophysiological contribution to PAH as well as their therapeutic potential in PAH.

Background Understanding of Long COVID has advanced through patient-led initiatives. However, research about barriers to accessing Long COVID services is limited. This study aimed to better understand the need for, access to, and quality of, Long COVID services. We explored health needs and experiences of services, including ability of services to address needs. Methods Our study was informed by the Levesque et al.’s (2013) “conceptual framework of access to health care.” We used Interpretive Description, a qualitative approach partly aimed at informing clinical decisions. We recruited participants across five settings. Participants engaged in one-time, semi-structured, virtual interviews. Interviews were transcribed verbatim. We used reflexive thematic analysis. Best practice to ensure methodological rigour was employed. Results Three key themes were generated from 56 interviews. The first theme illustrated the rollercoaster-like nature of participants’ Long COVID symptoms and the resulting impact on function and health. The second theme highlighted participants’ attempts to access Long COVID services. Guidance received from healthcare professionals and self-advocacy impacted initial access. When navigating Long COVID services within the broader system, participants encountered barriers to access around stigma; appointment logistics; testing and ‘normal’ results; and financial precarity and affordability of services. The third theme illuminated common factors participants liked and disliked about Long COVID services. We framed each sub-theme as the key lesson (stemming from all likes and dislikes) that, if acted upon, the health system can use to improve the quality of Long COVID services. This provides tangible ways to improve the system based directly on what we heard from participants. Conclusion With Long COVID services continuously evolving, our findings can inform decision makers within the health system to better understand the lived experiences of Long COVID and tailor services and policies appropriately.

The current study aimed to explore patient and provider perspectives of the impact of the pandemic on cystic fibrosis healthcare access and service delivery. We used Interpretive Description, a qualitative approach with the end-goal of informing decisions and actions in clinical practice by generating findings that are clinically meaningful and useful. Levesque et al.'s \"Conceptual framework of access to health care\" informed the development of our interview guides. Interviews were conducted via telephone or Zoom and confidentially transcribed verbatim. Data generation and analysis occurred concurrently to allow for iterative refinements of the interview guides. Analysis was informed by Braun and Clarke's six phases of reflexive thematic analysis. Strategies to enhance rigour and trustworthiness of the findings were utilized. We completed 13 interviews: 8 with patients and 5 with providers. Three key themes were generated: (a) Tensions due to infection prevention at micro- meso-, and macro- levels; (b) Modifying aspects of person-focused care can bolster perceived quality of clinical encounters; and (c) Accessibility of appropriate healthcare services could improve efficiency of service delivery. Infection prevention at the individual level was not found to be burdensome. Society's compliance with public health measures, or lack thereof, impacted the level of stigma and anxiety experienced by patients with cystic fibrosis. A changed model of care reliant on patient self-report instead of clinician-led testing and in-person assessment due to the transition to virtual care was associated with mixed perceptions since patients with cystic fibrosis were comfortable making care decisions but many participants (patient and provider) felt challenged by the lack of objective data for decision-making. It was essential for patients with cystic fibrosis to feel known, heard, and seen by their providers in order to feel the care was effective. Finally, critical insights around the need for a balance of in-person and virtual care as well as the need for mental health supports were offered. The learnings from this study could be translated into practical strategies for improving cystic fibrosis care during the pandemic and beyond. We recommend: (1) a hybrid approach to care moving forward, (2) each patient having a lead physician with others filling in as necessary when scheduling demands, and (3) a reallocation of resources to fund a mental health practitioner position.

Pre-capillary pulmonary hypertension (PH) in systemic sclerosis (SSc) is a heterogeneous condition with an overall bad prognosis. The objective of this study was to identify and characterize homogeneous phenotypes by a cluster analysis in SSc patients with PH. Patients were identified from two prospective cohorts from the US and France. Clinical, pulmonary function, high-resolution chest tomography, hemodynamic and survival data were extracted. We performed cluster analysis using the k-means method and compared survival between clusters using Cox regression analysis. Cluster analysis of 200 patients identified four homogenous phenotypes. Cluster C1 included patients with mild to moderate risk pulmonary arterial hypertension (PAH) with limited or no interstitial lung disease (ILD) and low DLCO with a 3-year survival of 81.5% (95% CI: 71.4-88.2). C2 had pre-capillary PH due to extensive ILD and worse 3-year survival compared to C1 (adjusted hazard ratio [HR] 3.14; 95% CI 1.66-5.94; p = 0.0004). C3 had severe PAH and a trend towards worse survival (HR 2.53; 95% CI 0.99-6.49; p = 0.052). Cluster C4 and C1 were similar with no difference in survival (HR 0.65; 95% CI 0.19-2.27, p = 0.507) but with a higher DLCO in C4. PH in SSc can be characterized into distinct clusters that differ in prognosis.

Few studies have reported predictive factors of outcome after pulmonary endarterectomy (PEA) in chronic thromboembolic pulmonary hypertension. The purpose of this study was to determine factors influencing mortality and predictors of hemodynamic improvement after PEA. A total of 383 consecutive patients who underwent PEA between January 2005 and December 2009 were retrospectively reviewed. Among them, 150 were fully reevaluated 7.5±1 months after PEA by NYHA class, 6-minute walk distance (6MWD), percentage of predicted carbon monoxide transfer factor (TLCO) and right heart catheterisation. Mortality rates at 1 month, 1 year and 3 years were 2.8%, 6.9% and 7.5%, respectively. Preoperative pulmonary vascular resistance (PVR) independently predicted 1-month, 1- and 3-year mortality and age predicted mortality at 1 year and 3 years. Significant improvement in NYHA class and 6MWD were observed and PVR decreased from 773±353 to 307±221 dyn.sec.cm-5 (p<0.001). In 96 patients (64%), PVR decreased by at least 50% and/or was reduced to lower than 250 dyn.sec.cm-5. Preoperative cardiac output (CO) and TLCO predicted hemodynamic improvement. PEA is associated with an excellent long-term survival and a marked improvement in clinical status and hemodynamics. Some preoperative factors including PVR, CO and TLCO can predict postoperative outcomes.

While a survival improvement for this systemic sclerosis-associated PAH subgroup is optimistic and encouraging, analyses of systemic sclerosis patients over such a long period may be prone to lead-time bias, as active screening for PAH in systemic sclerosis patients also likely increased during their study duration. Historically, intravenous epoprostenol is the only PAH therapy that a demonstrated survival benefit in a single short-term multicentre randomized trial. CONFLICTS OF INTEREST Jason Weatherald has received research grants paid to his institution from Janssen, Actelion, Merck, and Bayer; consulting fees paid personally from Janssen; honoraria for lectures from Janssen; payment for expert testimony from Sprigings Intellectual Property Law; travel support from Janssen, and payment for participation in advisory boards from Janssen and Acceleron.

BackgroundInhaled treprostinil (iTre) is the only treatment approved for pulmonary hypertension due to interstitial lung disease (PH-ILD) to improve exercise capacity. This post hoc analysis evaluated clinical worsening and PH-ILD exacerbations from the 16-week INCREASE study and change in 6-minute walking distance (6MWD) in the INCREASE open-label extension (OLE) in patients with less severe haemodynamics.MethodsPatients were stratified by baseline pulmonary vascular resistance (PVR) of <4 Wood units (WU) versus ≥4 WU and ≤5 WU versus >5 WU. Exacerbations of underlying lung disease, clinical worsening and change in N-terminal prohormone of brain natriuretic peptide (NT-proBNP) in INCREASE were evaluated. For the OLE, patients previously assigned to placebo were considered to have a 16-week treatment delay. 6MWD and clinical events in the OLE were evaluated by PVR subgroup.ResultsOf the 326 patients enrolled in INCREASE, patients with less severe haemodynamics receiving iTre had fewer exacerbations of underlying lung disease and clinical worsening events. This was supported by the Bayesian analysis of the risk of disease progression (HR<1), and significant decreases in NT-proBNP levels. In the OLE, patients without a treatment delay had improved exercise capacity after 1-year compared with those with a 16-week treatment delay (22.1 m vs −10.3 m). Patients with a PVR of ≤5 WU without a treatment delay had a change of 5.5 m compared with −8.2 m for those with a treatment delay. Patients without a treatment delay had a prolonged time to hospitalisation, lung disease exacerbation and death.ConclusionTreatment with iTre led to consistent benefits in clinical outcomes in patients with PH-ILD and less severe haemodynamics. Earlier treatment in less severe PH-ILD may lead to better exercise capacity long-term, however, the subgroup analyses in this post hoc study were underpowered and confirmation of these findings is needed.

Background and Aims Post‐acute sequelae of COVID‐19 (PASC) commonly present with persistent respiratory symptoms, even in individuals with normal chest imaging and pulmonary function. Given the heterogeneity within this population, a personalized approach to respiratory physiotherapy could improve outcomes. The purpose of this study was to assess the feasibility and impact of a tailored respiratory physiotherapy program on health‐related quality of life (QoL), functional impairment, and patient‐reported outcome measures (PROMs) in individuals with persistent respiratory symptoms due to PASC. Methods A single‐arm, open‐label trial was conducted with 13 adults diagnosed with PASC, recruited from Long COVID clinics in Calgary, Canada. Participants underwent an 8‐session personalized physiotherapy program, including education, breathing exercises, and strengthening. Feasibility was measured through recruitment, retention, and session completion rates. PROMs were collected at baseline and post‐intervention, and qualitative interviews explored participant perspectives. Results The program was highly feasible, with 100% retention and a 99% completion rate. Significant improvements were observed in QoL, functional status (Post COVID‐19 Function Status scale), and self‐efficacy scores. The 6‐min walk test showed clinically meaningful improvements in three out of seven participants. Qualitative interviews (n = 8) identified three main themes: struggles with PASC, positive aspects of the program, and benefits from completing it. Participants valued the personalized approach, heart rate monitors, flexible scheduling, and a hybrid of in‐person and virtual sessions, reporting increased confidence, improved symptom management, and better mental health. Conclusion A personalized respiratory physiotherapy program is feasible and may benefit individuals with PASC. Larger trials are needed to assess long‐term efficacy and scalability. Trial Registration ClinicalTrials.gov identifier: NCT05040893