Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry

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Abstract

Aim. To assess demographic and clinical characteristics, to describe of the functional and hemodynamic status, profile of concomitant pathology, data of instrumental examination in chronic thromboembolic pulmonary hypertension (CTEPH) patients; to study the features of specific and supportive therapy according to the data of the Russian national registry.

Materials and methods. From 2012 till 2020 a multicenter, prospective study in 15 regional expert centers of the Russian Federation (www.medibase.pro) included 404 newly diagnosed CTEPH patients over the age of 18 years in the Russian registry of patients with pulmonary arterial hypertension and CTEPH (NCT03707561). The diagnosis was established by European and Russian clinical guidelines for the diagnosis and management of pulmonary hypertension. 154 inoperable CTEPH patients an additional analysis of specific and supportive therapy was performed.

Results. The study included 404 patients (55.6% women and 44.3% men) at the age of 58.6 [48.6; 69.3] years. Median time from symptom onset to the diagnosis verification waswas 2.4 years (from 0.1 to 2.9 years). 79.1% of patients were in the III and IV functional class (World Health Organization) at the time of diagnosis and in 44.1% – with RHF (right heart failure). In assessing the profile of concomitant pathology, it was noted that CTEPH patients were more often with arterial hypertension (39.1%), erosive-ulcerative lesions of the stomach/duodenum (16.1%), atrial fibrillation (13.8%), obesity (13.1%). Distance in 6MWD (6-min walk distance) was 337.2 [250; 422] m, Borg dyspnea index scale 4.1 [3.0; 5.0] points. Hemodynamic parameters according to right heart catheterization were: Mean PAP (pulmonary arterial pressure) (51.1±14.04) mmHg, CO (cardiac output) (3.5±0.98) l/min, CI (cardiac index) (2.0±0.48) l/min/m2, PVR (2008±528) dyn×s/cm5.

Conclusion. According to the Russian registry, inoperable CTEPH patients had precapillary PH (pulmonary hypertension) with severe functional status, in combination with frequent concomitant pathology (arterial hypertension, erosive-ulcerative lesions of the stomach/duodenum, atrial fibrillation, obesity, right heart failure). 66% of inoperable CTEPH patients received specific drug therapy.

About the authors

Zarina S. Valieva

Myasnikov Institute of Clinical Cardiology, National Medical Research Center of Cardiology; Pirogov Russian National Research Medical University

Author for correspondence.
Email: v.zarina.v@gmail.com
ORCID iD: 0000-0002-9041-3604

канд. мед. наук., ст. науч. сотр. отд. легочной гипертензии и заболеваний сердца Института клинической кардиологии им. А.Л. Мясникова ФГБУ «НМИЦ кардиологии»

Russian Federation, Москва

Tamila V. Martynyuk

Myasnikov Institute of Clinical Cardiology, National Medical Research Center of Cardiology; Pirogov Russian National Research Medical University

Email: v.zarina.v@gmail.com
ORCID iD: 0000-0002-9022-8097

д-р мед. наук, рук. отд. легочной гипертензии и заболеваний сердца Института клинической кардиологии им. А.Л. Мясникова ФГБУ «НМИЦ кардиологии», проф. каф. кардиологии фак-та ДПО ФГАОУ ВО «РНИМУ им. Н.И. Пирогова»

Russian Federation, Moscow; Moscow

Sergei N. Nakonechnikov

Pirogov Russian National Research Medical University

Email: v.zarina.v@gmail.com
ORCID iD: 0000-0003-1564-7619

д-р мед. наук, проф. каф. кардиологии ФГАОУ ВО «РНИМУ им. Н.И. Пирогова», ген. дир. РМОАГ

Russian Federation, Moscow

Irina E. Chazova

Myasnikov Institute of Clinical Cardiology, National Medical Research Center of Cardiology

Email: v.zarina.v@gmail.com
ORCID iD: 0000-0002-9822-4357

акад. РАН, д-р мед. наук, проф., зам. ген. дир. по научно-экспертной работе, рук. отд. гипертонии Института клинической кардиологии им. А.Л. Мясникова ФГБУ «НМИЦ кардиологии»

Russian Federation, Moscow

References

  1. Чазова И.Е., Мартынюк Т.В., Валиева З.С., и др. Евразийские рекомендации по диагностике и лечению хронической тромбоэмболической легочной гипертензии (2020). Евразийский кардиологический журнал. 2021;1:6-43 [Chazova IE, Martynyuk TV, Valieva ZS, et al. Eurasian Guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (2020). Eurasian Heart Journal. 2021;1:6-43 (in Russian)]. doi: 10.38109/2225-1685-2021-1-6-43
  2. Мартынюк Т.В. Легочная гипертензия: Диагностика и лечение. М.: Медицинское информационное агентство, 2018. [Martynyuk TV.Pulmonary hypertension. Moscow, Medical news agency, 2018 (in Russian)].
  3. Чазова И.Е., Мартынюк Т.В., Филиппов Е.В. Клинические рекомендации по диагностике и лечению хронической тромбоэмболической легочной гипертензии (I часть). Терапевтический архив. 2016;88(9):90-101 [Chazova IE, Martynyuk TV, Filippov EV. Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1). Terapevticheskii Arkhiv (Ter. Arkh.). 2016;88(9):90-101 (in Russian)]. doi: 10.17116/terarkh201688990-101
  4. 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. doi: 10.1161/CIRCULATIONAHA.110.015008
  5. Чазова И.Е., Валиева З.С., Наконечников С.Н., и др. Особенности клинико-функционального и гемодинамического профиля, лекарственной терапии и оценка прогноза у пациентов с неоперабельной хронической тромбоэмболической и идиопатической легочной гипертензией по данным Российского регистра. Терапевтический архив. 2019;88(10):77-87 [Chazova IE, Valieva ZS, Nakonechnikov SN, et al. Features of clinical, functional and hemodynamics profile, medical treatment and prognosis evaluation in patients with inoperable chronic thromboembolic pulmonary hypertension and idiopathic pulmonary arterial hypertension according to the Russian registry. Terapevticheskii Arkhiv (Ter. Arkh.). 2019;91(9):77-87 (in Russian)]. doi: 10.26442/00403660.2019.09.000343
  6. 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): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67-119. doi: 10.1183/13993003.01032-2015
  7. Radegran G, Kjellstrom B, Ekmehag B, et al. Characteristics and survival of adult Swedish PAH and CTEPH patients 2000–2014. Scand Cardiovasc J. 2016;50(4):243-50. doi: 10.1080/14017431.2016.1185532
  8. Humpert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173:1023-30. doi: 10.1164/rccm.200510-1668OC
  9. Hoeper MM, Huscher D, Pittrow D. Incidence and prevalence of pulmonary arterial hypertension in Germany. Int J Cardiol. 2016;203:612-3. doi: 10.1016/j.ijcard.2015.11.001
  10. Escribano-Subias P, Blanco I, Lopez-Meseguer M, et al. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur Respir J. 2012;40:596-603. doi: 10.1183/09031936.00101211
  11. Baptista R, Meireles J, Agapito A, et al. Pulmonary hypertension in Portugal: first data from a nationwide registry. Biomed Res Int. 2013;2013:489574. doi: 10.1155/2013/489574
  12. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62:D92-9. doi: 10.1016/j.jacc.2013.10.024
  13. Lang IM, Simonneau G, Pepke-Zaba JW, et al. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. Thromb Haemost. 2013;110:83-91. doi: 10.1160/TH13-02-0097
  14. 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(2):462-8. doi: 10.1183/09031936.00049312
  15. Weatherald J, Huertas A, Boucly A, et al. Association Between BMI and Obesity With Survival in Pulmonary Arterial Hypertension. Chest. 2018;154(4):872-81. doi: 10.1016/j.chest.2018.05.006
  16. Hoeper MM, Mayer E, Simonneau G, et al. Chronic thromboembolic pulmonary hypertension. Circulation. 2006;113:2011-20. doi: 10.1161/CIRCULATIONAHA.105.602565
  17. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107:216-23. doi: 10.7326/0003-4819-107-2-216
  18. Ghofrani HA, D’Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369(4):319-29. doi: 10.1056/NEJMoa1209657
  19. Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated Chronic Thromboembolic Pulmonary Hypertension patients. Eur Respir J. 2018;52(5):1800248. doi: 10.1183/13993003.00248-2018
  20. Simonneau G, D’Armini AM, Ghofrani HA, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J. 2015;45(5):1293-302. doi: 10.1183/09031936.00087114
  21. Hoeper MM, McLaughlin VV, Dalaan AM, et al. Treatment of pulmonary hypertension. Lancet Respir Med. 2016;4(4):323-36. doi: 10.1016/S2213-2600(15)00542-1
  22. Pepke-Zaba J, Jansa P, Kim NH, et al. Chronic thromboembolic pulmonary hypertension: role of medical therapy. Eur Respir J. 2013;41(4):985-90. doi: 10.1183/09031936.00201612
  23. Olschewski H, Simonneau G, Galiè N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002;347(5):322-9. doi: 10.1056/NEJMoa020204
  24. Чазова И.Е., Мартынюк Т.В., Филиппов Е.В., и др. Клинические рекомендации по диагностике и лечению хронической тромбоэмболической легочной гипертензии (II часть). Терапевтический архив. 2016;88(10):63-73 [Chazova IE, Martynyuk TV. Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 2). Terapevticheskii Arkhiv (Ter. Arkh.). 2016;88(10):63-73 (in Russian)]. doi: 10.17116/terarkh201688663-73

Supplementary files

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2. Fig. 1. Anticoagulant therapy in inoperable (a) and operable (b) patients with CTEPH.

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3. Fig. 2. Frequency of prescribing PAH-specific therapy (PAH – pulmonary arterial hypertension specific).

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