Chronic thromboembolic pulmonary hypertension: a review of risk factors, management and current challenges

References

• Simonneau G, Montani D, and Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913.
   PubMed Web of Science ®Google Scholar
• Galiè N, Kim NHS. Pulmonary microvascular disease in chronic thromboembolic pulmonary hypertension. Proc Am Thorac Soc. 2006;3(7):571–576.
   PubMedGoogle Scholar
• Moser KM, Bloor CM. Pulmonary vascular lesions occurring in patients with chronic major vessel thromboembolic pulmonary hypertension. Chest. 1993;103(3):685–692.
   PubMed Web of Science ®Google Scholar
• Kim NH, Delcroix M, Jais X, et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(1):1801915.
   PubMed Web of Science ®Google Scholar
• Riedel M, Stanek V, Widimsky J, et al. Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. Chest. 1982;81(2):151–158.
   PubMed Web of Science ®Google Scholar
• Becattini C, Agnelli G, Pesavento R, et al. Incidence of chronic thromboembolic pulmonary hypertension after a first episode of pulmonary embolism. Chest. 2006;130(1):172–175.
   PubMed Web of Science ®Google Scholar
• Klok FA, van Kralingen KW, Apj VD, et al. Prospective cardiopulmonary screening program to detect chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism. Haematologica. 2010;95:970–975.
   PubMed Web of Science ®Google Scholar
• Martí D, Gómez V, Escobar C, et al. Incidence of symptomatic and asymptomatic chronic thromboembolic pulmonary hypertension. Arch Bronconeumol. 2010;46(12):628–633.
   PubMed Web of Science ®Google Scholar
• Miniati M, Monti S, Bottai M, et al. Survival and restoration of pulmonary perfusion in a long-term follow-up of patients after acute pulmonary embolism. Medicine (Baltimore). 2006;85(5):253–262.
   PubMed Web of Science ®Google Scholar
• Pengo V, Lensing AWA, Prins MH, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350(22):2257–2264.
   PubMed Web of Science ®Google Scholar
• Ribeiro A, Lindmarker P, Johnsson H, et al. Pulmonary embolism: one-year follow-up with echocardiography Doppler and five-year survival analysis. Circulation. 1999;99(10):1325–1330.
   PubMed Web of Science ®Google Scholar
• Hoeper MM, Humbert M, Souza R, et al. A global view of pulmonary hypertension. Lancet Respir Med. 2016;4:306–322.
   PubMed Web of Science ®Google Scholar
• Guerin L, Couturaud F, Parent F, et al. Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Thromb Haemost. 2014;112(9):598–605.
   PubMed Web of Science ®Google Scholar
• Simonneau G, Torbicki A, Dorfmüller P, et al. The pathophysiology of chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2017;26(143):160112.
   PubMed Web of Science ®Google Scholar
• Pepke-Zaba J, Delcroix M, Lang I, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011;124(18):1973–1981.
   PubMed Web of Science ®Google Scholar
• Lang IM, Simonneau G, Pepke-Zaba, et al. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension: a case–control study. Thromb Haemost. 2013;110(7):83–91.
   PubMed Web of Science ®Google Scholar
• Lang IM, Dorfmüller P, Vonk Noordegraaf A. The pathobiology of chronic thromboembolic pulmonary hypertension. Ann Am Thorac Soc. 2016;13(Suppl. 3):S215–S221.
   PubMed Web of Science ®Google Scholar
• Wolf M, Boyer-Neumann C, Parent F, et al. Thrombotic risk factors in pulmonary hypertension. Eur Respir J. 2000;15(2):395–399.
   PubMed Web of Science ®Google Scholar
• Bonderman D, Turecek PL, Jakowitsch J, et al. High prevalence of elevated clotting factor VIII in chronic thromboembolic pulmonary hypertension. Thromb Haemost. 2003;90:372–376.
   PubMed Web of Science ®Google Scholar
• Bonderman D, Wilkens H, Wakounig S, et al. Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J. 2009;33(2):325–331.
   PubMed Web of Science ®Google Scholar
• Newnham M, South K, Bleda M, et al. The ADAMTS13–VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(3):1801805.
   PubMed Web of Science ®Google Scholar
• Condliffe R, Kiely DG, Gibbs JSR, et al. Prognostic and aetiological factors in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2009;33(2):332–338.
   PubMed Web of Science ®Google Scholar
• Suntharalingam J, Machado RD, Sharples LD, et al. Demographic features, BMPR2 status and outcomes in distal chronic thromboembolic pulmonary hypertension. Thorax. 2007;62(7):617–622.
   PubMed Web of Science ®Google Scholar
• Ulrich S, Szamalek-Hoegel J, Hersberger M, et al. Sequence variants in BMPR2 and genes involved in the serotonin and nitric oxide pathways in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: relation to clinical parameters and comparison with left heart disease. Respiration. 2010;79(4):279–287.
   PubMed Web of Science ®Google Scholar
• Galie N, Humbert M, Vachiery JL, et al. 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). Eur Respir J. 2015;46:903–975.
   PubMed Web of Science ®Google Scholar
• Tunariu N, Gibbs SJ, Win Z, et al. Ventilation–perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med. 2007;48(5):680–684.
   PubMed Web of Science ®Google Scholar
• He J, Fang W, Lv B, et al. Diagnosis of chronic thromboembolic pulmonary hypertension: comparison of ventilation/perfusion scanning and multidetector computed tomography pulmonary angiography with pulmonary angiography. Nucl Med Commun. 2012;33(5):459–463.
   PubMed Web of Science ®Google Scholar
• Held M, Kolb P, Grun M, et al. Functional characterization of patients with chronic thromboembolic disease. Respiration. 2016;91(6):503–509.
   PubMed Web of Science ®Google Scholar
• Jamieson SW, Kapelanski DP, Sakakibara N, et al. Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. Ann Thorac Surg. 2003;76(5):1457–1462.
   PubMed Web of Science ®Google Scholar
• Madani M, Jamieson SW. Pulmonary endarterectomy for chronic thromboembolic disease.Oper Tech Thorac Cardiovas Surg. 2006;114:264–274.
   Google Scholar
• Vuylsteke A, Sharples L, Charman G, et al. Circulatory arrest versus cerebral perfusion during pulmonary endarterectomy surgery (PEACOG): a randomised controlled trial. Lancet. 2011;378(9800):1379–1387.
   PubMed Web of Science ®Google Scholar
• Madani M, Auger W, Pretorius V, et al. Pulmonary endarterectomy: recent changes in a single institution’s experience of more than 2700 patients. Ann Thorac Surg. 2012;94(1):97–103.
   PubMed Web of Science ®Google Scholar
• Mayer E, Jenkins D, Lindner J, et al. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg. 2011 Mar;141(3):702–710.
   PubMed Web of Science ®Google Scholar
• Cannon JE, Su L, Kiely D, et al. Dynamic risk stratification of patient long-term outcome after pulmonary endarterectomy: results from the United Kingdom cohort. Circulation. 2016;133(18): 1761–1771.
   PubMed Web of Science ®Google Scholar
• Delcroix M, Lang I, Pepke-Zaba J, et al. Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Circulation. 2016;133(9):859–871.
   PubMed Web of Science ®Google Scholar
• Quadery SR, Swift AJ, Billings CG, et al. The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2018;52(3):1800589.
   PubMed Web of Science ®Google Scholar
• D’Armini AM, Morsolini M, Mattiucci G, et al. Pulmonary endarterectomy for distal chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg. 2014;148(3):1005–1011. 12 e1-2; discussion 11-2.
   PubMed Web of Science ®Google Scholar
• Newnham M, Bunclark K, Abraham N, et al. CAMPHOR score: patient-reported outcomes are improved by pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension (CTEPH). Eur Respir J. 2020;56(4):1902096.
   PubMed Web of Science ®Google Scholar
• Feinstein JA, Goldhaber SZ, Lock JE, et al. Balloon pulmonary angioplasty for treatment of chronic thromboembolic pulmonary hypertension. Circulation. 2001;103(1):10–30.
   PubMed Web of Science ®Google Scholar
• Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D92–9.
   PubMed Web of Science ®Google Scholar
• Lang I, Meyer BC, Ogo T, et al. Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2017;26(143):160119.
   PubMed Web of Science ®Google Scholar
• Hug KP, Coghlan JG, Cannon J, et al. Serial right heart catheter assessment between balloon pulmonary angioplasty sessions identify procedural factors that influence response to treatment. Jhlt. 2021;40:1223–1234.
   Google Scholar
• Gerges C, Friewald R, Gerges M, et al. Efficacy and safety of percutaneous pulmonary artery subtotal occlusion and chronic total occlusion intervention in chronic thromboembolic pulmonary hypertension. Circ Cardiovas Interv. 2021;14:e010243.
   PubMed Web of Science ®Google Scholar
• Inami T, Kataoka M, Shimura N, et al. Pressure-wire-guided percutaneous transluminal pulmonary angioplasty: a breakthrough in catheter-interventional therapy for chronic thromboembolic pulmonary hypertension. JACC Cardiovasc Interv. 2014;7(11): 1297–1306.
   PubMed Web of Science ®Google Scholar
• Khan MS, Amin E, Memon MM, et al. Meta-analysis of use of balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension. Int J Cardiol. 2019;291:134–139. doi:S0167-5273(18)36648-8 [pii] https://doi.org/10.1016/j.ijcard.2019.02.051
   PubMed Web of Science ®Google Scholar
• Broch K, Murbraech K, Ragnarsson A, et al. Echocardiographic evidence of right ventricular functional improvement after balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. 2016;35(1):80–86. published Online First: 2015/10/20.
   PubMed Web of Science ®Google Scholar
• Hoole SP, Coghlan JG, Cannon JE, et al. Balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension: the UK experience. Open Heart. 2020;7(1):e001144.
   PubMed Web of Science ®Google Scholar
• Mizoguchi H, Ogawa A, Munemasa M, et al. Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension. Circulation: Cardiovasc Interventions. 2012;5(6):748–755.
   PubMed Web of Science ®Google Scholar
• Inami T, Kataoka M, Yanagisawa R, et al. Long-term outcomes after percutaneous transluminal pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. Circulation. 2016;134(24): 2030–2032.
   PubMed Web of Science ®Google Scholar
• Tanabe N, Kawakami T, Satoh T, et al. Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: a systematic review. Respir Investig. 2018;56(4):332–341.
   PubMed Web of Science ®Google Scholar
• Inami T, Kataoka M, Shimura N, et al. Pulmonary edema predictive scoring index (PEPSI), a new index to predict risk of reperfusion pulmonary edema and improvement of hemodynamics in percutaneous transluminal pulmonary angioplasty. JACC Cardiovasc Interv. 2013;6(7): 725–736.
   PubMed Web of Science ®Google Scholar
• Phan K, Jo HE, Xu J, et al. Medical Therapy versus balloon angioplasty for CTEPH: a systematic review and meta-analysis. Heart Lung Circ. 2018;27(1):89–98.
   PubMed Web of Science ®Google Scholar
• Jais X, Brenot P, Bouvaist H, et al. Late breaking abstract - balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension: results from the randomised controlled RACE study. Eur Respir J. 2019;54(suppl63):RCT1885.
   Google Scholar
• Wiedenroth CB, Liebetrau C, Breithecker A, et al. Combined pulmonary endarterectomy and balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. 2016;35(5):591–596.
   PubMed Web of Science ®Google Scholar
• Bunclark K, Newnham M, Chiu Y‐D, et al. A multicenter study of anticoagulation in operable chronic thromboembolic pulmonary hypertension. J Thromb Haemost. 2020;18(1):114–122.
   PubMed Web of Science ®Google Scholar
• Cannon J, Pepke Zaba J. Is distal chronic thromboembolic pulmonary hypertension treatable with PAH targeted drugs? Seminars in Respiratory and Critical Care Medicine. 2013;34(5):620–626.
   PubMed Web of Science ®Google Scholar
• Ghofrani HA, D’Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369(4):319–329.
   PubMed Web of Science ®Google Scholar
• Jais X, D’Armini AM, Jansa P, et al. 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. J Am Coll Cardiol. 2008;52(25):2127–2134.
   PubMed Web of Science ®Google Scholar
• Ghofrani HA, Simonneau G, D’Armini AM, et al. Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study. Lancet Respir Med. 2017;5(10):785–794.
   PubMed Web of Science ®Google Scholar
• Soduchi-Kolici R, Jansa P, Kopec G, et al. Subcutaneous treprostinil for the treatment of severe non-operable chornic thromboembolic pulmonary hypertension (CTEPH): a double blind, phase 3, randomised controlled trial. Lancet Respir Med. 2019;7(3):239–248.
   PubMed Web of Science ®Google Scholar
• Jensen KW, Kerr KM, Fedullo PF, et al. Pulmonary hypertensive medical therapy in chronic thromboembolic pulmonary hypertension before pulmonary thromboendarterectomy. Circulation. 2009;120(13):1248–1254.
   PubMed Web of Science ®Google Scholar
• Jais X, Brenot P, Bouvaist H, et al. BPA and riociguat for the management of inoperable CTEPH: results of the extension study following the RACE randomized controlled. Trial (RCT) Am J Resp Crit Care Med. 2021;203. A1182.
   Web of Science ®Google Scholar
• Sugimura K, Fukumoto Y, Satoh K, et al. Percutaneous transluminal pulmonary angioplasty markedly improves pulmonary hemodynamics and long-term prognosis in patients with chronic thromboembolic pulmonary hypertension. Circ J. 2012;76(2):485–488.
   PubMed Web of Science ®Google Scholar
• Kataoka M, Inami T, Kawakami T, et al. Balloon pulmonary angioplasty (percutaneous transluminal pulmonary angioplasty) for chronic thromboembolic pulmonary hypertension: a Japanese perspective. JACC Cardiovasc Interv. 2019;12(14):1382–1388.
   PubMed Web of Science ®Google Scholar