Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1)

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is precapillary pulmonary hypertension, in which chronic obstruction of large and middle branches of pulmonary arteries (PAs) and secondary changes in the lung microcirculatory bed result in a progressive increase in pulmonary vascular resistance and PA pressure with the development of severe right cardiac dysfunction and heart failure. CTEPH is a unique form of pulmonary hypertension since it is potentially curable by surgical treatment. The diagnostic criteria for CTEPH are a mean PA pressure of ≥25 mm Hg, as evidenced by right heart catheterization; a PA wedge pressure of ≤15 mm Hg; a pulmonary vascular resistance of >2 Wood units; the presence of chronic/organized thrombi/emboli in the elastic PAs (pulmonary trunk, lobular, segmental, subsegmental PAs); effective anticoagulant therapy at therapeutic dosages over at least 3 months. Up to now, our country has had no guidelines for the diagnosis and treatment of this rare severe disease that, when appropriately untreated, has an extremely poor prognosis. The main task in the preparation of this document was to generalize and analyze the data of current registries, multicenter randomized clinical trials, national and international guidelines, and consensus documents recently published on this problem in order to optimize a diagnostic process and treatment in this category of patients. Part 1 gives a definition of CTEPH, its place in the clinical classification, epidemiology and prognosis, risk factors, pathogenesis and morphology, diagnostic approaches and determination of operability in patients, and specific features of differential diagnosis.

About the authors

I E Chazova

T V Martynyuk

References

  1. Чазова И.Е., Авдеев С.Н., Царева Н.А., Волков А.В., Мартынюк Т.В., Наконечников С.Н. Клинические рекомендации по диагностике и лечению легочной гипертонии. Терапевтическийархив. 2014;9:4-23.
  2. Konstantinides CV, Torbick A, Giancarlo Agnelli G et al. 2014 ESC Guidelines on the diagnosis and management of acute pulmonary embolism. Eur Heart J. 2014;35:3033-3080.
  3. Galie 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). Eur Respir J. 2015;46(4):903-975.
  4. Kim NH, Delcroix M, Jenkins DP et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62:D92-D99.
  5. Wilkens H, Lang I, Behr J et al. Recent progress in the diagnosis and management of chronic thromboembolic pulmonary hypertension. Chronic thromboembolic pulmonary hypertension (CTEPH): updated Recommendations of the Cologne Consensus Conference 2011. Respir Investig. 2013;51(3):134-146.
  6. Mehta S, Helmersen D, Provencher et al. Diagnostic evaluation and management of chronic thromboembolic pulmonary hypertension: a clinical practice guideline. Can Respir J. 2010;17(6):301-334.
  7. Pepke-Zaba J, Delcroix M, Lang I et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011;124:1973-1981.
  8. Pengo V, Lensing AW, Prins MH, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350:2257-2264.
  9. Lang I, Simonneau G, Pepke-Zaba J et al. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension: a case-control study. Thromb Haemost. 2013;110:83-91.
  10. Jenkins D, Mayer E, Screaton N, Madani M. State-of-the-art chronic thromboembolic pulmonary hypertension: diagnosis and management. Eur Respir Rev. 2012;21:32-39.
  11. Tunariu N, Gibbs S, 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;48:680-684.
  12. Lang IM, Plank C, Sadushi-Kolici R et al. Imaging in pulmonary hypertension. JACCCardiovasc Imaging. 2010;3:1287-1295.
  13. Guerin L, Couturaud F, Parent F et al. Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Thromb Haemost. 2014;112:598-605.
  14. Ley S, Ley-Zaporozhan J, Pitton MB et al. Diagnostic performance of state-of-the-art imaging techniques for morphological assessment of vascular abnormalities in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Eur Radiol. 2012;22: 607-616.
  15. Bergin CJ, Sirlin S, Hauschildt J et al. Chronic thromboembolism: diagnosis with helical CT and MR imaging with angiographic and surgical correlation. Radiology. 1997;204:695-702.
  16. Skoro-Sajer N, Marta G, Gerges C et al. Surgical specimens, haemodynamics and long-term outcomes after pulmonary endarterectomy. Thorax. 2014;69:116-122.
  17. Pepke-Zaba J, Hoeper MM, Humbert M. Chronic thromboembolic pulmonary hypertension: advances from bench to patient management. Eur Respir J. 2013;41:8-9.

Statistics

Views

Abstract: 547

PDF (Russian): 244

Dimensions

Article Metrics

Metrics Loading ...

PlumX

Refbacks

  • There are currently no refbacks.

Copyright (c) 2016 Chazova I.E., Martynyuk T.V.

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
 

Address of the Editorial Office:

  • Novij Zykovskij proezd, 3, 40, Moscow, 125167

Correspondence address:

  • Novoslobodskaya str 31c4., Moscow, 127005, Russian Federation

Managing Editor:

 

© 2018-2021 "Consilium Medicum" Publishing house


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies