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Aims The impact of exercise training on the right heart and pulmonary circulation has not yet been invasively assessed in patients with pulmonary hypertension (PH) and right heart failure. This prospective randomized controlled study investigates the effects of exercise training on peak VO2/kg, haemodynamics, and further clinically relevant parameters in PH patients. Methods and results Eighty-seven patients with pulmonary arterial hypertension and inoperable chronic thrombo-embolic PH (54% female, 56±15 years, 84% World Health Organization functional class III/IV, 53% combination therapy) on stable diseasetargeted medication were randomly assigned to a control and training group. Medication remained unchanged during the study period. Non-invasive assessments and right heart catheterization at rest and during exercise were performed at baseline and after 15 weeks. Primary endpoint was the change in peak VO2/kg. Secondary endpoints included changes in haemodynamics. For missing data, multiple imputation and responder analyses were performed. The study results showed a significant improvement of peak VO2/kg in the training group (difference from baseline to 15 weeks: training ±3.1±2.7 mL/min/kg equals ±24.3% vs. control 20.2±2.3 mL/min/kg equals ±0.9%, P < 0.001). Cardiac index (CI) at rest and during exercise, mean pulmonary arterial pressure, pulmonary vascular resistance, 6 min walking distance, quality of life, and exercise capacity significantly improved by exercise training. Conclusion Low-dose exercise training at 4-7 days/week significantly improved peak VO2/kg, haemodynamics, and further clinically relevant parameters. The improvements of CI at rest and during exercise indicate that exercise training may improve the right ventricular function. Further, large multicentre trials are necessary to confirm these results. [ABSTRACT FROM AUTHOR]

Care of patients with pulmonary arterial hypertension (PAH) needs a multi-facetet concept and measures, including management of adverse reactions, right heart insufficiency as well as information on pregnancy, travels by air, psychosocial support, physical exercise training and prophylaxis by vaccination.Positive study results led to an higher recommendation of specialized exercise training in pulmonary hypertension. Also, the recommendation on iron substitution was amended according to the current evidence.In the current guidelines, special focus was given to the elaboration of recommendations regarding pregnancy, including patient information, contraception and patient management in case of pregnancy.This article aims to provide an overview on the recommendations of general measuremes, special circumstances and patient management according to the ESC/ERS guidelines. Amendments to the guideline recommendations are given as comments from the authors of this article., Competing Interests: M.L.: Unterstützung zur Teilnahme an medizinischen Kongressen/Fort- und Weiterbildungen: MSD, Orpha Swiss, Janssen. Advisory Boards: MSD (bezahlt), Orpha Swiss (unbezahlt).S.H., R.N., F.M.W., S.U., A.F.: kein Interessenkonflikt.E.G. hat Honorare für Vorträge/Konsultationen von Bayer/MSD, Ferrer, GEBRO, GSK, Janssen und OMT erhalten. Forschungsförderung für klinische Studien wurde von Acceleron, Actelion, BayerHealthCare, MSD, Bellerophon, GossamerBio, Janssen, Novartis, OMT, Pfizer, REATE und United Therapeutics erhalten.H.J.K.: Reise-/Übernachtungskosten, Honorare, Forschungsunterstützung von folgenden Firmen: Anamed; AstraZeneca; Bayer; Berlin Chemie/Menarini; Bezirksärztekammer Südbaden, Boehringer Ingelheim; Chiesi; Daiichi-Sankyo; Deutsche Forschungsgemeinschaft; Dräger; Fisher & Paykel Healthcare; Grifols; GSK; Heinen & Löwenstein; Janssen Johnson & Johnson; Lilly; MSD; Mundipharma; Novartis; Pfizer; Portola; Philips Healthcare; Pulmonx; ResMed; Roche; Sanofi Genzyme; Sapio Life; Weinmann.D.R. erhielt Honorare für Vortragstätigkeiten von Janssen und MSD.N.B. erhielt Honorare für Vortragstätigkeit durch das Universitätsspital Zürich., (Thieme. All rights reserved.)