{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T22:28:20Z","timestamp":1793831300840,"version":"4.6.1"},"reference-count":115,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2023,2,16]],"date-time":"2023-02-16T00:00:00Z","timestamp":1676505600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition in which an organic thrombus remains in the pulmonary artery (PA) even after receiving anticoagulation therapy for more than 3 months and is complicated by pulmonary hypertension (PH), leading to right-sided heart failure and death. CTEPH is a progressive pulmonary vascular disease with a poor prognosis if left untreated. The standard treatment for CTEPH is pulmonary endarterectomy (PEA), which is usually performed only in specialized centers. In recent years, balloon pulmonary angioplasty (BPA) and drug therapy for CTEPH have also shown good results. This review discusses the complex pathogenesis of CTEPH and presents the standard of care, PEA, as well as a new device called BPA, which is showing remarkable progress in efficacy and safety. Additionally, several drugs are now demonstrating established evidence of efficacy in treating CTEPH.<\/jats:p>","DOI":"10.3390\/ijms24043979","type":"journal-article","created":{"date-parts":[[2023,2,16]],"date-time":"2023-02-16T02:52:56Z","timestamp":1676515976000},"page":"3979","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension"],"prefix":"10.3390","volume":"24","author":[{"given":"Naoyuki","family":"Otani","sequence":"first","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, Nikko 321-1298, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryo","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Dokkyo Medical University, Mibu 321-0293, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Tomoe","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, Nikko 321-1298, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8260-833X","authenticated-orcid":false,"given":"Shigeru","family":"Toyoda","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Dokkyo Medical University, Mibu 321-0293, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1940-2347","authenticated-orcid":false,"given":"Takanori","family":"Yasu","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, Nikko 321-1298, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takaaki","family":"Nakamoto","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, Nikko 321-1298, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,2,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"D34","DOI":"10.1016\/j.jacc.2013.10.029","article-title":"Updated clinical classification of pulmonary hypertension","volume":"62","author":"Simonneau","year":"2013","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1465","DOI":"10.1056\/NEJMra010902","article-title":"Chronic thromboembolic pulmonary hypertension","volume":"345","author":"Fedullo","year":"2001","journal-title":"N. Engl. J. Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2045894020977300","DOI":"10.1177\/2045894020977300","article-title":"Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Identification of the most accurate estimates from a systematic literature review","volume":"11","author":"Leber","year":"2021","journal-title":"Pulm. Circ."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2002828","DOI":"10.1183\/13993003.02828-2020","article-title":"ERS statement on chronic thromboembolic pulmonary hypertension","volume":"57","author":"Delcroix","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1007\/s00392-018-1215-5","article-title":"Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany","volume":"107","author":"Kramm","year":"2018","journal-title":"Clin. Res. Cardiol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1801787","DOI":"10.1183\/13993003.01787-2018","article-title":"Limitations of resting haemodynamics in chronic thromboembolic disease without pulmonary hypertension","volume":"53","author":"Swietlik","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"2257","DOI":"10.1056\/NEJMoa032274","article-title":"Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism","volume":"350","author":"Pengo","year":"2004","journal-title":"N. Engl. J. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"160112","DOI":"10.1183\/16000617.0112-2016","article-title":"The pathophysiology of chronic thromboembolic pulmonary hypertension","volume":"26","author":"Simonneau","year":"2017","journal-title":"Eur. Respir. Rev."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1275","DOI":"10.1183\/09031936.00169113","article-title":"Microvascular disease in chronic thromboembolic pulmonary hypertension: A role for pulmonary veins and systemic vasculature","volume":"44","author":"Ghigna","year":"2014","journal-title":"Eur. Respir. J."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"702","DOI":"10.1016\/j.jtcvs.2010.11.024","article-title":"Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: Results from an international prospective registry","volume":"141","author":"Mayer","year":"2011","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","article-title":"2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"43","author":"Humbert","year":"2022","journal-title":"Eur. Heart J."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"2493","DOI":"10.1093\/eurheartj\/ehp297","article-title":"Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT)","volume":"30","author":"Hoeper","year":"2009","journal-title":"Eur. Heart J."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1177\/1089253214536621","article-title":"Pulmonary endarterectomy: Part I. Pathophysiology, clinical manifestations, and diagnostic evaluation of chronic thromboembolic pulmonary hypertension","volume":"18","author":"Banks","year":"2014","journal-title":"Semin. Cardiothorac. Vasc. Anesth."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1973","DOI":"10.1161\/CIRCULATIONAHA.110.015008","article-title":"Chronic thromboembolic pulmonary hypertension (CTEPH): Results from an international prospective registry","volume":"124","author":"Delcroix","year":"2011","journal-title":"Circulation"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1183\/16000617.00001115","article-title":"Chronic thromboembolic pulmonary hypertension: A distinct disease entity","volume":"24","author":"Lang","year":"2015","journal-title":"Eur. Respir. Rev."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"10S","DOI":"10.1378\/chest.107.1_Supplement.10S","article-title":"Pathology of pulmonary thromboembolism","volume":"107","author":"Wagenvoort","year":"1995","journal-title":"Chest"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1016\/j.jacc.2008.12.038","article-title":"C-reactive protein: A new predictor of adverse outcome in pulmonary arterial hypertension","volume":"53","author":"Quarck","year":"2009","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1183\/09031936.00009914","article-title":"Contribution of inflammation and impaired angiogenesis to the pathobiology of chronic thromboembolic pulmonary hypertension","volume":"46","author":"Quarck","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"951","DOI":"10.1183\/09031936.00145013","article-title":"Comprehensive analysis of inflammatory markers in chronic thromboembolic pulmonary hypertension patients","volume":"44","author":"Zabini","year":"2014","journal-title":"Eur. Respir. J."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1378\/chest.126.1.135","article-title":"Cytokine response to pulmonary thromboendarterectomy","volume":"26","author":"Langer","year":"2004","journal-title":"Chest"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1161\/ATVBAHA.107.156000","article-title":"Role for staphylococci in misguided thrombus resolution of chronic thromboembolic pulmonary hypertension","volume":"28","author":"Bonderman","year":"2008","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1160\/TH04-10-0657","article-title":"Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension","volume":"93","author":"Bonderman","year":"2005","journal-title":"Thromb. Haemost."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1183\/09031936.00087608","article-title":"Risk factors for chronic thromboembolic pulmonary hypertension","volume":"33","author":"Bonderman","year":"2009","journal-title":"Eur. Respir. J."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1016\/j.cyto.2006.09.003","article-title":"Splenectomy changes the pattern of cytokine production in beta-thalassemic patients","volume":"35","author":"Gholamhoseinian","year":"2006","journal-title":"Cytokine"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1016\/j.thromres.2017.06.011","article-title":"Current understanding of the pathophysiology of chronic thromboembolic pulmonary hypertension","volume":"164","author":"Sharma","year":"2018","journal-title":"Thromb. Res."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1034\/j.1399-3003.2000.15b28.x","article-title":"Thrombotic risk factors in pulmonary hypertension","volume":"15","author":"Wolf","year":"2000","journal-title":"Eur. Respir. J."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1056\/NEJM200008173430702","article-title":"High plasma levels of factor VIII and the risk of recurrent venous thromboembolism","volume":"343","author":"Kyrle","year":"2000","journal-title":"N. Engl. J. Med."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1160\/TH03-02-0067","article-title":"High prevalence of elevated clotting factor VIII in chronic thromboembolic pulmonary hypertension","volume":"90","author":"Bonderman","year":"2003","journal-title":"Thromb. Haemost."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1183\/09031936.00055107","article-title":"Fibrinogen Aalpha Thr312Ala polymorphism is associated with chronic thromboembolic pulmonary hypertension","volume":"31","author":"Suntharalingam","year":"2008","journal-title":"Eur. Respir. J."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1270","DOI":"10.1164\/rccm.200506-916OC","article-title":"Fibrin derived from patients with chronic thromboembolic pulmonary hypertension is resistant to lysis","volume":"173","author":"Morris","year":"2006","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1161\/01.CIR.86.4.1241","article-title":"Endogenous fibrinolytic system in chronic large-vessel thromboembolic pulmonary hypertension","volume":"86","author":"Olman","year":"1992","journal-title":"Circulation"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1293","DOI":"10.1161\/ATVBAHA.115.306845","article-title":"Thrombin-activatable fibrinolysis inhibitor in chronic thromboembolic pulmonary hypertension","volume":"36","author":"Yaoita","year":"2016","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_33","first-page":"9700","article-title":"Platelet abnormalities in chronic thromboembolic pulmonary hypertension","volume":"8","year":"2015","journal-title":"Int. J. Clin. Exp. Med."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"2486","DOI":"10.1161\/ATVBAHA.114.304404","article-title":"Platelets are highly activated in patients of chronic thromboembolic pulmonary hypertension","volume":"34","author":"Yaoita","year":"2014","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"Zabini, D., Nagaraj, C., Stacher, E., Lang, I.M., Nierlich, P., Klepetko, W., Heinemann, A., Olschewski, H., B\u00e1lint, Z., and Olschewski, A. (2012). Angiostatic factors in the pulmonary endarterectomy material from chronic thromboembolic pulmonary hypertension patients cause endothelial dysfunction. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0043793"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1007\/s00441-013-1779-3","article-title":"PECAM-1: Regulator of endothelial junctional integrity","volume":"355","author":"Privratsky","year":"2014","journal-title":"Cell Tissue Res."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"3376","DOI":"10.1182\/blood-2013-04-499558","article-title":"Platelet endothelial cell adhesion molecule 1 deficiency misguides venous thrombus resolution","volume":"122","author":"Kellermair","year":"2013","journal-title":"Blood"},{"key":"ref_38","first-page":"78","article-title":"Cancer-associated thrombosis","volume":"4","author":"Karimi","year":"2010","journal-title":"Open Cardiovasc. Med. J."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1499","DOI":"10.1182\/blood-2017-03-743211","article-title":"Cancer-associated pathways and biomarkers of venous thrombosis","volume":"130","author":"Hisada","year":"2017","journal-title":"Blood"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1111\/j.1538-7836.2007.02818.x","article-title":"ABO(H) blood groups and vascular disease: A systematic review and meta-analysis","volume":"6","author":"Wu","year":"2008","journal-title":"J. Thromb. Haemost."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1016\/j.ijcard.2011.02.037","article-title":"Characterization of myofibroblasts in chronic thromboembolic pulmonary hypertension","volume":"159","author":"Maruoka","year":"2012","journal-title":"Int. J. Cardiol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1016\/j.ajpath.2011.06.001","article-title":"Role of endothelial-mesenchymal transition (EndoMT) in the pathogenesis of fibrotic disorders","volume":"179","author":"Li","year":"2011","journal-title":"Am. J. Pathol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"286S","DOI":"10.1378\/chest.122.6_suppl.286S","article-title":"The myofibroblast in pulmonary fibrosis","volume":"122","author":"Phan","year":"2002","journal-title":"Chest"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1161\/ATVBAHA.113.302991","article-title":"Defective angiogenesis delays thrombus resolution: A potential pathogenetic mechanism underlying chronic thromboembolic pulmonary hypertension","volume":"34","author":"Alias","year":"2014","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1111\/j.1538-7836.2011.04225.x","article-title":"Autopsy-proven untreated previous pulmonary thromboembolism: Frequency and distribution in the pulmonary artery and correlation with patients\u2019 clinical characteristics","volume":"9","author":"Ro","year":"2011","journal-title":"J. Thromb. Haemost."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1457","DOI":"10.1016\/S0003-4975(03)00828-2","article-title":"Pulmonary endarterectomy: Experience and lessons learned in 1500 cases","volume":"76","author":"Jamieson","year":"2003","journal-title":"Ann. Thorac. Surg."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"25S","DOI":"10.1016\/j.jacc.2004.02.033","article-title":"Pathologic assessment of vasculopathies in pulmonary hypertension","volume":"43","author":"Pietra","year":"2004","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1160\/TH13-07-0538","article-title":"Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Prevalence of CTEPH after pulmonary embolism","volume":"112","author":"Couturaud","year":"2014","journal-title":"Thromb. Haemost."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1801904","DOI":"10.1183\/13993003.01904-2018","article-title":"Diagnosis of pulmonary hypertension","volume":"53","author":"Frost","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1183\/13993003.01032-2015","article-title":"2015 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT)","volume":"46","author":"Humbert","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/j.echo.2017.09.009","article-title":"Echocardiographic estimation of mean pulmonary artery pressure: A comparison of different approaches to assign the likelihood of pulmonary hypertension","volume":"31","author":"Hellenkamp","year":"2018","journal-title":"J. Am. Soc. Echocardiogr."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"160108","DOI":"10.1183\/16000617.0108-2016","article-title":"Diagnosis of chronic thromboembolic pulmonary hypertension","volume":"26","author":"Gopalan","year":"2017","journal-title":"Eur. Respir. Rev."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1097\/MNM.0b013e32835085d9","article-title":"Diagnosis of chronic thromboembolic pulmonary hypertension: Comparison of ventilation\/perfusion scanning and multidetector computed tomography pulmonary angiography with pulmonary angiography","volume":"33","author":"He","year":"2012","journal-title":"Nucl. Med. Commun."},{"key":"ref_54","doi-asserted-by":"crossref","unstructured":"Dong, C., Zhou, M., Liu, D., Long, X., Guo, T., and Kong, X. (2015). Diagnostic accuracy of computed tomography for chronic thromboembolic pulmonary hypertension: A systematic review and meta-analysis. PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0126985"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.1378\/chest.12-0407","article-title":"Role of 320-slice CT imaging in the diagnostic workup of patients with chronic thromboembolic pulmonary hypertension","volume":"143","author":"Sugiura","year":"2013","journal-title":"Chest"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1016\/j.ejrad.2016.12.013","article-title":"Efficacy and safety of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension guided by cone-beam computed tomography and electrocardiogram-gated area detector computed tomography","volume":"89","author":"Ogo","year":"2017","journal-title":"Eur. J. Radiol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT)","volume":"37","author":"Humbert","year":"2016","journal-title":"Eur. Heart J."},{"key":"ref_58","first-page":"274","article-title":"Techniques and outcomes of pulmonary endarterectomy","volume":"14","author":"Thistlethwaite","year":"2008","journal-title":"Ann. Thorac. Cardiovasc. Surg."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1203","DOI":"10.1067\/mtc.2002.127313","article-title":"The operative classification of thromboembolic disease determines the outcome of pulmonary endarterectomy","volume":"124","author":"Thistlethwaite","year":"2002","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/j.athoracsur.2012.04.004","article-title":"Pulmonary endarterectomy: Recent changes in a single institution\u2019s experience with more than 2,700 patients","volume":"94","author":"Madani","year":"2012","journal-title":"Ann. Thorac. Surg."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1801915","DOI":"10.1183\/13993003.01915-2018","article-title":"Chronic thromboembolic pulmonary hypertension","volume":"53","author":"Kim","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"31","DOI":"10.12968\/hmed.2013.74.1.31","article-title":"Surgical management of chronic thromboembolic pulmonary hypertension","volume":"74","author":"Ng","year":"2013","journal-title":"Br. J. Hosp. Med."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"2139","DOI":"10.1016\/j.athoracsur.2006.07.020","article-title":"Venovenous extracorporeal life support after pulmonary endarterectomy: Indications, techniques, and outcomes","volume":"82","author":"Thistlethwaite","year":"2006","journal-title":"Ann. Thorac. Surg."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1183\/16000617.00000815","article-title":"Pulmonary endarterectomy: The potentially curative treatment for patients with chronic thromboembolic pulmonary hypertension","volume":"24","author":"Jenkins","year":"2015","journal-title":"Eur. Respir. Rev."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1016\/j.athoracsur.2008.06.037","article-title":"Successful extracorporeal membrane oxygenation support after pulmonary thromboendarterectomy","volume":"86","author":"Berman","year":"2008","journal-title":"Ann. Thorac. Surg."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1016\/j.healun.2017.06.011","article-title":"Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension","volume":"37","author":"Lankeit","year":"2017","journal-title":"J. Heart Lung Transplant."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1652","DOI":"10.1378\/chest.119.6.1652","article-title":"Extensive sampling improves preoperative bronchoscopic assessment of airway invasion by supracarinal esophageal cancer: A prospective study in 166 patients","volume":"119","author":"Riedel","year":"2001","journal-title":"Chest"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1161\/CIRCULATIONAHA.115.016522","article-title":"Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: Results from an international prospective registry","volume":"133","author":"Delcroix","year":"2016","journal-title":"Circulation"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1428","DOI":"10.1253\/circj.CJ-17-1242","article-title":"Long-term outcome of chronic thromboembolic pulmonary hypertension at a single Japanese pulmonary endarterectomy center","volume":"82","author":"Miwa","year":"2018","journal-title":"Circ. J."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1016\/j.jtcvs.2009.12.056","article-title":"Survival after pulmonary thromboendarterectomy: Effect of residual pulmonary hypertension","volume":"141","author":"Freed","year":"2011","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"D92","DOI":"10.1016\/j.jacc.2013.10.024","article-title":"Chronic thromboembolic pulmonary hypertension","volume":"62","author":"Kim","year":"2013","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1111\/aor.13628","article-title":"Extracorporeal life support during and after bilateral sequential lung transplantation in patients with pulmonary artery hypertension","volume":"44","author":"Campisi","year":"2020","journal-title":"Artif. Organs"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"161","DOI":"10.21037\/acs-2021-pte-139","article-title":"End-stage chronic thromboembolic pulmonary hypertension: Is there still room for lung transplantation?-the past, the present and the future","volume":"11","author":"Faccioli","year":"2022","journal-title":"Ann. Cardiothorac. Surg."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1016\/j.healun.2015.10.030","article-title":"Combined pulmonary endarterectomy and balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension","volume":"35","author":"Wiedenroth","year":"2016","journal-title":"J. Heart Lung Transplant."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"1635","DOI":"10.1183\/09031936.00050114","article-title":"Outcomes of pulmonary endarterectomy for symptomatic chronic thromboembolic disease","volume":"44","author":"Taboada","year":"2014","journal-title":"Eur. Respir. J."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"1800458","DOI":"10.1183\/13993003.00458-2018","article-title":"Exercise right heart catheterisation before and after pulmonary endarterectomy in patients with chronic thromboembolic disease","volume":"52","author":"Guth","year":"2018","journal-title":"Eur. Respir. J."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1161\/01.CIR.103.1.10","article-title":"Balloon pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension","volume":"103","author":"Feinstein","year":"2001","journal-title":"Circulation"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"748","DOI":"10.1161\/CIRCINTERVENTIONS.112.971077","article-title":"Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension","volume":"5","author":"Mizoguchi","year":"2012","journal-title":"Circ. Cardiovasc. Interv."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1161\/CIRCINTERVENTIONS.112.971390","article-title":"Percutaneous transluminal pulmonary angioplasty for treatment of chronic thromboembolic pulmonary hypertension","volume":"5","author":"Kataoka","year":"2012","journal-title":"Circ. Cardiovasc. Interv."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"e004029","DOI":"10.1161\/CIRCOUTCOMES.117.004029","article-title":"Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: Results of a multicenter registry","volume":"10","author":"Ogawa","year":"2017","journal-title":"Circ. Cardiovasc. Qual. Outcomes"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1016\/j.healun.2021.06.011","article-title":"Serial right heart catheter assessment between balloon pulmonary angioplasty sessions identifies procedural factors that influence the response to treatment","volume":"40","author":"Hug","year":"2021","journal-title":"J. Heart Lung Transplant."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"e010243","DOI":"10.1161\/CIRCINTERVENTIONS.120.010243","article-title":"Efficacy and safety of percutaneous pulmonary artery subtotal occlusion and chronic total occlusion interventions in chronic thromboembolic pulmonary hypertension","volume":"14","author":"Gerges","year":"2021","journal-title":"Circ. Cardiovasc. Interv."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1016\/S2213-2600(22)00171-0","article-title":"Balloon pulmonary angioplasty versus riociguat in inoperable chronic thromboembolic pulmonary hypertension (MR BPA): An open-label, randomised controlled trial","volume":"10","author":"Kawakami","year":"2022","journal-title":"Lancet Respir. Med."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"8870","DOI":"10.1038\/s41598-020-65697-4","article-title":"Comparison of balloon pulmonary angioplasty and pulmonary vasodilator for inoperable chronic thromboembolic pulmonary hypertension: A systematic review and meta-analysis","volume":"10","author":"Kalra","year":"2020","journal-title":"Sci. Rep."},{"key":"ref_85","first-page":"1","article-title":"Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension","volume":"11","author":"Kurzyna","year":"2015","journal-title":"Adv. Interv. Cardiol. \/Post\u0119py W Kardiol. Interwencyjnej"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1016\/S2213-2600(22)00214-4","article-title":"Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): A multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study","volume":"10","author":"Brenot","year":"2022","journal-title":"Lancet Respir. Med."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1122","DOI":"10.1164\/rccm.200712-1841OC","article-title":"Improved outcomes in medically and surgically treated patients with chronic thromboembolic pulmonary hypertension","volume":"177","author":"Condliffe","year":"2008","journal-title":"Am. J. Respir. Crit Care Med."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1111\/jth.14649","article-title":"A multicenter study of anticoagulation in operable chronic thromboembolic pulmonary hypertension","volume":"18","author":"Bunclark","year":"2020","journal-title":"J. Thromb. Haemost."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1177\/1356336X221104909","article-title":"The health and educational impact of removing financial constraints for school sport","volume":"29","author":"Sulz","year":"2023","journal-title":"Eur. Phy. Educ. Rev."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"985","DOI":"10.1183\/09031936.00201612","article-title":"Chronic thromboembolic pulmonary hypertension: Role of medical therapy","volume":"41","author":"Jansa","year":"2013","journal-title":"Eur. Respir. J."},{"key":"ref_91","doi-asserted-by":"crossref","unstructured":"Otani, N., Tomoe, T., Kawabe, A., Sugiyama, T., Horie, Y., Sugimura, H., Yasu, T., and Nakamoto, T. (2022). Recent advances in the treatment of pulmonary arterial hypertension. Pharmaceuticals, 15.","DOI":"10.3390\/ph15101277"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1016\/j.healun.2006.12.014","article-title":"Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension","volume":"26","author":"Cabrol","year":"2007","journal-title":"J. Heart Lung Transplant."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1007\/s11748-016-0724-2","article-title":"Prospective, randomized study of inhaled prostacyclin versus nitric oxide in patients with residual pulmonary hypertension after pulmonary endarterectomy","volume":"65","author":"Abe","year":"2017","journal-title":"Gen. Thorac. Cardiovasc. Surg."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1378\/chest.123.2.338","article-title":"Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension","volume":"123","author":"Nagaya","year":"2003","journal-title":"Chest"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1159\/000107977","article-title":"Acutely improved hemodynamics following inhaled iloprost in patients with chronic thromboembolic pulmonary hypertension","volume":"76","author":"Krug","year":"2008","journal-title":"Respiration"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1016\/S0003-4975(03)00728-8","article-title":"Inhaled iloprost in patients with chronic thromboembolic pulmonary hypertension: Effects before and after pulmonary thromboendarterectomy","volume":"76","author":"Kramm","year":"2003","journal-title":"Ann. Thorac. Surg."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"1583","DOI":"10.1378\/chest.123.5.1583","article-title":"Effect of orally active prostacyclin analogue on survival in patients with chronic thromboembolic pulmonary hypertension without major vessel obstruction","volume":"123","author":"Ono","year":"2003","journal-title":"Chest"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1016\/S2213-2600(18)30367-9","article-title":"Subcutaneous treprostinil for the treatment of severe non-operable chronic thromboembolic pulmonary hypertension (CTREPH): A double-blind, phase 3, randomized controlled trial","volume":"7","author":"Jansa","year":"2019","journal-title":"Lancet Respir. Med."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1111\/j.1538-7836.2007.02394.x","article-title":"Treprostinil for severe inoperable chronic thromboembolic pulmonary hypertension","volume":"5","author":"Bonderman","year":"2007","journal-title":"J. Thromb. Haemost."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"2065","DOI":"10.1111\/crj.12774","article-title":"Bosentan therapy for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: A systemic review and meta-analysis","volume":"12","author":"Chen","year":"2018","journal-title":"Clin. Respir. J."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"2127","DOI":"10.1016\/j.jacc.2008.08.059","article-title":"Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable forms of chronic thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial","volume":"52","author":"Jais","year":"2008","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1016\/S2213-2600(17)30305-3","article-title":"Macitentan for the treatment of inoperable CTEPH (MERIT-1): Results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study","volume":"5","author":"Ghofrani","year":"2017","journal-title":"Lancet Respir. Med."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1183\/09031936.00039007","article-title":"Long-term treatment with sildenafil for chronic thromboembolic pulmonary hypertension","volume":"30","author":"Reichenberger","year":"2007","journal-title":"Eur. Respir. J."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1378\/chest.07-2681","article-title":"Long-term use of sildenafil for inoperable chronic thromboembolic pulmonary hypertension","volume":"134","author":"Suntharalingam","year":"2008","journal-title":"Chest"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"00850","DOI":"10.1183\/23120541.00850-2020","article-title":"Current strategies for managing chronic thromboembolic pulmonary hypertension: Results of the worldwide prospective CTEPH Registry","volume":"7","author":"Guth","year":"2021","journal-title":"ERJ Open Res."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1016\/j.chest.2016.05.024","article-title":"Riociguat: Mode of action and clinical development in pulmonary hypertension","volume":"151","author":"Ghofrani","year":"2017","journal-title":"Chest"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"792","DOI":"10.1183\/09031936.00182909","article-title":"Riociguat for chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension: A phase II study","volume":"36","author":"Ghofrani","year":"2010","journal-title":"Eur. Respir. J."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1056\/NEJMoa1209657","article-title":"Riociguat for treatment of chronic thromboembolic pulmonary hypertension","volume":"369","author":"Ghofrani","year":"2013","journal-title":"N. Engl. J. Med."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"1293","DOI":"10.1183\/09031936.00087114","article-title":"Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: A long-term extension study (CHEST-2)","volume":"45","author":"Simonneau","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1253\/circj.CJ-66-0158","article-title":"Guidelines for the treatment of pulmonary hypertension (JCS 2017\/JPCPHS 2017)","volume":"83","author":"Fukuda","year":"2019","journal-title":"Circ. J."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"2250","DOI":"10.1161\/CIRCULATIONAHA.109.192230","article-title":"ACCF\/AHA 2009 Expert consensus document on pulmonary hypertension: A report of the American college of cardiology foundation task force on expert consensus documents and the American heart association: Developed in collaboration with the American college of chest physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association","volume":"119","author":"McLaughlin","year":"2009","journal-title":"Circulation"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1183\/09031936.04.00020004","article-title":"Continuous intravenous epoprostenol for chronic thromboembolic pulmonary hypertension","volume":"23","author":"Bresser","year":"2004","journal-title":"Eur. Respir. J."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1016\/j.jtcvs.2009.03.053","article-title":"Bosentan as a bridge to pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension","volume":"139","author":"Reesink","year":"2010","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"1248","DOI":"10.1161\/CIRCULATIONAHA.109.865881","article-title":"Pulmonary hypertension medical therapy for chronic thromboembolic pulmonary hypertension before pulmonary thromboendarterectomy","volume":"120","author":"Jensen","year":"2009","journal-title":"Circulation"},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"2045894019898377","DOI":"10.1177\/2045894019898377","article-title":"World experience with riociguat as potential bridging therapy in patients with chronic thromboembolic pulmonary hypertension: A case series","volume":"10","author":"Balasubramanian","year":"2020","journal-title":"Pulm. Circ."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/24\/4\/3979\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T18:37:33Z","timestamp":1760121453000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/24\/4\/3979"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,16]]},"references-count":115,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2023,2]]}},"alternative-id":["ijms24043979"],"URL":"https:\/\/doi.org\/10.3390\/ijms24043979","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,2,16]]}}}