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著者: Marion Delcroix, Adam Torbicki, Deepa Gopalan, Olivier Sitbon, Frederikus A Klok, Irene Lang, David Jenkins, Nick H Kim, Marc Humbert, Xavier Jais, Anton Vonk Noordegraaf, Joanna Pepke-Zaba, Philippe Brénot, Peter Dorfmuller, Elie Fadel, Hossein-Ardeschir Ghofrani, Marius M Hoeper, Pavel Jansa, Michael Madani, Hiromi Matsubara, Takeshi Ogo, Ekkehard Grünig, Andrea D'Armini, Nazzareno Galie, Bernhard Meyer, Patrick Corkery, Gergely Meszaros, Eckhard Mayer, Gérald Simonneau
雑誌名: Eur Respir J. 2021 Jun;57(6). doi: 10.1183/13993003.02828-2020. Epub 2021 Jun 17.
Abstract/Text
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.
Copyright ©ERS 2021. For reproduction rights and permissions contact permissions@ersnet.org.
PMID 33334946 Eur Respir J. 2021 Jun;57(6). doi: 10.1183/13993003.02828-2020. Epub 2021 Jun 17.
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