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ERS statement on exercise training and rehabilitation in patients with severe chronic pulmonary hypertension

Ekkehard Grünig, Christina Eichstaedt, Joan-Albert Barberà, Nicola Benjamin, Isabel Blanco, Eduardo Bossone, Antonio Cittadini, Gerry Coghlan, Paul Corris, Michele D'Alto, Antonello D'Andrea, Marion Delcroix, Frances de Man, Sean Gaine, Stefano Ghio, Simon Gibbs, Lina Gumbiene, Luke S. Howard, Martin Johnson, Elena Jurevičienė, David G. Kiely, Gabor Kovacs, Alison MacKenzie, Alberto M. Marra, Noel McCaffrey, Paul McCaughey, Robert Naeije, Horst Olschewski, Joanna Pepke-Zaba, Abílio Reis, Mário Santos, Stéphanie Saxer, Robert M. Tulloh, Silvia Ulrich, Anton Vonk Noordegraaf, Andrew J. Peacock
European Respiratory Journal 2018; DOI: 10.1183/13993003.00332-2018
Ekkehard Grünig
1Centre for Pulmonary Hypertension, Thoraxclinic at the University Hospital Heidelberg, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Heidelberg, Germany
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Christina Eichstaedt
1Centre for Pulmonary Hypertension, Thoraxclinic at the University Hospital Heidelberg, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Heidelberg, Germany
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Joan-Albert Barberà
2Hospital Clinic IDIBAPS, University of Barcelona, Barcelona and Biomedical Research Networking Center on Respiratory Diseases, Madrid, Spain
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Nicola Benjamin
1Centre for Pulmonary Hypertension, Thoraxclinic at the University Hospital Heidelberg, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Heidelberg, Germany
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Isabel Blanco
2Hospital Clinic IDIBAPS, University of Barcelona, Barcelona and Biomedical Research Networking Center on Respiratory Diseases, Madrid, Spain
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Eduardo Bossone
3Department of Cardiac Surgery, University Hospital Salerno, Salerno, Italy
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Antonio Cittadini
3Department of Cardiac Surgery, University Hospital Salerno, Salerno, Italy
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Gerry Coghlan
4Pulmonary Hypertension Unit, Royal Free Hospital, University College London, London, United Kingdom
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Paul Corris
5Department of Respiratory Medicine, Freeman Hospital, University of Newcastle, Newcastle upon Tyne, United Kingdom
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Michele D'Alto
6Department of Cardiology, Second University of Naples - Monaldi Hospital, Naples, Italy
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Antonello D'Andrea
6Department of Cardiology, Second University of Naples - Monaldi Hospital, Naples, Italy
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Marion Delcroix
7Department of Pneumology, University Hospital Leuven, Leuven, Belgium
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  • ORCID record for Marion Delcroix
Frances de Man
8Department of Pulmonology, VU University Medical Center Amsterdam, Amsterdam, Netherlands
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Sean Gaine
9Department of Respiratory Medicine, Mater Misericordiae University Hospital, Dublin, Ireland
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Stefano Ghio
10Divisione di Cardiologia, Fondazione IRCCS Policlinico S Matteo, Pavia, Italy
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Simon Gibbs
11Faculty of Medicine, Imperial College London, National Heart and Lung Institute, London, United Kingdom
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Lina Gumbiene
12Competence Centre of Pulmonary Hypertension, Vilnius University Hospital Santaros Klinikos, Vilnius, Lithuania
13Faculty of Medicine, Vilnius University, Vilnius, Lithuania
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Luke S. Howard
14Hammersmith Hospital, Imperial College NHS Trust, London, United Kingdom
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Martin Johnson
15Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, United Kingdom
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Elena Jurevičienė
12Competence Centre of Pulmonary Hypertension, Vilnius University Hospital Santaros Klinikos, Vilnius, Lithuania
13Faculty of Medicine, Vilnius University, Vilnius, Lithuania
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David G. Kiely
16Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom
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Gabor Kovacs
17Department of Internal Medicine, Div. Pulmonology, Medical University of Graz, Graz, Austria
18Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria
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Alison MacKenzie
15Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, United Kingdom
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Alberto M. Marra
19Department of Cardiovascular Imaging, IRCCS S.D.N., Naples, Italy
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Noel McCaffrey
9Department of Respiratory Medicine, Mater Misericordiae University Hospital, Dublin, Ireland
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Paul McCaughey
15Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, United Kingdom
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Robert Naeije
20Department of Cardiology, University Clinic Brussels - Erasme Hospital, Brussels, Belgium
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Horst Olschewski
17Department of Internal Medicine, Div. Pulmonology, Medical University of Graz, Graz, Austria
18Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria
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Joanna Pepke-Zaba
21Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, United Kingdom
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Abílio Reis
22Department of Internal Medicine, Hospital Geral de Santo António, Porto, Portugal
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Mário Santos
22Department of Internal Medicine, Hospital Geral de Santo António, Porto, Portugal
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Stéphanie Saxer
23Centre for Pulmonary Hypertension, University Hospital Zurich, Zurich, Switzerland
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Robert M. Tulloh
24Department of Congenital Heart Disease, Bristol Royal Hospital for Children, Bristol, United Kingdom
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Silvia Ulrich
23Centre for Pulmonary Hypertension, University Hospital Zurich, Zurich, Switzerland
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Anton Vonk Noordegraaf
8Department of Pulmonology, VU University Medical Center Amsterdam, Amsterdam, Netherlands
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Andrew J. Peacock
15Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, United Kingdom
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Abstract

Objectives of this ERS 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 pathophysiologic mechanisms.

In studies with 784 PH patients in total, including 6 randomised controlled trials, 3 controlled trials, 10 prospective cohort studies, and 4 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 health care institutions, commissioners of health care and research funding institutions are of high need. There is a strong need to establish specialised rehabilitation programmes for PH patients to enhance patient access to this treatment intervention.

Footnotes

This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.

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. Jureviciene 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 from Lecture Fee 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.

This is a PDF-only article. Please click on the PDF link above to read it.

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ERS statement on exercise training and rehabilitation in patients with severe chronic pulmonary hypertension
Ekkehard Grünig, Christina Eichstaedt, Joan-Albert Barberà, Nicola Benjamin, Isabel Blanco, Eduardo Bossone, Antonio Cittadini, Gerry Coghlan, Paul Corris, Michele D'Alto, Antonello D'Andrea, Marion Delcroix, Frances de Man, Sean Gaine, Stefano Ghio, Simon Gibbs, Lina Gumbiene, Luke S. Howard, Martin Johnson, Elena Jurevičienė, David G. Kiely, Gabor Kovacs, Alison MacKenzie, Alberto M. Marra, Noel McCaffrey, Paul McCaughey, Robert Naeije, Horst Olschewski, Joanna Pepke-Zaba, Abílio Reis, Mário Santos, Stéphanie Saxer, Robert M. Tulloh, Silvia Ulrich, Anton Vonk Noordegraaf, Andrew J. Peacock
European Respiratory Journal Jan 2018, 1800332; DOI: 10.1183/13993003.00332-2018

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ERS statement on exercise training and rehabilitation in patients with severe chronic pulmonary hypertension
Ekkehard Grünig, Christina Eichstaedt, Joan-Albert Barberà, Nicola Benjamin, Isabel Blanco, Eduardo Bossone, Antonio Cittadini, Gerry Coghlan, Paul Corris, Michele D'Alto, Antonello D'Andrea, Marion Delcroix, Frances de Man, Sean Gaine, Stefano Ghio, Simon Gibbs, Lina Gumbiene, Luke S. Howard, Martin Johnson, Elena Jurevičienė, David G. Kiely, Gabor Kovacs, Alison MacKenzie, Alberto M. Marra, Noel McCaffrey, Paul McCaughey, Robert Naeije, Horst Olschewski, Joanna Pepke-Zaba, Abílio Reis, Mário Santos, Stéphanie Saxer, Robert M. Tulloh, Silvia Ulrich, Anton Vonk Noordegraaf, Andrew J. Peacock
European Respiratory Journal Jan 2018, 1800332; DOI: 10.1183/13993003.00332-2018
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