{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T06:41:47Z","timestamp":1792651307789,"version":"4.5.0"},"reference-count":30,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"17","license":[{"start":{"date-parts":[[2024,10,22]],"date-time":"2024-10-22T00:00:00Z","timestamp":1729555200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation"],"published-print":{"date-parts":[[2024,10,22]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND:<\/jats:title>\n                    <jats:p>The European Chronic Thromboembolic Pulmonary Hypertension (CTEPH) registry, conducted between 2007 and 2012, reported the major impact of pulmonary endarterectomy (PEA) on the long-term survival of patients with CTEPH. Since then, 2 additional treatments for inoperable CTEPH have become available: balloon pulmonary angioplasty (BPA), and an approved oral drug therapy with the guanylate cyclase stimulator riociguat. The current registry aimed to evaluate the effect of these new therapeutic approaches in a worldwide context.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS:<\/jats:title>\n                    <jats:p>Participation in this international global registry included 34 centers in 20 countries. Between February 2015 and September 2016, 1009 newly diagnosed, consecutive patients were included and followed until September 2019.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS:<\/jats:title>\n                    <jats:p>\n                      Overall, 605 patients (60%) underwent PEA and 185 (18%) underwent BPA; 76% of the 219 remaining patients not receiving mechanical intervention (ie, neither PEA nor BPA) were treated with pulmonary hypertension drugs. Of patients undergoing PEA and BPA, 38% and 78% also received drugs for pulmonary hypertension, respectively. Median age at diagnosis was higher in the BPA and No PEA\/BPA groups than in the PEA group: 66 and 69, respectively, versus 60 years. Pulmonary vascular resistance (PVR) was similar in all groups, with an average of 643 dynes.s.cm\n                      <jats:sup>\u22125<\/jats:sup>\n                      . During the observation period (&gt;3 years; \u22645.6 years), death was reported in 7%, 11%, and 27% of patients treated by PEA and BPA, and those receiving no mechanical intervention (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt;0.001). In Kaplan-Meier analysis, 3-year survival was 94%, 92%, and 71% in the 3 groups, respectively. PEA 3-year survival improved by 5% from that observed between 2007 and 2012. There was no survival difference in patients receiving vitamin K antagonists and non\u2013vitamin K oral anticoagulants (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      =0.756). In Cox regression, reduced mortality was associated with: PEA and BPA in the global cohort; history of venous thromboembolism and lower PVR in the PEA group; lower right atrial pressure in the BPA group; and use of pulmonary hypertension drugs, oxygen therapy, and lower right atrial pressure, as well as functional class in the group receiving no mechanical intervention.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS:<\/jats:title>\n                    <jats:p>This second international CTEPH registry reveals important improvement in patient survival since the introduction of BPA and an approved drug for pulmonary hypertension. The type of anticoagulation regimen did not influence survival.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>REGISTRATION:<\/jats:title>\n                    <jats:p>\n                      URL:\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"https:\/\/www.clinicaltrials.gov\">https:\/\/www.clinicaltrials.gov<\/jats:ext-link>\n                      ; Unique identifier: NCT02656238.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circulationaha.124.068610","type":"journal-article","created":{"date-parts":[[2024,9,17]],"date-time":"2024-09-17T13:02:04Z","timestamp":1726578124000},"page":"1354-1365","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":105,"title":["Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy"],"prefix":"10.1161","volume":"150","clinical-trial-number":[{"clinical-trial-number":"nct02656238","registry":"10.18810\/clinical-trials-gov"}],"author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8394-9809","authenticated-orcid":true,"given":"Marion","family":"Delcroix","sequence":"first","affiliation":[{"name":"Clinical Department of Respiratory Diseases, University Hospitals of Leuven, Belgium (M.D., R.Q.)."},{"name":"Laboratory of Respiratory Diseases and Thoracic Surgery, Department of Chronic Diseases and Metabolism, University of Leuven, Belgium (M.D., R.Q.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanna","family":"Pepke-Zaba","sequence":"additional","affiliation":[{"name":"Royal Papworth Hospital, Cambridge, UK (J.P-Z., S.P.H., D.P.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7824-5249","authenticated-orcid":true,"given":"Andrea M.","family":"D\u2019Armini","sequence":"additional","affiliation":[{"name":"Division of Cardiac Surgery II and Chronic Thromboembolic Pulmonary Hypertension Center, Cardio-Thoracic and Vascular Department, Foundation IRCCS Policlinico San Matteo, Pavia, Italy (A.M.D.)."},{"name":"University of Pavia, School of Medicine, Italy (A.M.D.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9290-4589","authenticated-orcid":true,"given":"Elie","family":"Fadel","sequence":"additional","affiliation":[{"name":"Department of Thoracic and Vascular Surgery and Heart-Lung Transplantation, Marie Lannelongue Hospital, Paris-Saclay University, Le Plessis Robinson, France (E.F.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2238-9574","authenticated-orcid":true,"given":"Stefan","family":"Guth","sequence":"additional","affiliation":[{"name":"Department of Thoracic Surgery, Kerckhoff Heart and Lung Center, Bad Nauheim, Germany (S.G., C.B.W., E.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3530-3808","authenticated-orcid":true,"given":"Stephen P.","family":"Hoole","sequence":"additional","affiliation":[{"name":"Royal Papworth Hospital, Cambridge, UK (J.P-Z., S.P.H., D.P.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David P.","family":"Jenkins","sequence":"additional","affiliation":[{"name":"Royal Papworth Hospital, Cambridge, UK (J.P-Z., S.P.H., D.P.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0184-6502","authenticated-orcid":true,"given":"David G.","family":"Kiely","sequence":"additional","affiliation":[{"name":"NIHR Biomedical Research Centre Sheffield and Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, UK (D.G.K.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4702-650X","authenticated-orcid":true,"given":"Nick H.","family":"Kim","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine (N.H.K.), University of California, San Diego, La Jolla."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael M.","family":"Madani","sequence":"additional","affiliation":[{"name":"Cardiovascular and Thoracic Surgery (M.M.M.), University of California, San Diego, La Jolla."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiromi","family":"Matsubara","sequence":"additional","affiliation":[{"name":"National Hospital Organization Okayama Medical Center, Japan (H.M., A.O.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuhiko","family":"Nakayama","sequence":"additional","affiliation":[{"name":"Division of Cardiovascular Medicine, Department of Internal Medicine, 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Diseases, University Hospitals of Leuven, Belgium (M.D., R.Q.)."},{"name":"Laboratory of Respiratory Diseases and Thoracic Surgery, Department of Chronic Diseases and Metabolism, University of Leuven, Belgium (M.D., R.Q.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roela","family":"Sadushi-Kolici","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Austria (R.S.-K., I.M.L.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00e9rald","family":"Simonneau","sequence":"additional","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris, Service de Pneumologie, H\u00f4pital Bic\u00eatre, Universit\u00e9 Paris-Sud, Laboratoire d\u2019Excellence en Recherche sur le M\u00e9dicament et Innovation Th\u00e9rapeutique, and Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 999, Le Kremlin-Bic\u00eatre, France 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