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Acute decompensated pulmonary hypertension

Laurent Savale, Jason Weatherald, Xavier Jaïs, Constance Vuillard, Athénaïs Boucly, Mitja Jevnikar, David Montani, Olaf Mercier, Gerald Simonneau, Elie Fadel, Olivier Sitbon, Marc Humbert
European Respiratory Review 2017 26: 170092; DOI: 10.1183/16000617.0092-2017
Laurent Savale
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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  • For correspondence: laurent.savale@bct.aphp.fr
Jason Weatherald
4University of Calgary, Dept of Medicine, Division of Respirology, Calgary, AB, Canada
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Xavier Jaïs
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Constance Vuillard
2AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
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Athénaïs Boucly
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Mitja Jevnikar
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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David Montani
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Olaf Mercier
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
5Depts of Thoracic and Vascular Surgery and Heart-Lung Transplantation, Marie Lannelongue Hospital, Le Plessis Robinson, France
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Gerald Simonneau
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Elie Fadel
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
5Depts of Thoracic and Vascular Surgery and Heart-Lung Transplantation, Marie Lannelongue Hospital, Le Plessis Robinson, France
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Olivier Sitbon
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Marc Humbert
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Abstract

Acute right heart failure in chronic precapillary pulmonary hypertension is characterised by a rapidly progressive syndrome with systemic congestion resulting from impaired right ventricular filling and/or reduced right ventricular flow output. This clinical picture results from an imbalance between the afterload imposed on the right ventricle and its adaptation capacity. Acute decompensated pulmonary hypertension is associated with a very poor prognosis in the short term. Despite its major impact on survival, its optimal management remains very challenging for specialised centres, without specific recommendations. Identification of trigger factors, optimisation of fluid volume and pharmacological support to improve right ventricular function and perfusion pressure are the main therapeutic areas to consider in order to improve clinical condition. At the same time, specific management of pulmonary hypertension according to the aetiology is mandatory to reduce right ventricular afterload. Over the past decade, the development of extracorporeal life support in refractory right heart failure combined with urgent transplantation has probably contributed to a significant improvement in survival for selected patients. However, there remains a considerable need for further research in this field.

Abstract

Acute decompensated PH is a life-threatening condition requiring specific management in a specialised centre http://ow.ly/non530fkhmA

Footnotes

  • Number 2 in the Series “Acute exacerbations in pulmonary medicine” Edited by Michael Kreuter and Vincent Cottin

  • Previous articles in this series: No. 1: Kondoh Y, Cottin V, Brown KK. Recent lessons learned in the management of acute exacerbation of idiopathic pulmonary fibrosis. Eur Respir Rev 2017; 26: 170050.

  • Conflict of interest: Disclosures can be found alongside this article at err.ersjournals.com

  • Provenance: Commissioned article, peer reviewed.

  • Received August 18, 2017.
  • Accepted September 17, 2017.
  • Copyright ©ERS 2017.

ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

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Acute decompensated pulmonary hypertension
Laurent Savale, Jason Weatherald, Xavier Jaïs, Constance Vuillard, Athénaïs Boucly, Mitja Jevnikar, David Montani, Olaf Mercier, Gerald Simonneau, Elie Fadel, Olivier Sitbon, Marc Humbert
European Respiratory Review Dec 2017, 26 (146) 170092; DOI: 10.1183/16000617.0092-2017

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Acute decompensated pulmonary hypertension
Laurent Savale, Jason Weatherald, Xavier Jaïs, Constance Vuillard, Athénaïs Boucly, Mitja Jevnikar, David Montani, Olaf Mercier, Gerald Simonneau, Elie Fadel, Olivier Sitbon, Marc Humbert
European Respiratory Review Dec 2017, 26 (146) 170092; DOI: 10.1183/16000617.0092-2017
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  • Article
    • Abstract
    • Abstract
    • Introduction
    • Pathophysiology of right ventricular failure in pulmonary hypertension
    • Definition and characteristics of acute decompensated pulmonary hypertension
    • Survival and prognostic factors of acute decompensated pulmonary hypertension
    • Management of acute decompensated pulmonary hypertension
    • Management of refractory right ventricular failure
    • Monitoring of acute right ventricular failure in pulmonary hypertension
    • Conclusion
    • Disclosures
    • Footnotes
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  • Pulmonary vascular disease
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Acute Exacerbations in Pulmonary Medicine

  • Exacerbations of COPD
  • Management of acute exacerbation of IPF
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