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Aims: This prospective, randomized, controlled, multicentre study aimed to evaluate efficacy and safety of exercise training in patients with pulmonary arterial (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH)., Methods and Results: For the first time a specialized PAH/CTEPH rehabilitation programme was implemented in 11 centres across 10 European countries. Out of 129 enrolled patients, 116 patients (58 vs. 58 randomized into a training or usual care control group) on disease-targeted medication completed the study [85 female; mean age 53.6 ± 12.5 years; mean pulmonary arterial pressure 46.6 ± 15.1 mmHg; World Health Organization (WHO) functional class II 53%, III 46%; PAH n = 98; CTEPH n = 18]. Patients of the training group performed a standardized in-hospital rehabilitation with mean duration of 25 days [95% confidence interval (CI) 17-33 days], which was continued at home. The primary endpoint, change of 6-min walking distance, significantly improved by 34.1 ± 8.3 m in the training compared with the control group (95% CI, 18-51 m; P < 0.0001). Exercise training was feasible, safe, and well-tolerated. Secondary endpoints showed improvements in quality of life (short-form health survey 36 mental health 7.3 ± 2.5, P = 0.004), WHO-functional class (training vs. control: improvement 9:1, worsening 4:3; χ2P = 0.027) and peak oxygen consumption (0.9 ± 0.5 mL/min/kg, P = 0.048) compared with the control group., Conclusion: This is the first multicentre and so far the largest randomized, controlled study on feasibility, safety, and efficacy of exercise training as add-on to medical therapy in PAH and CTEPH. Within this study, a standardized specialized training programme with in-hospital start was successfully established in 10 European countries., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2020. For permissions, please email: journals.permissions@oup.com.)

The term idiopathic pulmonary arterial hypertension (IPAH) is used to categorize patients with pre-capillary pulmonary hypertension of unknown origin. There is considerable variability in the clinical presentation of these patients. Using data from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension, we performed a cluster analysis of 841 patients with IPAH based on age, sex, diffusion capacity of the lung for carbon monoxide (DLCO; <45% vs ≥45% predicted), smoking status, and presence of comorbidities (obesity, hypertension, coronary heart disease, and diabetes mellitus). A hierarchical agglomerative clustering algorithm was performed using Ward's minimum variance method. The clusters were analyzed in terms of baseline characteristics; survival; and response to pulmonary arterial hypertension (PAH) therapy, expressed as changes from baseline to follow-up in functional class, 6-minute walking distance, cardiac biomarkers, and risk. Three clusters were identified: Cluster 1 (n = 106; 12.6%): median age 45 years, 76% females, no comorbidities, mostly never smokers, DLCO ≥45%; Cluster 2 (n = 301; 35.8%): median age 75 years, 98% females, frequent comorbidities, no smoking history, DLCO mostly ≥45%; and Cluster 3 (n = 434; 51.6%): median age 72 years, 72% males, frequent comorbidities, history of smoking, and low DLCO. Patients in Cluster 1 had a better response to PAH treatment than patients in the 2 other clusters. Survival over 5 years was 84.6% in Cluster 1, 59.2% in Cluster 2, and 42.2% in Cluster 3 (unadjusted p < 0.001 for comparison between all groups). The population of patients diagnosed with IPAH is heterogenous. This cluster analysis identified distinct phenotypes, which differed in clinical presentation, response to therapy, and survival., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Objectives of this European Respiratory Society task force were to summarise current studies, to develop strategies for future research and to increase availability and awareness of exercise training for pulmonary hypertension (PH) patients.An evidence-based approach with clinical expertise of the task force members, based on both literature search and face-to-face meetings was conducted. The statement summarises current knowledge and open questions regarding clinical effects of exercise training in PH, training modalities, implementation strategies and pathophysiological mechanisms.In studies (784 PH patients in total, including six randomised controlled trials, three controlled trials, 10 prospective cohort studies and four meta-analyses), exercise training has been shown to improve exercise capacity, muscular function, quality of life and possibly right ventricular function and pulmonary haemodynamics. Nevertheless, further studies are needed to confirm these data, to investigate the impact on risk profiles and to identify the most advantageous training methodology and underlying pathophysiological mechanisms.As exercise training appears to be effective, cost-efficient and safe, but is scarcely reimbursed, support from healthcare institutions, commissioners of healthcare and research funding institutions is greatly needed. There is a strong need to establish specialised rehabilitation programmes for PH patients to enhance patient access to this treatment intervention., Competing Interests: Conflict of interest: E. Grünig reports grants and personal fees from Actelion and Bayer/MSD, grants from GSK, United Therapeutics and Novartis, and personal fees from SCOPE, OrPha Swiss GmbH and Zurich Heart House, outside the submitted work. Conflict of interest: C. Eichstaedt has nothing to disclose. Conflict of interest: J-A. Barberà has nothing to disclose. Conflict of interest: N. Benjamin reports personal fees for speaking from Bayer and Actelion, outside the submitted work. Conflict of interest: I. Blanco has nothing to disclose. Conflict of interest: E. Bossone has nothing to disclose. Conflict of interest: A. Cittadini has nothing to disclose. Conflict of interest: G. Coghlan has nothing to disclose. Conflict of interest: P. Corris reports grants and personal fees from Actelion and Bayer, and personal fees from MSD, outside the submitted work. Conflict of interest: M. D'Alto has nothing to disclose. Conflict of interest: A. D'Andrea has nothing to disclose. Conflict of interest: M. Delcroix has nothing to disclose. Conflict of interest: F. de Man has nothing to disclose. Conflict of interest: S. Gaine reports personal fees from Actelion, United Therapeutics, MSD and GSK, outside the submitted work. Conflict of interest: S. Ghio has nothing to disclose. Conflict of interest: S. Gibbs reports grants and personal fees from Actelion, Bayer and MSD, personal fees from Arena, Bellopheron, Acceleron, Complexa and Pfizer, and grants from GSK, Amco and United Therapeutics, during the conduct of the study. Conflict of interest: L. Gumbiene has nothing to disclose. Conflict of interest: L.S. Howard has nothing to disclose. Conflict of interest: M. Johnson reports research grants and personal fees for attendance at meeting and speaking from Actelion, Bayer, GSK and MSD, outside the submitted work. Conflict of interest: E. Jurevičienė has nothing to disclose. Conflict of interest: D.G. Kiely reports grants, personal fees and non-financial support from Actelion, Bayer and GSK, and personal fees and non-financial support from MSD, outside the submitted work. Conflict of interest: G. Kovacs reports personal fees for lecturing, travel support and advisory board work from Actelion and MSD, personal fees for lecturing and advisory board work from GSK, personal fees for lecturing, consultancy and advisory board work from Boehringer Ingelheim and Chiesi, personal fees for lecturing and travel support from Bayer, and personal fees for lecturing from Pfizer and Novartis, outside the submitted work. Conflict of interest: A. MacKenzie has nothing to disclose. Conflict of interest: A.M. Marra reports a grant for Young Researcher (Principal Investigator “Ricerca Finalizzata under 40” n. GR-2016-02364727) from the Italian Healthcare Ministry, and personal fees for lecturing from Bayer Healthcare, during the conduct of the study. Conflict of interest: N. McCaffrey has nothing to disclose. Conflict of interest: P. McCaughey has nothing to disclose. Conflict of interest: R. Naeije has nothing to disclose. Conflict of interest: H. Olschewski reports personal fees and non-financial support from Bayer, MSD, Pfizer and Novartis, grants, personal fees and non-financial support from Actelion, grants from Inventiva, and personal fees from Bellerophon, outside the submitted work; and is a part time employee of Ludwig Boltzmann Insitute for Lung Vascular Research. Conflict of interest: J. Pepke-Zaba or her institution have received educational/research grants and J. Pepke-Zaba serves on advisory boards for Actelion, Merck, Bayer and GSK. Conflict of interest: A. Reis has nothing to disclose. Conflict of interest: M. Santos has nothing to disclose. Conflict of interest: S. Saxer has nothing to disclose. Conflict of interest: R.M. Tulloh has received honoraria and speaker fees from Actelion, Pfizer, Bayer and GSK. Conflict of interest: S. Ulrich reports grants from Swiss National Science Foundation and Zurich Lung, grants and personal fees from Actelion SA and Orpha Swiss, and personal fees from MSD SA, outside the submitted work. Conflict of interest: A. Vonk Noordegraaf reports speaker fees from Actelion, and grants from MSD, Actelion and GSK, outside the submitted work. Conflict of interest: A.J. Peacock reports grants from Actelion, Bayer and GSK, and personal fees from MSD and Arena, outside the submitted work., (Copyright ©ERS 2019.)