Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, either symptomatic or not. The occlusion of proximal pulmonary arteries by fibrotic intravascular material, in combination with a secondary microvasculopathy of vessels <500 µm, leads to increased pulmonary vascular resistance and progressive right heart failure. The mechanism responsible for the transformation of red clots into fibrotic material remnants has not yet been elucidated. In patients with pulmonary hypertension, the diagnosis is suspected when a ventilation/perfusion lung scan shows mismatched perfusion defects, and confirmed by right heart catheterisation and vascular imaging. Today, in addition to lifelong anticoagulation, treatment modalities include surgery, angioplasty and medical treatment according to the localisation and characteristics of the lesions.This statement outlines a review of the literature and current practice concerning diagnosis and management of CTEPH. It covers the definitions, diagnosis, epidemiology, follow-up after acute pulmonary embolism, pathophysiology, treatment by pulmonary endarterectomy, balloon pulmonary angioplasty, drugs and their combination, rehabilitation and new lines of research in CTEPH.It represents the first collaboration of the European Respiratory Society, the International CTEPH Association and the European Reference Network-Lung in the pulmonary hypertension domain. The statement summarises current knowledge, but does not make formal recommendations for clinical practice.

2nd Department of Medicine Dept of Cardiovascular Medicine 1st Faculty of Medicine Charles University and General University Hospital Prague Prague Czech Republic

BREATHE Dept CHROMETA KU Leuven Leuven Belgium

Clinical Dept of Respiratory Diseases Pulmonary Hypertension Center UZ Leuven Leuven Belgium

Co chair

Dept of Medicine Imperial College London London UK

Dept of Medicine Thrombosis and Hemostasis Leiden University Medical Center Leiden The Netherlands

Dept of Pneumology Kerckhoff Clinic Bad Nauheim Bad Nauheim Germany

Dept of Pulmonary Circulation Thrombo embolic Diseases and Cardiology Center of Postgraduate Medical Education ECZ Otwock Otwock Poland

Dept of Pulmonary Medicine Amsterdam UMC Vrije Universiteit Amsterdam Amsterdam Cardiovascular Sciences Amsterdam The Netherlands

Dept of Radiology Imperial College Hospitals NHS Trusts London UK

Dept of Thoracic Surgery Kerckhoff Clinic Bad Nauheim Bad Nauheim Germany

Division of Pulmonary Critical Care and Sleep Medicine University of California San Diego La Jolla CA USA

Equal contribution

Hannover Medical School Hannover Germany

Institute for Diagnostic and Interventional Radiology Hannover Medical School Hannover Germany

Marie Lannelongue Hospital Paris South University Le Plessis Robinson France

Medical University of Vienna Vienna Austria

National Cerebral and Cardiovascular Centre Osaka Japan

National Hospital Organization Okayama Medical Center Okayama Japan

Patient representative

PHA Europe Hungary

PHA Ireland Dublin Ireland

Royal Papworth Hospital Cambridge University Hospital Cambridge UK

Section editors

Sulpizio Cardiovascular Centre University of California San Diego CA USA

Thoraxklinik Heidelberg at Heidelberg University Hospital Heidelberg Germany

Unit of Cardiac Surgery Intrathoracic Transplantation and Pulmonary Hypertension University of Pavia School of Medicine Foundation 1 R C C S Policlinico San Matteo Pavia Italy

Université Paris Saclay; Inserm UMR_S 999 Service de Pneumologie Hôpital Bicêtre Le Kremlin Bicêtre France

University of Bologna Bologna Italy

University of Giessen and Marburg Lung Center German Center of Lung Research Giessen Germany

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