open access

Vol 80, No 6 (2022)
Expert opinion
Published online: 2022-06-06
Get Citation

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation on screening for chronic thromboembolic pulmonary hypertension patients after acute pulmonary embolism: Update

Michał Ciurzyński1, Marcin Kurzyna2, Grzegorz Kopeć34, Piotr Błaszczak5, Łukasz Chrzanowski6, Karol Kamiński78, Katarzyna Mizia-Stec910, Tatiana Mularek-Kubzdela11, Andrzej Biederman12, Dariusz Zieliński12, Piotr Pruszczyk1, Adam Torbicki2, Ewa Mroczek13
DOI: 10.33963/KP.a2022.0141
·
Pubmed: 35665906
·
Kardiol Pol 2022;80(6):723-732.
Affiliations
  1. Department of Internal Medicine and Cardiology with the Center for Diagnosis and Treatment of Venous Thromboembolism, Medical University of Warsaw, Warszawa, Poland
  2. Department of Pulmonary Circulation, Thromboembolism and Cardiology, Medical Center for Postgraduate Education in Warsaw, European Health Center, Otwock, Poland
  3. Pulmonary Circulation Center, Department of Cardiac and Vascular Diseases, Jagiellonian University Medical College, Kraków, Poland
  4. Department of Cardiovascular Diseases with Cardiac Intensive Care Unit, John Paul II Hospital, Kraków, Poland
  5. Department of Cardiology and Intensive Cardiac Care, Stefan Cardinal Wyszynski Provincial Specialist Hospital, Lublin, Poland
  6. Department of Cardiology, Medical University of Lodz, Łódź, Poland
  7. Department of Population Medicine and Lifestyle Diseases Prevention, Medical University of Bialystok, Białystok, Poland
  8. Department of Cardiology, University Hospital in Bialystok, Białystok, Poland
  9. 1st Department of Cardiology, Faculty of Medicine, Medical University of Silesia in Katowice, Katowice, Poland
  10. European Reference Network for Rare, Low Prevalence, or Complex Diseases of the Heart (ERN GUARD Heart)
  11. 1st Department of Cardiology, Poznan University of Medical Sciences, Poznań, Poland
  12. Department of Cardiac Surgery, Medicover Hospital, Warszawa, Poland
  13. Department of Cardiology, University Clinical Hospital, Institute of Heart Diseases, Wrocław, Poland

open access

Vol 80, No 6 (2022)
Expert opinion and position paper
Published online: 2022-06-06

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism (APE). Both pharmacological and invasive treatments for CTEPH are available in Poland, and awareness of the disease among physicians is growing. It has been suggested that the COVID-19 pandemic may increase the incidence of CTEPH and facilitate disease detection during more advanced stages of the illness. Thus, the Polish Cardiac Society’s Working Group on Pulmonary Circulation, in cooperation with independent experts in this field, launched the updated statement on the algorithm to guide a CTEPH diagnosis in patients with previous APE. CTEPH should be suspected in individuals after APE with dyspnea, despite at least 3 months of effective anticoagulation, particularly when specific risk factors are present. Echocardiography is the main screening tool for CTEPH. A diagnostic workup of patients with significant clinical suspicion of CTEPH and right ventricular overload evident on echocardiography should be performed in reference centers. Pulmonary scintigraphy is a safe and highly sensitive screening test for CTEPH. Computed tomography pulmonary angiography with precise detection of thromboembolic residues in the pulmonary circulation is important for the planning of a pulmonary thromboendarterectomy. Right heart catheterization definitively confirms the presence of pulmonary hypertension and direct pulmonary angiography allows for the identification of lesions suitable for thromboendarterectomy or balloon pulmonary angioplasty. In this document, we propose a diagnostic algorithm for patients with suspected CTEPH. With an individualized and sequential diagnostic strategy, each patient can be provided with suitable and tailored therapy provided by a dedicated CTEPH Heart Team.

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism (APE). Both pharmacological and invasive treatments for CTEPH are available in Poland, and awareness of the disease among physicians is growing. It has been suggested that the COVID-19 pandemic may increase the incidence of CTEPH and facilitate disease detection during more advanced stages of the illness. Thus, the Polish Cardiac Society’s Working Group on Pulmonary Circulation, in cooperation with independent experts in this field, launched the updated statement on the algorithm to guide a CTEPH diagnosis in patients with previous APE. CTEPH should be suspected in individuals after APE with dyspnea, despite at least 3 months of effective anticoagulation, particularly when specific risk factors are present. Echocardiography is the main screening tool for CTEPH. A diagnostic workup of patients with significant clinical suspicion of CTEPH and right ventricular overload evident on echocardiography should be performed in reference centers. Pulmonary scintigraphy is a safe and highly sensitive screening test for CTEPH. Computed tomography pulmonary angiography with precise detection of thromboembolic residues in the pulmonary circulation is important for the planning of a pulmonary thromboendarterectomy. Right heart catheterization definitively confirms the presence of pulmonary hypertension and direct pulmonary angiography allows for the identification of lesions suitable for thromboendarterectomy or balloon pulmonary angioplasty. In this document, we propose a diagnostic algorithm for patients with suspected CTEPH. With an individualized and sequential diagnostic strategy, each patient can be provided with suitable and tailored therapy provided by a dedicated CTEPH Heart Team.

Get Citation

Keywords

chronic thromboembolic pulmonary hypertension, acute pulmonary embolism, echocardiography, diagnostic algorithm, computed tomography pulmonary angiography

Supp./Additional Files (1)
Supplementary material
Download
162KB
About this article
Title

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation on screening for chronic thromboembolic pulmonary hypertension patients after acute pulmonary embolism: Update

Journal

Kardiologia Polska (Polish Heart Journal)

Issue

Vol 80, No 6 (2022)

Article type

Expert opinion

Pages

723-732

Published online

2022-06-06

Page views

680

Article views/downloads

204

DOI

10.33963/KP.a2022.0141

Pubmed

35665906

Bibliographic record

Kardiol Pol 2022;80(6):723-732.

Keywords

chronic thromboembolic pulmonary hypertension
acute pulmonary embolism
echocardiography
diagnostic algorithm
computed tomography pulmonary angiography

Authors

Michał Ciurzyński
Marcin Kurzyna
Grzegorz Kopeć
Piotr Błaszczak
Łukasz Chrzanowski
Karol Kamiński
Katarzyna Mizia-Stec
Tatiana Mularek-Kubzdela
Andrzej Biederman
Dariusz Zieliński
Piotr Pruszczyk
Adam Torbicki
Ewa Mroczek

References (32)
  1. Konstantinides S, Meyer G, Becattini C, et al. 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (ERS). Eur Heart J. 2020; 41(4): 543–603.
  2. Delcroix M, Torbicki A, Gopalan D, et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J. 2021; 57(6).
  3. Kopeć G, Dzikowska-Diduch O, Mroczek E, et al. Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL). Ther Adv Chronic Dis. 2021; 12: 20406223211002961.
  4. 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(3): 183–189.
  5. Klok FA, van der Hulle T, den Exter PL, et al. The post-PE syndrome: a new concept for chronic complications of pulmonary embolism. Blood Rev. 2014; 28(6): 221–226.
  6. Kahn SR, Comerota AJ, Cushman M, et al. The postthrombotic syndrome: evidence-based prevention, diagnosis, and treatment strategies: a scientific statement from the American Heart Association. Circulation. 2014; 130(18): 1636–1661.
  7. Klok FA, Barco S, Konstantinides SV, et al. Determinants of diagnostic delay in chronic thromboembolic pulmonary hypertension: results from the European CTEPH Registry. Eur Respir J. 2018; 52(6).
  8. Ende-Verhaar YM, van den Hout WB, Bogaard HJ, et al. Healthcare utilization in chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. J Thromb Haemost. 2018; 16(11): 2168–2174.
  9. Boon GJ, Ende-Verhaar YM, Beenen LFM, et al. Prediction of chronic thromboembolic pulmonary hypertension with standardised evaluation of initial computed tomography pulmonary angiography performed for suspected acute pulmonary embolism. Eur Radiol. 2022; 32(4): 2178–2187.
  10. 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.
  11. Tunariu N, Gibbs SJR, 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.
  12. Gopalan D, Blanchard D, Auger WR. Diagnostic evaluation of chronic thromboembolic pulmonary hypertension. Ann Am Thorac Soc. 2016; 13 Suppl 3: S222–S239.
  13. Baumgartner H, De Backer J, Baumgartner H, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021; 42(6): 563–645.
  14. Kurzyna M, Araszkiewicz A, Błaszczak P, et al. Summary of recommendations for the haemodynamic and angiographic assessment of the pulmonary circulation. Joint statement of the Polish Cardiac Society's Working Group on Pulmonary Circulation and Association of Cardiovascular Interventions. Kardiol Pol. 2015; 73(1): 63–68.
  15. Hoeper MM, Lee SH, Voswinckel R, et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol. 2006; 48(12): 2546–2552.
  16. Kopeć G, Kurzyna M, Mroczek E, et al. Database of pulmonary hypertension in the Polish population (BNP‑PL): design of the registry. Kardiol Pol. 2019; 77(10): 972–974.
  17. Siennicka A, Darocha S, Banaszkiewicz M, et al. Treatment of chronic thromboembolic pulmonary hypertension in a multidisciplinary team. Ther Adv Respir Dis. 2019; 13: 1753466619891529.
  18. Guth S, D'Armini AM, Delcroix M, et al. Current strategies for managing chronic thromboembolic pulmonary hypertension: results of the worldwide prospective CTEPH Registry. ERJ Open Res. 2021; 7(3).
  19. Kerr KM, Elliott CG, Chin K, et al. Results From the United States Chronic Thromboembolic Pulmonary Hypertension Registry: Enrollment Characteristics and 1-Year Follow-up. Chest. 2021; 160(5): 1822–1831.
  20. Wieteska M, Biederman A, Kurzyna M, et al. Outcome of medically versus surgically treated patients with chronic thromboembolic pulmonary hypertension. Clin Appl Thromb Hemost. 2016; 22(1): 92–99.
  21. Darocha S, Roik M, Kopeć G, et al. Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension: a multicentre registry. EuroIntervention. 2022; 17(13): 1104–1111.
  22. Bunclark K, Newnham M, Chiu YD, et al. A multicenter study of anticoagulation in operable chronic thromboembolic pulmonary hypertension. J Thromb Haemost. 2020; 18(1): 114–122.
  23. Humbert M, Simonneau G, Pittrow D, et al. Safety of Riociguat in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension with Concomitant Novel Oral Anticoagulants or Vitamin K Antagonist Use: Data from the EXPERT Registry. Am J Respir Crit Care Med. 2020; 201: A6043.
  24. Ghofrani HA, D'Armini AM, Kim NH, et al. CHEST-1 Study Group. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013; 369(4): 319–329.
  25. Sadushi-Kolici R, Jansa P, Kopec G, et al. 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. 2019; 7(3): 239–248.
  26. Montani D, Savale L, Beurnier A, et al. Multidisciplinary approach for post-acute COVID-19 syndrome: time to break down the walls. Eur Respir J. 2021; 58(1).
  27. Zuin M, Bilato C, Quadretti L, et al. Incidence of acute pulmonary embolism in COVID-19 patients: Systematic review and meta-analysis. Eur J Intern Med. 2020; 82(4): 29–37.
  28. Pellicori P, Doolub G, Wong CM, et al. COVID-19 and its cardiovascular effects: a systematic review of prevalence studies. Cochrane Database Syst Rev. 2021; 3: CD013879.
  29. Miró Ò, Jiménez S, Mebazaa A, et al. Pulmonary embolism in patients with COVID-19: incidence, risk factors, clinical characteristics, and outcome. Eur Heart J. 2021; 42(33): 3127–3142.
  30. Newman J, Boubriak I, Jenkins D, et al. Rising COVID-19 related acute pulmonary emboli but falling national chronic thromboembolic pulmonary hypertension referrals from a large national dataset. ERJ Open Res. 2021; 7(4).
  31. Belge C, Quarck R, Godinas L, et al. COVID-19 in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: a reference centre survey. ERJ Open Res. 2020; 6(4).
  32. Wieteska-Miłek M, Szmit S, Florczyk M, et al. Fear of COVID-19, Anxiety and Depression in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension during the Pandemic. J Clin Med. 2021; 10(18).

Regulations

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

By VM Media Sp. z o.o. VM Group Sp.k., ul. Świętokrzyska 73 , 80–180 Gdańsk, Poland

phone:+48 58 320 94 94, fax:+48 58 320 94 60, e-mail: viamedica@viamedica.pl