Chronic thromboembolic pulmonary hypertension

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Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy, which is the guideline recommended treatment. For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention.

Abstract

State of the art and research perspectives in chronic thromboembolic pulmonary hypertension, including treatment algorithm http://ow.ly/C3Iy30mfUly

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Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry.

Marion Delcroix, Irene Lang, Joanna Pepke-Zaba … (2016)

Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension.

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Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry.

Eckhard Mayer, David Jenkins, Jaroslav Lindner … (2011)

Pulmonary endarterectomy is a curative surgical treatment option for the majority of patients with chronic thromboembolic pulmonary hypertension. The current surgical management and postoperative outcome of patients enrolled in an international registry on chronic thromboembolic pulmonary hypertension were investigated. The registry included newly diagnosed (≤6 months) consecutive patients with chronic thromboembolic pulmonary hypertension from February 2007 to January 2009. A total of 679 patients were registered from 1 Canadian and 26 European centers, of whom 386 (56.8%) underwent surgery. The median age of patients undergoing surgery was 60 years, and 54.1% were male. Previous pulmonary embolism was confirmed for 79.8% of patients. Perioperative complications occurred in 189 patients (49.2%): infection (18.8%), persistent pulmonary hypertension (16.7%), neurologic (11.2%) or bleeding (10.2%) complications, pulmonary reperfusion edema (9.6%), pericardial effusion (8.3%), need for extracorporeal membrane oxygenation (3.1%), and in-hospital mortality due to perioperative complications (4.7%). Documented 1-year mortality was 7%. Preoperative exercise capacity was predictive of 1-year mortality. Postoperative pulmonary vascular resistance predicted in-hospital and 1-year mortality. In patients evaluated within 1 year after surgery, the median pulmonary vascular resistance had decreased from 698 to 235 dyn x s x cm(-5) (95% confidence limit, 640-874 and 211-255, respectively, n = 70) and the median 6-minute walk distance had increased from 362 to 459 m (95% confidence limit, 340-399 and 440-473, respectively, n = 168). New York Heart Association functional class improved with most patients progressing from class III/IV to class I/II. Pulmonary endarterectomy is associated with a low in-hospital mortality rate and improvements in hemodynamics and exercise capacity. Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

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Chronic thromboembolic pulmonary hypertension.

Marion Delcroix, Eckhard Mayer, Irene Lang … (2013)

Since the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension (CTEPH). Emerging clinical data and advances in technology have led to reinforcing and updated guidance on diagnostic approaches to pulmonary hypertension, guidelines that we hope will lead to better recognition and more timely diagnosis of CTEPH. We have new data on treatment practices across international boundaries as well as long-term outcomes for CTEPH patients treated with or without pulmonary endarterectomy. Furthermore, we have expanded data on alternative treatment options for select CTEPH patients, including data from multiple clinical trials of medical therapy, including 1 recent pivotal trial, and compelling case series of percutaneous pulmonary angioplasty. Lastly, we have garnered more experience, and on a larger international scale, with pulmonary endarterectomy, which is the treatment of choice for operable CTEPH. This report overviews and highlights these important interval developments as deliberated among our task force of CTEPH experts and presented at the 2013 World Symposium on Pulmonary Hypertension in Nice, France. Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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Journal

Journal ID (nlm-ta): Eur Respir J

Journal ID (iso-abbrev): Eur. Respir. J

Journal ID (publisher-id): ERJ

Journal ID (hwp): erj

Title: The European Respiratory Journal

Publisher: European Respiratory Society

ISSN (Print): 0903-1936

ISSN (Electronic): 1399-3003

Publication date Collection: January 2019

Publication date (Electronic): 24 January 2019

Volume: 53

Issue: 1

Electronic Location Identifier: 1801915

Affiliations

[1 ]Dept of Medicine, University of California San Diego, La Jolla, CA, USA

[2 ]Dept of Respiratory Diseases, University Hospitals of Leuven and Respiratory Division, Dept CHROMETA, KU Leuven – University of Leuven, Leuven, Belgium

[3 ]Université Paris-Sud, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Service de Pneumologie, Département Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Hôpital Bicêtre, Le Kremlin-Bicêtre, France

[4 ]Cardiovascular and Thoracic Surgery, University of California San Diego, La Jolla, CA, USA

[5 ]National Hospital Organization Okayama Medical Center, Okayama, Japan

[6 ]Kerckhoff Clinic Bad Nauheim, University of Giessen, Bad Nauheim, Germany

[7 ]Division of Advanced Medical Research in Pulmonary Hypertension, Dept of Pulmonary Circulation, National Cerebral and Cardiovascular Center, Osaka, Japan

[8 ]Dept of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA

[9 ]University of Giessen and Marburg Lung Centre (UGMLC), Justus-Liebig University Giessen and Member of the German Center for Lung Research (DZL), Giessen, Germany

[10 ]Dept of Medicine, Imperial College London, London, UK

[11 ]Royal Papworth Hospital, Cambridge, UK

[12 ]These two authors contributed equally to this work

Author notes

Nick H. Kim, Dept of Medicine, University of California San Diego, 9300 Campus Point Drive, MC 7381, La Jolla, CA 92037, USA. E-mail: h33kim@ 123456ucsd.edu

Author information

Marion Delcroix https://orcid.org/0000-0001-8394-9809

Hossein-Ardeschir Ghofrani https://orcid.org/0000-0002-2029-4419

Article

Publisher ID: ERJ-01915-2018

DOI: 10.1183/13993003.01915-2018

PMC ID: 6351341

PubMed ID: 30545969

SO-VID: 7445e066-720f-4e19-93da-4f31a8e312d6

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This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

Chronic thromboembolic pulmonary hypertension

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Most cited references 74

Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry.

Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry.

Chronic thromboembolic pulmonary hypertension.

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