Abstract
Chronic thromboembolic pulmonary hypertension is an underdiagnosed condition. Patients typically present with the symptoms of right heart failure. Diagnosis is usually done by radionuclide ventilation/perfusion (VQ) scan, high-quality multidetector computed tomography (CT) or pulmonary angiography at expert centers. Pulmonary endarterectomy remains the corner stone in management of chronic thromboembolic pulmonary hypertension. Deep hypothermic circulatory arrest is commonly used for the operation at most centers. In-hospital mortality ranges from 1.7 to 14.2%. Pulmonary hemorrhage, reperfusion lung injury, and right ventricular failure remain major early post-operative concerns. Five-year survival is reported to be 76 to 89%. Long-term outcome depends on residual pulmonary hypertension. Balloon pulmonary angioplasty and medical management play an adjunctive role. Here, we provide a comprehensive review on surgical management of chronic thromboembolic pulmonary hypertension.
Similar content being viewed by others
References
Lang IM, Pesavento R, Bonderman D, Yuan JX. Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2013;41:462–8.
Bonderman D, Lang IM. Risk factors for chronic thromboembolic pulmonary hypertension. Textbook of Pulmonary Vascular Disease. Springer; 2011. p. 1253–9.
Madani MM. Surgical treatment of chronic thromboembolic pulmonary hypertension: pulmonary thromboendarterectomy. Methodist Debakey Cardiovasc J. 2016;12:213–8. https://doi.org/10.14797/mdcj-12-4-213.
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;133:859–71.
Chambers DC, Cherikh WS, Harhay MO, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-sixth adult lung and heart-lung transplantation Report-2019; Focus theme: Donor and recipient size match. J Heart Lung Transplant. 2019;38:1042–55. https://doi.org/10.1016/j.healun.2019.08.001.
Freed DH, Thomson BM, Berman M, et al. Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension. J Thorac Cardiovasc Surg. 2011;141:383–7. https://doi.org/10.1016/j.jtcvs.2009.12.056.
Lopez Gude MJ, Perez de la Sota E, Forteza Gil A, et al. Pulmonary thromboendarterectomy in 106 patients with chronic thromboembolic pulmonary hypertension. Arch Bronconeumol. 2015;51:502–8. https://doi.org/10.1016/j.arbres.2014.11.012.
Madani MM, Auger WR, Pretorius V, et al. Pulmonary endarterectomy: recent changes in a single institution's experience of more than 2,700 patients. Ann Thorac Surg. 2012;94:97–103.
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;141:702–10. https://doi.org/10.1016/j.jtcvs.2010.11.024.
Miwa H, Tanabe N, Jujo T, et al. Long-term outcome of chronic thromboembolic pulmonary hypertension at a single Japanese pulmonary endarterectomy Center. Circ J. 2018;82:1428–36. https://doi.org/10.1253/circj.CJ-17-1242.
Nierlich P, Hold A, Ristl R. Outcome after surgical treatment of chronic thromboembolic pulmonary hypertension: dealing with different patient subsets. A single-centre experience. Eur J Cardiothorac Surg. 2016;50:898–906. https://doi.org/10.1093/ejcts/ezw099.
Sakurai Y, Takami Y, Amano K, et al. Predictors of outcomes after surgery for chronic thromboembolic pulmonary hypertension. Ann Thorac Surg. 2019;108:1154–61. https://doi.org/10.1016/j.athoracsur.2019.03.100.
Scholzel B, Snijder R, Morshuis W, Saouti N, Plokker T, Post M. Clinical worsening after pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. Neth Heart J. 2011;19:498–503. https://doi.org/10.1007/s12471-011-0203-4.
Vistarini N, Morsolini M, Klersy C, et al. Pulmonary endarterectomy in the elderly: safety, efficacy and risk factors. J Cardiovasc Med (Hagerstown). 2016;17:144–51. https://doi.org/10.2459/JCM.0000000000000327.
Raza F, Vaidya A, Lacharite-Roberge A-S, et al. Initial clinical and hemodynamic results of a regional pulmonary thromboendarterectomy program. J Cardiovasc Surg (Torino). 2018;59:428–37. https://doi.org/10.23736/S0021-9509.17.10188-6.
Yıldızeli B, Taş S, Yanartaş M, et al. Pulmonary endarterectomy for chronic thrombo-embolic pulmonary hypertension: an institutional experience. Eur J Cardiothorac Surg. 2013;44:e219–e27.
van der Plas MN, Surie S, Reesink HJ, van Steenwijk RP, Kloek JJ, Bresser P. Longitudinal follow-up of six-minute walk distance after pulmonary endarterectomy. Ann Thorac Surg. 2011;91:1094–9.
Schölzel B, Snijder R, Morshuis W, Saouti N, Plokker T, Post M. Clinical worsening after pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. Neth Heart J. 2011;19:498–503.
Sihag S, Le B, Witkin AS, et al. Quantifying the learning curve for pulmonary thromboendarterectomy. J Cardiothorac Surg. 2017;12:121. https://doi.org/10.1186/s13019-017-0686-1.
Yan S, Lou S, Zhu J, et al. Perfusion strategy and mid-term results of 58 consecutive pulmonary endarterectomy. Perfusion. 2019;34:475–81. https://doi.org/10.1177/0267659119831518.
Gu S, Liu Y, Su P-x, Zhai Z-g, Yang Y-h, Wang C. Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: preliminary exploration in China. Chin Med J (Engl). 2010;123:979–83.
Luo W-C, Huang S-C, Lin Y-H, et al. Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension--A single-center experience in Taiwan. J Formos Med Assoc. 2015;114:1197–203. https://doi.org/10.1016/j.jfma.2014.08.009.
Oh SJ, Bok JS, Hwang HY, Kim K-H, Kim KB, Ahn H. Clinical outcomes of thromboendarterectomy for chronic thromboembolic pulmonary hypertension: 12-year experience. Korean J Thorac Cardiovasc Surg. 2013;46:41–8. https://doi.org/10.5090/kjtcs.2013.46.1.41.
Galiè N, Humbert M, Vachiery J-L, et al. 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). Eur Heart J. 2016;37:67–119.
Fares WH, Heresi GA. Chronic thromboembolic pulmonary hypertension: a worldwide view of how far we have come. Lung. 2016;194:483–5. https://doi.org/10.1007/s00408-016-9863-6.
Pepke-Zaba J, Delcroix M, Lang I, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011;124:1973–81. https://doi.org/10.1161/CIRCULATIONAHA.110.015008.
Machuca T, de Perrot M. When to refer a patient with chronic thromboembolic pulmonary hypertension for pulmonary endarterectomy. Can J Cardiol. 2015;31:509–14. https://doi.org/10.1016/j.cjca.2015.01.042.
Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62:D92–9. https://doi.org/10.1016/j.jacc.2013.10.024.
Chloros T, Burt C, Dunning J. The role of intraoperative transesophageal echocardiography in identifying a fenestrated occlusion of the inferior vena cava during pulmonary thromboendarterectomy. J Cardiothorac Vasc Anesth. 2018;32:1329–32. https://doi.org/10.1053/j.jvca.2017.09.004.
Galie N, Humbert M, Vachiery J-L, et al. 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). Eur Respir J. 2015;46:903–75. https://doi.org/10.1183/13993003.01032-2015.
Madani M, Mayer E, Fadel E, Jenkins DP. Pulmonary endarterectomy. Patient selection, technical challenges, and outcomes. Ann Am Thorac Soc. 2016;13:S240–7.
Madani MM, Higgins JR. Pulmonary Thromboendarterectomy: Springer International Publishing; 2020. p. 717–26.
Jamieson SW. Re: Outcome after surgical treatment of chronic thromboembolic pulmonary hypertension: dealing with different patient subsets. A single-centre experience. Eur J Cardiothorac Surg. 2016;50:907–8. https://doi.org/10.1093/ejcts/ezw145.
Matthews DT, Le CN, Robbins IM, et al. Severity of pulmonary hypertension and obesity are not associated with worse functional outcomes after pulmonary thromboendarterectomy. Pulm Circ. 2016;6:174–80. https://doi.org/10.1086/685736.
Fernandes TM, Auger WR, Fedullo PF, et al. Baseline body mass index does not significantly affect outcomes after pulmonary thromboendarterectomy. Ann Thorac Surg. 2014;98:1776–81. https://doi.org/10.1016/j.athoracsur.2014.06.045.
Shenoy V, Anton JM, Collard CD, Youngblood SC. Pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension. Anesthesiology. 2014;120:1255–61. https://doi.org/10.1097/ALN.0000000000000228.
Simonneau G, D'Armini AM, Ghofrani H-A, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J. 2015;45:1293–302. https://doi.org/10.1183/09031936.00087114.
Vuylsteke A, Sharples L, Charman G, et al. Circulatory arrest versus cerebral perfusion during pulmonary endarterectomy surgery (PEACOG): a randomised controlled trial. Lancet. 2011;378:1379–87. https://doi.org/10.1016/S0140-6736(11)61144-6.
Lafci G, Tasoglu I, Ulas MM, Yalcinkaya A, Cagli K. Pulmonary endarterectomy: with use of moderate hypothermia and antegrade cerebral perfusion without circulatory arrest. Tex Heart Inst J. 2012;39:65–7.
Ishida K, Masuda M, Imamaki M, Katsumata M, Maruyama T, Miyazaki M. Improvement of tricuspid regurgitation after pulmonary thromboendarterectomy. Asian Cardiovasc Thorac Ann. 2010;18:229–33. https://doi.org/10.1177/0218492310367684.
Kratzert WB, Boyd EK, Saggar R, Channick R. Critical care of patients after pulmonary thromboendarterectomy. J Cardiothorac Vasc Anesth. 2019;33:3110–26. https://doi.org/10.1053/j.jvca.2019.03.005.
Fernandes TM, Poch DS, Papamatheakis DG, et al. Pre-operative determinants of reperfusion lung injury after pulmonary thromboendarterectomy. J Heart Lung Transplant. 2016;35:S175-S176. https://doi.org/10.1016/j.healun.2016.01.487.
Kerr KM, Auger WR, Marsh JJ, et al. Efficacy of methylprednisolone in preventing lung injury following pulmonary thromboendarterectomy. Chest. 2012;141:27–35. https://doi.org/10.1378/chest.10-2639.
Bunclark K, Newnham M, Chiu Y-D, et al. A multicenter study of anticoagulation in operable chronic thromboembolic pulmonary hypertension. J Thromb Haemost. 2020;18:114–22. https://doi.org/10.1111/jth.14649.
Guth S, Wiedenroth CB, Wollenschlager M, et al. Short-term venoarterial extracorporeal membrane oxygenation for massive endobronchial hemorrhage after pulmonary endarterectomy. J Thorac Cardiovasc Surg. 2018;155:643–9. https://doi.org/10.1016/j.jtcvs.2017.09.045.
Edemskiy A, Chernyavskiy M, Tarkova A, Chernyavskiy A. Central extracorporeal membrane oxygenation for treatment of reperfusion oedema following pulmonary thromboendarterectomy: a case report. J Cardiothorac Surg. 2016;11:76. https://doi.org/10.1186/s13019-016-0476-1.
Donahoe L, Granton J, McRae K, et al. Role of extracorporeal life support after pulmonary endarterectomy: a single-centre experience. Interact Cardiovasc Thorac Surg. 2016;23:74–8. https://doi.org/10.1093/icvts/ivw075.
Shetty DP, Nair HC, Shetty V, Punnen J. A novel treatment for pulmonary hemorrhage during thromboendarterectomy surgery. Ann Thorac Surg. 2015;99:e77–8. https://doi.org/10.1016/j.athoracsur.2014.11.060.
Yildizeli B, Arslan O, Tas S, et al. Management of massive pulmonary hemorrhage following pulmonary endarterectomy. Thorac Cardiovasc Surg. 2014;62:89–91. https://doi.org/10.1055/s-0032-1324711.
Reddy S, Rajanbabu BB, Kumar NKS, Rajani I. Temporary clamping of branch pulmonary artery for pulmonary hemorrhage after endarterectomy. Ann Thorac Surg. 2013;96:1459–61. https://doi.org/10.1016/j.athoracsur.2012.12.067.
Morsolini M, Azzaretti A, Orlandoni G, D’Armini AM. Airway bleeding during pulmonary endarterectomy: the “bubbles” technique. J Thorac Cardiovasc Surg. 2013;145:1409–10.
Kolnikova I, Kunstyr J, Lindner J, et al. Extracorporeal membrane oxygenation used in a massive lung bleeding following pulmonary endarterectomy. Prague Med Rep. 2012;113:299–302.
Mangukia C, Forfia P, Vaidya A, et al. Percutaneous coil embolization to manage pulmonary artery hemorrhage after distal endarterectomy. JTCVS Techniques. 2020;4:147–9. https://doi.org/10.1016/j.xjtc.2020.08.050.
Wong DJ, Sampat U, Gibson MA, et al. Tricuspid annular plane systolic excursion in chronic thromboembolic pulmonary hypertension before and after pulmonary thromboendarterectomy. Echocardiography. 2016;33:1805–9. https://doi.org/10.1111/echo.13364.
Marston N, Brown JP, Olson N, et al. Right ventricular strain before and after pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Echocardiography. 2015;32:1115–21. https://doi.org/10.1111/echo.12812.
Cronin B, O'Brien EO, Gu W, Banks D, Maus T. Intraoperative 3-dimensional echocardiography-derived right ventricular volumetric analysis in chronic thromboembolic pulmonary hypertension patients before and after pulmonary thromboendarterectomy. J Cardiothorac Vasc Anesth. 2019;33:1498–503. https://doi.org/10.1053/j.jvca.2018.09.038.
Han QJ, Contijoch F, Forfia PR, Han Y. Four-dimensional flow magnetic resonance imaging visualizes drastic changes in the blood flow in a patient with chronic thromboembolic pulmonary hypertension after pulmonary thromboendarterectomy. Eur Heart J. 2016;37:2802. https://doi.org/10.1093/eurheartj/ehw064.
Waziri F, Mellemkjaer S, Clemmensen TS, et al. Long-term changes of resting and exercise right ventricular systolic performance in patients with chronic thromboembolic pulmonary hypertension following pulmonary thromboendarterectomy - A two-dimensional and three-dimensional echocardiographic study. Echocardiography. 2019;36:1656–65. https://doi.org/10.1111/echo.14456.
Triantafyllidi H, Katogiannis K, Mayer E, Anthi A, Orfanos S, Lekakis J. The time difference between clinical improvement and exercise tolerance increase following pulmonary thromboendarterectomy. Int J Cardiol. 2016;222:267–9. https://doi.org/10.1016/j.ijcard.2016.07.244.
Kamenskaya O, Klinkova A, Loginova I, et al. Determinants of health-related quality of life 1 year after pulmonary thromboendarterectomy. Ann Vasc Surg. 2018;51:254–61. https://doi.org/10.1016/j.avsg.2018.02.019.
Agrawal A, Shah R, Bacchetta MD, Talwar A. Successful pulmonary thromboendarterectomy in a patient with sickle cell disease and associated resolution of a leg ulcer. Lung India. 2018;35:73–7. https://doi.org/10.4103/lungindia.lungindia_47_17.
Marques MB, Wille KM, Ren Z, Sheth M, McGiffin DC. Successful pulmonary thromboendarterectomy in a patient with sickle cell disease treated with a single preoperative red blood cell exchange. Transfusion. 2014;54:1901–2. https://doi.org/10.1111/trf.12651.
Wang W, Wen L, Song Z, Shi W, Wang K, Huang W. Balloon pulmonary angioplasty vs riociguat in patients with inoperable chronic thromboembolic pulmonary hypertension: A systematic review and meta-analysis. Clin Cardiol. 2019;42:741–52.
Funding
None.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Research involving human participants and/or animals
Not applicable as per institutional ethical committee as the scientific information presented in paper does not fall into category of clinical trial or usage of experimental modalities.
Informed consent
Obtained.
Conflicts of interest
The authors declare no competing interests.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Mangukia, C., Rali, P., Desai, P. et al. Pulmonary endarterectomy. Indian J Thorac Cardiovasc Surg 37, 662–672 (2021). https://doi.org/10.1007/s12055-021-01208-y
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12055-021-01208-y