2015 ESC/ERS Guidelines for the diagnosis and treatment of ...

1 2015 ESC/ERS Guidelines for thediagnosis and treatment of pulmonaryhypertensionNazzareno Gali 1(ESC Chairperson), Marc Humbert2(ERS Chairperson),Jean-Luc Vachiery3, Simon Gibbs1, Irene Lang1, Adam Torbicki1,G rald Simonneau2, Andrew Peacock2, Anton Vonk Noordegraaf2,Maurice Beghetti4, Ardeschir Ghofrani2, Miguel Angel Gomez Sanchez1,Georg Hansmann4, Walter Klepetko3, Patrizio Lancellotti1, Marco Matucci5,Theresa McDonagh1, Luc A. Pierard1, Pedro T. Trindade1, Maurizio Zompatori6and Marius Hoeper2 Affiliations:1 Representing the European Society of the European the International Society for Heart and Lung theAssociation for European Paediatric and congenital the European League the European Society of Radiology.

2 A full list of collaborators and documentreviewers can be found in the Joint Task Force for the diagnosis and treatment of PulmonaryHypertension of the European Society of Cardiology (ESC) and theEuropean Respiratory Society (ERS)Endorsed by: Association for European Paediatric and congenital Cardiology(AEPC), International Society for Heart and Lung Transplantation (ISHLT)Correspondence:Nazzareno Gali , Dept of Experimental, Diagnostic and Specialty Medicine DIMES, University of Bologna, ViaMassarenti 9, 40138 Bologna, Italy. E-mail: Humbert, Service de Pneumologie, H pital Bic tre, Universit Paris-Sud, Assistance Publique H pitaux deParis, 78 rue du G n ral Leclerc, 94270 Le Kremlin-Bic tre, France.

3 E-mail: @ERSpublications2015 ESC/ERS pulmonary hypertension Guidelines incorporate changes and adaptationsfocusing on clinical Comments inEur Respir J2015; 46: 879 882 [DOI: ].This article has been revised according to the erratum published in the December 2015 issue of theEuropeanRespiratory content of these European Society of Cardiology (ESC) and European Respiratory Society (ERS) Guidelines has beenpublished for personal and educational use only. No commercial use is authorized. No part of the ESC/ERS Guidelinesmay be translated or reproduced in any form without written permission from the ESC and/or ERS.

4 Permission can beobtained upon submission of a written request to Oxford University Press, the publisher of theEuropean Heart Journalor from the European Respiratory Society, the publisher ofEuropean Respiratory Journaland the party authorized tohandle such permissions on behalf of the ESC and article is being published concurrently in theEuropean Heart Journal( ) and theEuropeanRespiratory Journal( ). The articles are identical except for minor stylistic and spellingdifferences in keeping with each journal s style. Either citation can be used when citing this of interest: Disclosures can be found alongside the online version of this article at on behalf of the European Society of Cardiology and European Respiratory Society.

5 All rights reserved. 2015 European Society of Cardiology & European Respiratory Respir J 2015; 46: 903 975 | DOI: GUIDELINESPULMONARY HYPERTENSIONT able of ContentsAbbreviation and acronyms1. Preamble .. 9052. Introduction .. 9073. Definitions and classifications .. Definitions .. 9084. Epidemiology and genetics of pulmonary hypertension .. Epidemiology and risk 9115. Pulmonary hypertension diagnosis .. Clinical presentation .. Electrocardiogram .. Chest radiograph .. Pulmonary function tests and arterial blood gases.

6 Ventilation/perfusion lung High-resolution computed tomography, contrast enhancedcomputed tomography, and pulmonary angiography .. Cardiac magnetic resonance imaging .. Blood tests and Abdominal ultrasound Right heart catheterization and Genetic testing .. Diagnostic algorithm .. 9196. Pulmonary arterial hypertension (group 1) .. Clinical Evaluation of severity .. Clinical parameters, imaging and haemodynamics .. Exercise capacity .. Biochemical Comprehensive prognostic evaluation and Definition of patient treatment goals and follow-up Therapy.

7 General measures .. Physical activity and supervised rehabilitation .. Pregnancy, birth control, and post-menopausalhormonal therapy .. Elective Infection Psychosocial support .. Adherence to treatments .. Travel .. Genetic Supportive therapy .. Oral anticoagulants .. Diuretics .. Oxygen .. Digoxin and other cardiovascular drugs .. Anaemia and iron Specific drug therapy .. Calcium channel blockers .. Endothelin receptor antagonists .. Phosphodiesterase type 5 inhibitors and guanylate Prostacyclin analogues and prostacyclin receptor agonists.

8 Experimental compounds and strategies .. Combination therapy .. Drug interactions .. Balloon atrial septostomy .. Advanced right ventricular Intensive care unit management .. Right ventricle treatment algorithm .. diagnosis and treatment of pulmonary arterialhypertension Arrhythmias .. Mechanical End of life care and ethical issues .. 9407. Specific pulmonary (arterial) hypertension subsets .. Paediatric pulmonary arterial hypertension .. Therapy .. Pulmonary arterial hypertension associated with adultcongenital heart disease.

9 Therapy .. Pulmonary arterial hypertension associated withconnective tissue Therapy .. Pulmonary arterial hypertension associated with portal hypertension .. Therapy .. Pulmonary arterial hypertension associated with humanimmunodeficiency virus infection .. Therapy .. Pulmonary veno-occlusive disease and pulmonarycapillary haemangiomatosis .. Therapy .. 9498. Pulmonary hypertension due to left heart disease (group 2) .. Therapy .. 9529. Pulmonary hypertension due to lung diseases and/or hypoxia (group 3).

10 Therapy .. 95410. Chronic thromboembolic pulmonary hypertension (group 4).. Therapy .. Medical .. Interventional .. 95711. Pulmonary hypertension with unclear and/ormultifactorial mechanisms (group 5) .. 95812. Definition of a pulmonary hypertension referral centre .. 95813. To do and not to do messages from the Guidelines .. 96014. Appendix ..96015. Web 96216. : The ESC/ERS Guidelines represent the views of the ESC and ERS and were produced after carefulconsideration of the scientific and medical knowledge and the evidence available at the time of their publication.