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The PEGASUS study is the first multicentric and prospective assessment of the safety of air travel flying in pulmonary hypertension (PH) (NCT03051763). Data of air travel from 60 patients with PH was available. No severe adverse events occurred. Nine patients self-reported mild adverse events during flight (13%), while after landing, 12 patients reported events (20%). Solely one patient (2%) had an adverse event leading to medical consultation. In patients with PH and World Health Organization functional classes II and III, air travel was safe., Competing Interests: Henning Gall reports grants from the German Research Foundation and nonfinancial support from the University of Giessen during the conduct of the study, and personal fees from Actelion, AstraZeneca, Bayer, BMS, GSK, Janssen‐Cilag, Lilly, MSD, Novartis, OMT, Pfizer, and United Therapeutics outside the submitted work. Athiththan Yogeswaran reports non‐financialnonfinancial support from the University of Giessen during the conduct of the study. Natascha Sommer reports personal fees from Actelion outside the submitted work. Hossein A. Ghofrani reports grants from the German Research Foundation and nonfinancial support from the University of Giessen during the conduct of the study, and personal fees from Bayer, Actelion, Pfizer, Merck, GSK, and Takeda, grants and personal fees from Novartis, Bayer HealthCare, and Encysive/Pfizer, and grants from Aires, the German Research Foundation, Excellence Cluster Cardiopulmonary Research, and the German Ministry for Education and Research outside the submitted work. Werner Seeger reports grants from the German Research Foundation and nonfinancial support from the University of Giessen during the conduct of the study, and personal fees from Pfizer and Bayer Pharma AG outside the submitted work. Manuel J. Richter reports grants from the German Research Foundation and nonfinancial support from the University of Giessen during the conduct of the study, and grants from United Therapeutics, grants and personal fees from Bayer, and personal fees from Actelion, Mundipharma, Roche, and OMT outside the submitted work. Khodr Tello reports grants from the German Research Foundation and nonfinancial support from the University of Giessen during the conduct of the study, and personal fees from Actelion and Bayer outside the submitted work. Heinrike Wilkens received fees for lectures and/or consultations from Actelion, AOP, Bayer, Biotest, Boehringer‐Ingelheim, Daiichi Sankyo, Ferrer, GSK, Janssen Cilag, and MSD outside the submitted work. Michael Halank reports personal fees from AstraZeneca, Janssen‐Cilag, and MSD outside the submitted work. Melanie Heberling reports personal fees from Janssen‐Cilag und MSD outside the submitted work. Dr. Ulrich receives research grants from the Swiss National Science Foundation, Zurich and Swiss Lung League, Orpha‐Swiss, Emdo Foundation and travel support, lecture fees, and advisory compensation from MSD SA, Orpha Swiss, Janssen SA, Novartis SA, all unrelated to the present work. Katrin Milger reports speaker and/or advisory board honoraria from AOP Pharma, Ferrer, Janssen, MSD. Tobias J. Lange reports personal fees from Acceleron Pharma, AstraZeneca, Bayer, Böhringer Ingelheim, Ferrer, Gossamer Bio, Janssen Cilag, MSD, Orphacare, and Pfizer. Stefan Stadler reports personal fees from Acceleron Pharma, AOP Health, Gossamer Bio, Janssen Cilag, MSD, and Pfizer. Jan C. Kamp is supported by PRACTIS—Clinician Scientist Program of Hannover Medical School, funded by the German Research Foundation, grant no. ME 3696/3‐1, KFO311— 286251789. Ekkehard Grünig has received fees for lectures and/or consultations from Actelion, Bayer/MSD, Ferrer, GEBRO, GSK, Janssen, and OMT. Research grants to his institution have been received from Acceleron, Actelion, BayerHealthCare, MSD, Bellerophon, GossamerBio, GSK, Janssen, Novartis, OMT, Pfizer, REATE, and United Therapeutics outside the submitted work. Satenik Harutynova has received support from Janssen, OMT, Bayer Pharma, GSK and speaker fees from Janssen and OMT outside submitted work. Karen Olsson has received fees for lectures and/or consultations from Acceleron, Actelion, AOP, MSD, Ferrer, Janssen, and OMT. Research grants to her institution have been received from Actelion, all outside the submitted work. All other authors declare no conflicts of interest., (© 2024 The Author(s). Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

Background: We aimed to evaluate the effectiveness of different antibody therapies on nasal polyp symptoms in patients treated for severe asthma., Methods: We performed a retrospective analysis of patients with severe asthma and comorbid CRSwNP who were treated with anti-IgE, anti-IL-5/R or anti-IL-4R. CRSwNP symptom burden was evaluated before and after 6 months of therapy., Results: Fifty patients were included hereof treated with anti-IgE: 9, anti-IL-5/R: 26 and anti-IL-4R: 15 patients. At baseline median SNOT-20 was similar among groups (anti-IgE: 55, anti-IL-5/R: 52 and anti-IL-4R: 56, p = 0.76), median visual analogue scale (VAS) for nasal symptoms was 4, 7 and 8 (p = 0.14) and VAS for total symptoms was higher in the anti-IL-4R group (4, 5 and 8, p = 0.002). After 6 months SNOT-20 improved significantly in all patient groups with median improvement of anti-IgE: -8 (p < 0.01), anti-IL-5/R: -13 (p < 0.001) and anti-IL-4R: -18 (p < 0.001), with larger improvement in the anti-IL-4R group than in anti-IgE (p < 0.001) and anti-IL-5/R (p < 0.001) groups. VAS nasal symptoms improved by median anti-IgE: 0 (n.s.), anti-IL-5/R: -1 (p < 0.01) and anti-IL-4R: -3 (p < 0.001), VAS total symptoms by anti-IgE: -1 (n.s.), anti-IL-5/R: -2 (p < 0.001) and anti-IL-4R: -2 (p < 0.001)., Conclusions: Treatment by all antibodies showed effectiveness in reducing symptoms of CRSwNP in patients with severe asthma, with the largest reduction observed in anti-IL-4R-treated patients., (© 2021 The Authors. Clinical and Translational Allergy published by John Wiley and Sons Ltd on behalf of European Academy of Allergy and Clinical Immunology.)

Pulmonary hypertension is frequently underdiagnosed, and referral is delayed with subsequent impact on outcomes. During the SARS-CoV-2 pandemic, restrictions on daily life and changes in hospitals' daily routine care were introduced in Germany. This multi-centre study provides evidence for a negative influence of these restrictions on patient care in pulmonary hypertension expert referral centres., (© The Author(s) 2020.)

Selexipag is an orally available selective IP prostacyclin-receptor agonist licensed since 2016 for the therapy of pulmonary arterial hypertension (PAH). We aimed to describe real-life data of patients with pulmonary hypertension (PH) treated with selexipag. We analyzed all patients initiated with selexipag from July 2016 to April 2018 at the Department of Internal Medicine V, University of Munich. Non-invasive and invasive parameters corresponding to the risk assessment were collected at baseline and follow-up (FU). Furthermore, we recorded tolerability. Twenty-six patients were treated with selexipag, of whom 23 had PAH and three had chronic thromboembolic PH. At baseline, most patients were in function class (FC) II or III (42% and 54%, respectively). All patients were under medical treatment for PH, mostly dual therapy (92%). One or more side effects were noted in 19 patients, while seven reported no side-effects. FU assessment was available in 20 patients after 149 ± 80 days of treatment. Nt-proBNP (median, baseline 1641 pg/mL, FU 1185 pg/mL, P = 0.05) and PVR (mean ± SD, baseline 8.5 ± 4.3 WU, FU 5.6 ± 1.1 WU; P < 0.05) improved significantly. At FU, at least one risk assessment parameter improved in nine patients (45%), all parameters remained in the same risk group in seven patients (35%), and at least one parameter deteriorated in four patients (20%). Interestingly, patients with any side effect throughout the dose titration had a better treatment response than those without any side effects. In our real-life cohort, the majority of patients with PH treated with selexipag showed a stable or improved risk assessment at FU.

Vasoreactivity testing with inhaled NO is recommended for pulmonary arterial hypertension (PAH) because of its therapeutic and prognostic value. Sildenafil has acute pulmonary vasodilating properties, but its diagnostic and prognostic impact in PAH is unknown. Our objective was to compare acute vasodilating responses to sildenafil and those to NO during right heart catheterization and also their prognostic values in patients with PAH. Ninety-nine patients with idiopathic PAH and 99 with associated PAH underwent vasoreactivity testing with NO and sildenafil. Only mild adverse effects of sildenafil, in the form of hypotension, were observed, at a rate of 4.5%. The acute responder rate was 8.1% for NO and 11.6% for sildenafil. The NO-induced response in mean pulmonary arterial pressure and cardiac output correlated with the response to sildenafil. Thirteen patients were long-term responders to calcium channel blockers (CCBs), and 3 of them were correctly identified by acute vasoreactivity test with both drugs. The specificity of the vasoreactivity test for identifying long-term CCB responders was 88.9% for NO and 85.1% for sildenafil testing. A trend toward better survival was found in sildenafil and NO responders, compared with nonresponders. Use of sildenafil for vasoreactivity testing is safe. Sildenafil may be useful as alternative vasoreactivity-testing agent, identifying the same number of long-term CCB responders as NO. However, NO seems to be a more ideal testing drug because of its pharmacologic properties. Moreover, sildenafil vasoreactivity testing might contribute to an improved estimate of prognosis among patients with PAH.

Abstract Hemodynamic measurements provide important parameters for determining prognosis and therapy in patients with pulmonary arterial hypertension (PAH). Current guidelines do not incorporate the possible predictive value of individual changes in hemodynamic variables during the disease time course, and there is no consensus about the time point for hemodynamic reevaluation. We aimed to assess the long-term prognostic value of short-term changes in hemodynamic parameters. The study included 122 patients with PAH from the Giessen Pulmonary Hypertension Registry who underwent hemodynamic evaluation at baseline and at 16 weeks (±2.5 standard deviations [SDs]; range: 4-29 weeks) after initial assessment. At baseline, mean pulmonary vascular resistance (PVR) was 1,109 dyn s cm(-5), and 82% of patients were in World Health Organization (WHO) functional class III or IV. Fifty patients died, and 2 underwent lung transplantation during long-term observation (≤10 years; mean: 4.7 years). Kaplan-Meier estimates for transplant-free survival were 93.3%, 76.1%, 57.8%, and 53.1% at 1, 3, 5, and 7 years, respectively. When assigned to prognostic groups, improvements in cardiac output of >0.22 L min(-1) (hazard ratio [HR]: 2.05; [Formula: see text]) and a decrease in PVR of >176 dyn s cm(-5) (HR: 1.89; [Formula: see text]) at 4-29 weeks were associated with long-term transplant-free survival. Changes in mean pulmonary arterial pressure did not predict long-term prognosis. Of 2 noninvasive parameters assessed in this selected patient group, change in WHO functional class, but not in 6-minute walk distance, predicted long-term prognosis. Short-term assessment of changes in hemodynamic parameters at [Formula: see text] after initial invasive evaluation is useful to determine long-term prognosis in patients with PAH.