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      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.

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          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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              Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension.

              Hiromi Matsubara, Hiroshi Mikouchi, Mitsuru Munemasa … (2012)
              Although balloon pulmonary angioplasty (BPA) for inoperable patients with chronic thromboembolic pulmonary hypertension was first reported over a decade ago, its clinical application has been restricted because of limited efficacy and complications. We have refined the procedure of BPA to maximize its clinical efficacy. Sixty-eight consecutive patients with inoperable chronic thromboembolic pulmonary hypertension underwent BPA. We evaluated pulmonary artery diameters and determined the appropriate balloon size by using intravascular ultrasound. We performed BPA in a staged fashion over multiple, separate procedures to maximize efficacy and reduce the risk of reperfusion pulmonary injury. A total of 4 (2-8) sessions were performed in each patient, and the number of vessels dilated per session was 3 (1-14). The World Health Organization functional class improved from 3 to 2 (P<0.01), and mean pulmonary arterial pressure was decreased from 45.4 ± 9.6 to 24.0 ± 6.4 mm Hg (P<0.01). One patient died because of right heart failure 28 days after BPA. During follow-up for 2.2 ± 1.4 years after the final BPA, another patient died of pneumonia, and the remaining 66 patients are alive. In 57 patients who underwent right heart catheterization at follow-up, improvement of mean pulmonary arterial pressure was maintained (24.0 ± 5.8 mm Hg at 1.0 ± 0.9 years). Forty-one patients (60%) developed reperfusion pulmonary injury after BPA, but mechanical ventilation was required in only 4 patients. Our refined BPA procedure improves clinical status and hemodynamics of inoperable patients with chronic thromboembolic pulmonary hypertension, with a low mortality. A refined BPA procedure could be considered as a therapeutic approach for patients with inoperable chronic thromboembolic pulmonary hypertension.
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                Author and article information

                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
                https://orcid.org/0000-0001-8394-9809
                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
                Copyright © Copyright ©ERS 2019
                License:

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

                History
                Date received : 06 October 2018
                Date accepted : 09 October 2018
                Related
                Categories
                Subject: Series
                Subject: World Symposium on Pulmonary Hypertension

                ScienceOpen disciplines: Respiratory medicine
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                ScienceOpen disciplines: Respiratory medicine

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