Skip to main content

Advertisement

Log in

Chronic thromboembolic pulmonary hypertension—medical, interventional, and surgical therapy

Chronische thromboembolische pulmonale Hypertonie – medikamentöse, interventionelle und chirurgische Behandlung

  • Main topic
  • Published:
Herz Aims and scope Submit manuscript

This article has been updated

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is an important late sequela of pulmonary embolism and a common form of pulmonary hypertension. Currently, three specific treatment modalities are available: pulmonary endarterectomy, balloon pulmonary angioplasty, and targeted medical therapy. The treatment decision depends mainly on the exact localization of the underlying pulmonary arterial obstructions. Pulmonary endarterectomy is the gold standard treatment of CTEPH. For inoperable patients, riociguat and treprostinil are approved. In addition, interventional therapy is recommended if appropriate target lesions are proven. Evaluation and treatment of patients with CTEPH in experienced centers are mandatory.

Zusammenfassung

Die chronische thromboembolische pulmonale Hypertonie (CTEPH) stellt eine wichtige Spätkomplikation der akuten Lungenembolie und eine häufige Form von pulmonaler Hypertonie dar. Es stehen derzeit 3 Therapiemodalitäten zur Verfügung: die pulmonale Endarteriektomie, die pulmonale Ballonangioplastie und die gezielte medikamentöse Behandlung. Das therapeutische Konzept orientiert sich maßgeblich an der Lokalisation der pulmonalarteriellen Obstruktionen. Die pulmonale Endarteriektomie ist die Behandlung der Wahl bei CTEPH. Für inoperable Patienten sind Riociguat und Treprostinil zugelassene Therapien. Daneben wird bei Vorhandensein entsprechender Zielläsionen die interventionelle Behandlung empfohlen. Evaluation und Therapie von CTEPH-Patienten sollte in entsprechend erfahrenen Zentren erfolgen.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

Change history

  • 27 May 2023

    The second sentence in the abstract should have read ‘Currently, three specific treatmentmodalities are available: pulmonary endarterectomy, balloon pulmonary angioplasty, and targeted medical therapy.’ instead of ‘Currently, three specific treatmentmodalities are available: pulmonary endarterectomy, pulmonary balloon angioplasty, and targeted medical therapy.’

References

  1. Humbert M, Kovacs G, Hoeper MM et al (2022) 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 25:2200879. https://doi.org/10.1183/13993003.00879-2022

    Article  Google Scholar 

  2. Guerin L, Couturaud F, Parent F et al (2014) Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Prevalence of CTEPH after pulmonary embolism. Thromb Haemost 112:598–605. https://doi.org/10.1160/TH13-07-0538

    Article  CAS  PubMed  Google Scholar 

  3. Pengo V, Lensing AWA, Prins MH et al (2004) Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 350(22):2257–2264. https://doi.org/10.1056/NEJMoa032274

    Article  CAS  PubMed  Google Scholar 

  4. Klok FA, van Kralingen KW, van Dijk APJ et al (2010) Prospective cardiopulmonary screening program to detect chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism. Haematologica 95(6):970–975. https://doi.org/10.3324/haematol.2009.018960

    Article  PubMed  PubMed Central  Google Scholar 

  5. Ende-Verhaar YM, Cannegieter SC, Vonk Noordegraaf A et al (2017) Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: a contemporary view of the published literature. Eur Respir J 49(2):1601792. https://doi.org/10.1183/13993003.01792-2016

    Article  PubMed  Google Scholar 

  6. Bochenek ML, Rosinus NS, Lankeit M et al (2017) From thrombosis to fibrosis in chronic thromboembolic pulmonary hypertension. Thromb Haemost 117(4):769–783. https://doi.org/10.1160/TH16-10-0790

    Article  PubMed  Google Scholar 

  7. Klok FA, Dzikowska-Diduch O, Kostrubiec M et al (2016) Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. J Thromb Haemost 14:121–128. https://doi.org/10.1111/jth.13175

    Article  CAS  PubMed  Google Scholar 

  8. Lang IM, Pesavento R, Bonderman D, Yuan JX (2013) Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J 41(2):462–468. https://doi.org/10.1183/09031936.00049312

    Article  PubMed  Google Scholar 

  9. Narechania S, Renapurkar R, Heresi GA (2020) Mimickers of chronic thromboembolic pulmonary hypertension on imaging tests: a review. Pulm Circ 10:2045894019882620. https://doi.org/10.1177/2045894019882620

    Article  PubMed  PubMed Central  Google Scholar 

  10. Riedel M, Stanek V, Widimsky J, Prerovsky I (1982) Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. Chest 81(2):151–158

    Article  CAS  PubMed  Google Scholar 

  11. Konstantinides SV, Meyer G, Becattini C et al (2019) 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 54(3):1901647. https://doi.org/10.1183/13993003.01647-2019

    Article  PubMed  Google Scholar 

  12. Lasch F, Karch A, Koch A et al (2020) Comparison of MRI and VQ-SPECT as a screening test for patients with suspected CTEPH: CHANGE-MRI study design and rationale. Front Cardiovasc Med 7:51. https://doi.org/10.3389/fcvm.2020.00051

    Article  PubMed  PubMed Central  Google Scholar 

  13. de Perrot M, Gopalan D, Jenkins D et al (2021) Evaluation and management of patients with chronic thromboembolic pulmonary hypertension – consensus statement from the ISHLT. J Heart Lung Transplant 40(11):1301–1326. https://doi.org/10.1016/j.healun.2021.07.020

    Article  PubMed  Google Scholar 

  14. Hinrichs JB, Renne J, Hoeper MM, Olsson KM, Wacker FK, Meyer BC (2016) Balloon pulmonary angioplasty: applicability of C‑Arm CT for procedure guidance. Eur Radiol 26(11):4064–4071. https://doi.org/10.1007/s00330-016-4280-z

    Article  PubMed  Google Scholar 

  15. Humbert MS, Simonneau G, Pittrow D et al (2022) Oral anticoagulants (NOAC and VKA) in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 41:716–721. https://doi.org/10.1016/j.healun.2022.02.002

    Article  PubMed  Google Scholar 

  16. Pengo V, Denas G, Zoppellaro G et al (2018) Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood 132:1365–1371. https://doi.org/10.1182/blood-2018-04-848333

    Article  CAS  PubMed  Google Scholar 

  17. Nagel C, Prange F, Guth S et al (2012) Exercise training improves exercise capacity and quality of life in patients with inoperable or residual chronic thromboembolic pulmonary hypertension. PLoS One 7:e41603. https://doi.org/10.1371/journal.pone.0041603

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Nagel C, Nasereddin M, Benjamin N et al (2020) Supervised exercise training in patients with chronic thromboembolic pulmonary hypertension as early follow-up treatment after pulmonary endarterectomy: a prospective cohort study. Respiration 99:577–588. https://doi.org/10.1159/000508754

    Article  PubMed  Google Scholar 

  19. Kim NH, Delcroix M, Jais X et al (2019) Chronic thromboembolic pulmonary hypertension. Eur Respir J 53:1801915. https://doi.org/10.1183/13993003.01915-2018

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Lankeit M, Krieg V, Hobohm L et al (2017) Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. https://doi.org/10.1016/j.healun.2017.06.011

    Article  PubMed  Google Scholar 

  21. Delcroix M, Lang I, Pepke-Zaba J et al (2016) Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Circulation 133:859–871. https://doi.org/10.1161/CIRCULATIONAHA.115.016522

    Article  CAS  PubMed  Google Scholar 

  22. Quadery SR, Swift AJ, Billings CG et al (2018) The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension. Eur Respir J 52:1800589. https://doi.org/10.1183/13993003.00589-2018

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Ghofrani HA, D’Armini AM, Grimminger F et al (2013) Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 369:319–329. https://doi.org/10.1056/NEJMoa1209657

    Article  CAS  PubMed  Google Scholar 

  24. Sadushi-Kolici R, Jansa P, Kopec G et al (2019) Subcutaneous treprostinil for the treatment of severe non-operable chronic thromboembolic pulmonary hypertension (CTREPH): a double-blind, phase 3, randomised controlled trial. Lancet Respir Med 7:239–248. https://doi.org/10.1016/S2213-2600(18)30367-9

    Article  CAS  PubMed  Google Scholar 

  25. Guth S, D’Armini AM, Delcroix M et al (2021) Current strategies for managing chronic thromboembolic pulmonary hypertension: results of the worldwide prospective CTEPH registry. ERJ Open Res. https://doi.org/10.1183/23120541.00850-2020

    Article  PubMed  PubMed Central  Google Scholar 

  26. Brenot P, Jais X, Taniguchi Y et al (2019) French experience of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. Eur Respir J 53:1802095. https://doi.org/10.1183/13993003.02095-2018

    Article  PubMed  PubMed Central  Google Scholar 

  27. Kataoka M, Inami T, Hayashida K et al (2012) Percutaneous transluminal pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 5:756–762. https://doi.org/10.1161/CIRCINTERVENTIONS.112.971390

    Article  PubMed  Google Scholar 

  28. Mahmud E, Behnamfar O, Ang L et al (2018) Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. Interv Cardiol Clin 7:103–117. https://doi.org/10.1016/j.iccl.2017.09.003

    Article  PubMed  Google Scholar 

  29. Mizoguchi H, Ogawa A, Munemasa M et al (2012) Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 5:748–755. https://doi.org/10.1161/CIRCINTERVENTIONS.112.971077

    Article  PubMed  Google Scholar 

  30. Olsson KM, Wiedenroth CB, Kamp JC et al (2017) Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension: the initial German experience. Eur Respir J 49:1602409. https://doi.org/10.1183/13993003.02409-2016

    Article  PubMed  Google Scholar 

  31. Inami T, Kataoka M, Yanagisawa R et al (2016) Long-term outcomes after percutaneous transluminal pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. Circulation 134:2030–2032. https://doi.org/10.1161/CIRCULATIONAHA.116.024201

    Article  PubMed  Google Scholar 

  32. Gerges C, Friewald R, Gerges M et al (2021) Efficacy and safety of percutaneous pulmonary artery subtotal occlusion and chronic total occlusion intervention in chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 14(8):e10243. https://doi.org/10.1161/CIRCINTERVENTIONS.120.010243

    Article  CAS  PubMed  Google Scholar 

  33. Ogawa A, Satoh T, Fukuda T et al (2017) Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: results of a multicenter registry. Circ Cardiovasc Qual Outcomes. https://doi.org/10.1161/CIRCOUTCOMES.117.004029

    Article  PubMed  Google Scholar 

  34. Darocha S, Araszkiewicz A, Kurzyna M et al (2021) Balloon pulmonary angioplasty in technically operable and technically inoperable chronic thromboembolic pulmonary hypertension. J Clin Med. https://doi.org/10.3390/jcm10051038

    Article  PubMed  PubMed Central  Google Scholar 

  35. Wiedenroth CB, Rolf A, Steinhaus K et al (2022) Riociguat and balloon pulmonary angioplasty improve prognosis in patients with inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. https://doi.org/10.1016/j.healun.2022.08.011

    Article  PubMed  Google Scholar 

  36. Jaïs X, Brenot P, Bouvaist H et al (2022) 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. Lancet Respir Med. https://doi.org/10.1016/S2213-2600(22)00214-4

    Article  PubMed  Google Scholar 

  37. Cannon JE, Su L, Kiely DG et al (2016) Dynamic risk stratification of patient long-term outcome after pulmonary endarterectomy: results from the UK national cohort. Circulation 133:1761–1771. https://doi.org/10.1161/CIRCULATIONAHA.115.019470

    Article  PubMed  PubMed Central  Google Scholar 

  38. Wiedenroth CB, Liebetrau C, Breithecker A et al (2016) Combined pulmonary endarterectomy and balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 35:591–596. https://doi.org/10.1016/j.healun.2015.10.030

    Article  PubMed  Google Scholar 

  39. Mayer E, Jenkins D, Lindner J et al (2011) Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg 141:702–710. https://doi.org/10.1016/j.jtcvs.2010.11.024

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Christoph B. Wiedenroth.

Ethics declarations

Conflict of interest

C.B. Wiedenroth has received speaker fees and/or consultant honoraria from Actelion, AOP Orphan Pharmaceuticals AG, Bayer AG, BTG, MSD, OrphaCare GmbH, and Pfizer. D. Pruefer has received speaker fees and/or consultant honoraria from AstraZeneca, Johnson&Johnson, and Bayer AG. E. Mayer has received speaker fees and/or consultant honoraria from Actelion, Janssen, Bayer AG, and MSD. S. Guth has received speaker fees and/or consultant honoraria from Actelion, Bayer AG, MSD, and Pfizer. M.S. D. Adameit declares that she has no competing interests.

For this article no studies with human participants or animals were performed by any of the authors. All studies mentioned were in accordance with the ethical standards indicated in each case.

Additional information

The second sentence in the abstract should have read: “Currently, three specific treatment modalities are available: pulmonary endarterectomy, balloon pulmonary angioplasty, and targeted medical therapy.” instead of “Currently, three specific treatment modalities are available: pulmonary endarterectomy, pulmonary balloon angioplasty, and targeted medical therapy.”

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Wiedenroth, C.B., Pruefer, D., Adameit, M.S.D. et al. Chronic thromboembolic pulmonary hypertension—medical, interventional, and surgical therapy. Herz 48, 280–284 (2023). https://doi.org/10.1007/s00059-023-05172-8

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00059-023-05172-8

Keywords

Schlüsselwörter

Navigation