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Document Reviewers: Markus Schwerzmann (ESC Review Coordinator) (Switzerland), Anh-Tuan Dinh-Xuan (ERS Review Coordinator) (France), Andy Bush (United Kingdom), Magdy Abdelhamid (Egypt), Victor Aboyans (France), Eloisa Arbustini (Italy), Riccardo Asteggiano (Italy), Joan-Albert Barberà (Spain), Maurice Beghetti (Switzerland), Jelena Čelutkienė (Lithuania), Maja Cikes (Croatia), Robin Condliffe (United Kingdom), Frances de Man (Netherlands), Volkmar Falk (Germany), Laurent Fauchier (France), Sean Gaine (Ireland), Nazzareno Galié (Italy), Wendy Gin-Sing (United Kingdom), John Granton (Canada), Ekkehard Grünig (Germany), Paul M. Hassoun (United States of America), Merel Hellemons (Netherlands), Tiny Jaarsma (Sweden), Barbro Kjellström (Sweden), Frederikus A. Klok (Netherlands), Aleksandra Konradi (Russian Federation), Konstantinos C. Koskinas (Switzerland), Dipak Kotecha (United Kingdom), Irene Lang (Austria), Basil S. Lewis (Israel), Ales Linhart (Czech Republic), Gregory Y.H. Lip (United Kingdom), Maja-Lisa Løchen (Norway), Alexander G. Mathioudakis (United Kingdom), Richard Mindham (United Kingdom), Shahin Moledina1 (United Kingdom), Robert Naeije (Belgium), Jens Cosedis Nielsen (Denmark), Horst Olschewski (Austria), Isabelle Opitz (Switzerland), Steffen E. Petersen (United Kingdom), Eva Prescott (Denmark), Amina Rakisheva (Kazakhstan), Abilio Reis (Portugal), Arsen D. Ristić (Serbia), Nicolas Roche (France), Rita Rodrigues (Portugal), Christine Selton-Suty (France), Rogerio Souza (Brazil), Andrew J. Swift (United Kingdom), Rhian M. Touyz (Canada/United Kingdom), Silvia Ulrich (Switzerland), Martin R. Wilkins (United Kingdom), and Stephen John Wort (United Kingdom) All experts involved in the development of these guidelines have submitted declarations of interest. These have been compiled in a report and simultaneously published in a supplementary document to the guidelines. The report is also available on the ESC website www.escardio.org/Guidelines See the European Heart Journal online for supplementary data that includes background information and detailed discussion of the data that have provided the basis of the guidelines.

Keywords Guidelines • Pulmonary hypertension • Pulmonary arterial hypertension • Chronic thrombo-embolic pulmonary hypertension • Left heart disease • Congenital heart disease • Lung disease • Connective tissue disease • Endothelin receptor antagonists • Phosphodiesterase type 5 inhibitors • Soluble guanylate cyclase stimulators • Prostacyclin analogues • Prostacyclin receptor agonists • Pulmonary endarterectomy • Balloon pulmonary angioplasty • Lung transplantation

Table of contents

1. Preamble ............................................................................................................. 3624 2. Introduction ...................................................................................................... 3625 2.1. What is new ............................................................................................ 3625 2.2. Methods .................................................................................................... 3635 3. Definitions and classifications .................................................................... 3636 3.1. Definitions ................................................................................................ 3636 3.2. Classifications .......................................................................................... 3637 4. Epidemiology and risk factors .................................................................... 3638 4.1. Group 1, pulmonary arterial hypertension ................................ 3638 4.2. Group 2, pulmonary hypertension associated with left heart disease ................................................................................................................. 3640 4.3. Group 3, pulmonary hypertension associated with lung diseases and/or hypoxia .............................................................................. 3640 4.4. Group 4, pulmonary hypertension associated with chronic pulmonary artery obstruction .................................................................. 3640 4.5. Group 5, pulmonary hypertension with unclear and/or multifactorial mechanisms .......................................................................... 3640 5. Pulmonary hypertension diagnosis .......................................................... 3640 5.1. Diagnosis ................................................................................................... 3640 5.1.1. Clinical presentation ..................................................................... 3640 5.1.2. Electrocardiogram ......................................................................... 3640 5.1.3. Chest radiography ......................................................................... 3641 5.1.4. Pulmonary function tests and arterial blood gases ......... 3641 5.1.5. Echocardiography .......................................................................... 3642

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