Semin Respir Crit Care Med 2022; 43(06): 936-945
DOI: 10.1055/s-0042-1755570
Review Article

Chronic Thromboembolic Pulmonary Disease and Chronic Thromboembolic Pulmonary Hypertension

Martine Remy-Jardin
1   Department of Thoracic Imaging, Heart & Lung Institute, Lille Cedex, France
,
Antoine Hutt
1   Department of Thoracic Imaging, Heart & Lung Institute, Lille Cedex, France
,
Jacques Remy
1   Department of Thoracic Imaging, Heart & Lung Institute, Lille Cedex, France
› Author Affiliations

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) and chronic thromboembolic pulmonary disease (CTEPD) are two terms characterizing symptomatic patients with chronic thromboembolic occlusions of pulmonary arteries with or without pulmonary hypertension at rest. Their diagnosis follows evolving schemas that integrate technological advances of pivotal imaging modalities among which computed tomography angiography plays a major role. This review article summarizes the current knowledge on the natural history of acute pulmonary embolism and its evolution toward chronic pulmonary embolism, as well as the imaging clues, for the identification of chronically obstructed pulmonary arteries. The requirements for imaging at the time of therapeutic decisions are also described in the light of recent updates in the literature from multidisciplinary groups of experts. Because an early diagnosis of CTEPH remains a challenge for the medical community, several practical issues are included in this article with the objective of improving the knowledge and collaboration between radiologists and clinicians in service to the patient.



Publication History

Article published online:
28 October 2022

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  • References

  • 1 Simonneau G, Montani D, Celermajer DS. et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 2019; 53 (01) 1801913
  • 2 Sista AK, Klok FA. Late outcomes of pulmonary embolism: the post-PE syndrome. Thromb Res 2018; 164: 157-162
  • 3 Konstantinides SV, Meyer G, Becattini C. et al; The Task Force for the diagnosis and management of acute pulmonary embolism of the European Society of Cardiology (ESC). 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (ERS): The Task Force for the diagnosis and management of acute pulmonary embolism of the European Society of Cardiology (ESC). Eur Respir J 2019; 54 (03) 1901647
  • 4 Kim NH, Delcroix M, Jais X. et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J 2019; 53 (01) 1801915
  • 5 Dong BR, Hao Q, Yue J, Wu T, Liu GJ. Thrombolytic therapy for pulmonary embolism. Cochrane Database Syst Rev 2009; 3 (03) CD004437
  • 6 Remy-Jardin M, Louvegny S, Remy J. et al. Acute central thromboembolic disease: posttherapeutic follow-up with spiral CT angiography. Radiology 1997; 203 (01) 173-180
  • 7 Cosmi B, Nijkeuter M, Valentino M, Huisman MV, Barozzi L, Palareti G. Residual emboli on lung perfusion scan or multidetector computed tomography after a first episode of acute pulmonary embolism. Intern Emerg Med 2011; 6 (06) 521-528
  • 8 van Es J, Douma RA, Kamphuisen PW. et al. Clot resolution after 3 weeks of anticoagulant treatment for pulmonary embolism: comparison of computed tomography and perfusion scintigraphy. J Thromb Haemost 2013; 11 (04) 679-685
  • 9 Saeedan MB, Bullen J, Heresi GA, Rizk A, Karim W, Renapurkar RD. Morphologic and functional dual-energy CT parameters in patients with chronic thromboembolic pulmonary hypertension and chronic thromboembolic disease. AJR Am J Roentgenol 2020; 215 (06) 1335-1341
  • 10 Galiè N, Humbert M, Vachiery JL. 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 (04) 903-975
  • 11 Soler X, Hoh CK, Test VJ, Kerr KM, Marsh JJ, Morris TA. Single photon emission computed tomography in chronic thromboembolic pulmonary hypertension. Respirology 2011; 16 (01) 131-137
  • 12 Wang M, Wu D, Ma R. et al. Comparison of V/Q SPECT and CT angiography for the diagnosis of chronic thromboembolic pulmonary hypertension. Radiology 2020; 296 (02) 420-429
  • 13 Dong C, Zhou M, Liu D, Long X, Guo T, Kong X. Diagnostic accuracy of computed tomography for chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis. PLoS One 2015; 10 (04) e0126985
  • 14 Nakazawa T, Watanabe Y, Hori Y. et al. Lung perfused blood volume images with dual-energy computed tomography for chronic thromboembolic pulmonary hypertension: correlation to scintigraphy with single-photon emission computed tomography. J Comput Assist Tomogr 2011; 35 (05) 590-595
  • 15 Meinel FG, Graef A, Thierfelder KM. et al. Automated quantification of pulmonary perfused blood volume by dual-energy CTPA in chronic thromboembolic pulmonary hypertension. Röfo Fortschr Geb Röntgenstr Neuen Bildgeb Verfahr 2014; 186 (02) 151-156
  • 16 Takagi H, Ota H, Sugimura K. et al. Dual-energy CT to estimate clinical severity of chronic thromboembolic pulmonary hypertension: comparison with invasive right heart catheterization. Eur J Radiol 2016; 85 (09) 1574-1580
  • 17 Lambert L, Michalek P, Burgetova A. The diagnostic performance of CT pulmonary angiography in the detection of chronic thromboembolic pulmonary hypertension-systematic review and meta-analysis. Eur Radiol 2022; (e-pub ahead of print) DOI: 10.1007/s00330-022-08804-5.
  • 18 Dournes G, Verdier D, Montaudon M. et al. Dual-energy CT perfusion and angiography in chronic thromboembolic pulmonary hypertension: diagnostic accuracy and concordance with radionuclide scintigraphy. Eur Radiol 2014; 24 (01) 42-51
  • 19 Masy M, Giordano J, Petyt G. et al. Dual-energy CT (DECT) lung perfusion in pulmonary hypertension: concordance rate with V/Q scintigraphy in diagnosing chronic thromboembolic pulmonary hypertension (CTEPH). Eur Radiol 2018; 28 (12) 5100-5110
  • 20 Kröger JR, Gerhardt F, Dumitrescu D. et al. Diagnosis of pulmonary hypertension using spectral-detector CT. Int J Cardiol 2019; 285: 80-85
  • 21 Remy-Jardin M, Ryerson CJ, Schiebler ML. et al. Imaging of pulmonary hypertension in adults: a position paper from the Fleischner Society. Eur Respir J 2021; 57 (01) 2004455
  • 22 Delcroix M, Torbicki A, Gopalan D. et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J 2021; 57 (06) 2002828
  • 23 Kiley DG, Levin DL, Hassoun PM. et al. Statement on imaging and pulmonary hypertension from the Pulmonary Vascular Research Institute (PVRI). Pulm Circ 2019; 9: 2045894019841990
  • 24 Galiè N, Kim NHS. Pulmonary microvascular disease in chronic thromboembolic pulmonary hypertension. Proc Am Thorac Soc 2006; 3 (07) 571-576
  • 25 Simonneau G, Torbicki A, Dorfmüller P, Kim N. The pathophysiology of chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26 (143) 160112
  • 26 Dorfmüller P, Günther S, Ghigna MR. et al. Microvascular disease in chronic thromboembolic pulmonary hypertension: a role for pulmonary veins and systemic vasculature. Eur Respir J 2014; 44 (05) 1275-1288
  • 27 Reichelt A, Hoeper MM, Galanski M, Keberle M. Chronic thromboembolic pulmonary hypertension: evaluation with 64-detector row CT versus digital substraction angiography. Eur J Radiol 2009; 71 (01) 49-54
  • 28 Sugiura T, Tanabe N, Matsuura Y. et al. Role of 320-slice CT imaging in the diagnostic workup of patients with chronic thromboembolic pulmonary hypertension. Chest 2013; 143 (04) 1070-1077
  • 29 DiChiacchio L, Pasrija C, Boulos FM. et al. Occult chronic thromboembolic disease in patients presenting for surgical pulmonary embolectomy. Ann Thorac Surg 2019; 108 (04) 1183-1188
  • 30 Ende-Verhaar YM, Meijboom LJ, Kroft LJM. et al. Usefulness of standard computed tomography pulmonary angiography performed for acute pulmonary embolism for identification of chronic thromboembolic pulmonary hypertension: results of the InShape III study. J Heart Lung Transplant 2019; 38 (07) 731-738
  • 31 Klok FA, Ageno W, Ay C. et al. Optimal follow-up after acute pulmonary embolism: a position paper of the European Society of Cardiology Working Group on pulmonary circulation and right ventricular function, in collaboration with the European Society of Cardiology Working Group on atherosclerosis and vascular biology, endorsed by the European Respiratory Society. Eur Heart J 2022; 43 (03) 183-189
  • 32 Klok FA, van der Hulle T, den Exter PL, Lankeit M, Huisman MV, Konstantinides S. The post-PE syndrome: a new concept for chronic complications of pulmonary embolism. Blood Rev 2014; 28 (06) 221-226
  • 33 Klok FA, Barco S. Follow-up after acute pulmonary embolism. Hamostaseologie 2018; 38 (01) 22-32
  • 34 Klok FA, Dzikowska-Diduch O, Kostrubiec M. et al. Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. J Thromb Haemost 2016; 14 (01) 121-128