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
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
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; 91 (9): 77–87. DOI: 10.26442/00403660.2019.09.000343
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Аннотация
Цель: провести комплексный анализ клинического, функционального, гемодинамического профиля пациентов с неоперабельной хронической тромбоэмболической легочной гипертензией (ХТЭЛГ) в сравнении с группой идиопатической легочной гипертензии (ИЛГ) по данным Российского регистра на момент верификации диагноза, а также изучить особенности лекарственной терапии и ее влияние на прогноз.
Материалы и методы. В ретроспективном исследовании приняли участие 193 больных с ИЛГ и 130 – с неоперабельной ХТЭЛГ в возрасте старше 18 лет. Все больные являлись участниками Российского регистра (www.medibase.pro) в 15 экспертных центрах России в период с 01.01.2012 по 31.12.2018 г. Проведен сравнительный анализ клинических, функциональных, гемодинамических параметров. Диагноз устанавливался в соответствии с алгоритмом, предложенным в Российских рекомендациях по диагностике и лечению легочной гипертензии (ЛГ) и ХТЭЛГ (2016 г.).
Результаты. В рамках данного исследования проанализировано 193 пациента с ИЛГ (31 мужчина и 162 женщины) и 130 – с неоперабельной ХТЭЛГ (40 мужчин и 90 женщин). Пациенты с ХТЭЛГ были старше по сравнению с пациентами с ИЛГ: 52,2 [41,1; 60,6] года и 36,5 [26,8; 36,5] лет соответственно. Период от появления симптомов до подтверждения диагноза ХТЭЛГ составлял в среднем 1,08 [доверительный интервал (ДИ): 0,2; 3,1] года, при ИЛГ – 2,01 [ДИ: 0,6; 4,2] года. Более 80% пациентов с неоперабельной ХТЭЛГ имели III и IV функциональный класс (ФК) по классификации Всемирной организации здравоохранения на момент постановки диагноза против 61% пациентов с ИЛГ.
Заключение. Пациенты с неоперабельной формой ХТЭЛГ по сравнению с группой ИЛГ, по данным Российского регистра, старше, характеризуются более тяжелым исходным ФК и частой сопутствующей патологией (артериальная гипертония, хроническая сердечная недостаточность, повышенный индекс массы тела). При сопоставимой степени ЛГ по оценке среднего давления в легочной артерии (срДЛА) при эхокардиографии в группе пациентов с неоперабельной ХТЭЛГ отмечалась большая площадь правого предсердия. При оценке параметров центральной гемодинамики при ХТЭЛГ выявлены достоверно более высокие значения срДЛА и более низкие значения сатурации крови кислородом. В условиях частого применения стартовой комбинированной терапии у 75% пациентов c ХТЭЛГ (у 40% – комбинация риоцигуата и илопроста) 5-летняя выживаемость достигла 93% против 86,5% у пациентов с ИЛГ.
Ключевые слова: идиопатическая легочная гипертензия, неоперабельная хроническая тромбоэмболическая легочная гипертензия, риоцигуат, национальный регистр легочной гипертензии.
Materials and methods. In the retrospective study 193 patients with IPAH and 130 patients with inoperable CTEPH older than 18 years were included. All included patients were the participants of Russian registry (www.medibase.pro) in 15 Russian expert centers during the period from 01.01.2012 to 31.12.2018 year. The diagnosis was verified according to the algorithm, reflected in the Russian guidelines on diagnosis and treatment of PH and CTEPH (2016 year). The comparison analysis of clinical, functional, hemodynamics parameters in patients with IPAH and inoperable CTEPH was made.
Results. The status of 193 patients with IPAH (32 male and 162 female) and 130 patients with inoperable CTEPH – (40 male and 90 female) was analyzed during the study. The CTEPH patients were older compared to the IPAH patients: 52.2 [41.1; 60.6] and 36.5 [26.8; 36.5] years, respectively. The median period since symptom occurrence till CTEPH verification was 1.08 [0.2; 3.1] years, in IPAH patients – 2.01 [0.6; 4.2] years. More than 80% of inoperable CTEPH patients had III and IV functional class (FC) according to the World Health Organization classification at the time of diagnosis verification versus 61% of IPAH patients. According to echocardiography the level of mean pulmonary arterial pressure was comparable in two groups of PH patients. However in inoperable CTEPH patients the right atrial area was larger. The significantly higher value of mean pulmonary arterial pressure and lower value oxygen saturation of arterial blood according to the right heart catheterization were revealed. The 5-year survival in CTEPH patients, receiving initial dual combination therapy in 75% cases (in 40% – combination of riociguat and iloprost ) was 93% versus 86.5% in patients with IPAH.
Conclusion: It was revealed, that inoperable CTEPH patients were significantly older with severe functional and hemodynamics status at the time of diagnosis verification, although with higher level of 5-year survival compared to the IPAH patients according to the Russian registry.
Keywords: idiopathic pulmonary arterial hypertension, inoperable chronic thromboembolic pulmonary hypertension, riociguat, cardiopulmonary exercise test, national registry of pulmonary hypertension.
Материалы и методы. В ретроспективном исследовании приняли участие 193 больных с ИЛГ и 130 – с неоперабельной ХТЭЛГ в возрасте старше 18 лет. Все больные являлись участниками Российского регистра (www.medibase.pro) в 15 экспертных центрах России в период с 01.01.2012 по 31.12.2018 г. Проведен сравнительный анализ клинических, функциональных, гемодинамических параметров. Диагноз устанавливался в соответствии с алгоритмом, предложенным в Российских рекомендациях по диагностике и лечению легочной гипертензии (ЛГ) и ХТЭЛГ (2016 г.).
Результаты. В рамках данного исследования проанализировано 193 пациента с ИЛГ (31 мужчина и 162 женщины) и 130 – с неоперабельной ХТЭЛГ (40 мужчин и 90 женщин). Пациенты с ХТЭЛГ были старше по сравнению с пациентами с ИЛГ: 52,2 [41,1; 60,6] года и 36,5 [26,8; 36,5] лет соответственно. Период от появления симптомов до подтверждения диагноза ХТЭЛГ составлял в среднем 1,08 [доверительный интервал (ДИ): 0,2; 3,1] года, при ИЛГ – 2,01 [ДИ: 0,6; 4,2] года. Более 80% пациентов с неоперабельной ХТЭЛГ имели III и IV функциональный класс (ФК) по классификации Всемирной организации здравоохранения на момент постановки диагноза против 61% пациентов с ИЛГ.
Заключение. Пациенты с неоперабельной формой ХТЭЛГ по сравнению с группой ИЛГ, по данным Российского регистра, старше, характеризуются более тяжелым исходным ФК и частой сопутствующей патологией (артериальная гипертония, хроническая сердечная недостаточность, повышенный индекс массы тела). При сопоставимой степени ЛГ по оценке среднего давления в легочной артерии (срДЛА) при эхокардиографии в группе пациентов с неоперабельной ХТЭЛГ отмечалась большая площадь правого предсердия. При оценке параметров центральной гемодинамики при ХТЭЛГ выявлены достоверно более высокие значения срДЛА и более низкие значения сатурации крови кислородом. В условиях частого применения стартовой комбинированной терапии у 75% пациентов c ХТЭЛГ (у 40% – комбинация риоцигуата и илопроста) 5-летняя выживаемость достигла 93% против 86,5% у пациентов с ИЛГ.
Ключевые слова: идиопатическая легочная гипертензия, неоперабельная хроническая тромбоэмболическая легочная гипертензия, риоцигуат, национальный регистр легочной гипертензии.
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Materials and methods. In the retrospective study 193 patients with IPAH and 130 patients with inoperable CTEPH older than 18 years were included. All included patients were the participants of Russian registry (www.medibase.pro) in 15 Russian expert centers during the period from 01.01.2012 to 31.12.2018 year. The diagnosis was verified according to the algorithm, reflected in the Russian guidelines on diagnosis and treatment of PH and CTEPH (2016 year). The comparison analysis of clinical, functional, hemodynamics parameters in patients with IPAH and inoperable CTEPH was made.
Results. The status of 193 patients with IPAH (32 male and 162 female) and 130 patients with inoperable CTEPH – (40 male and 90 female) was analyzed during the study. The CTEPH patients were older compared to the IPAH patients: 52.2 [41.1; 60.6] and 36.5 [26.8; 36.5] years, respectively. The median period since symptom occurrence till CTEPH verification was 1.08 [0.2; 3.1] years, in IPAH patients – 2.01 [0.6; 4.2] years. More than 80% of inoperable CTEPH patients had III and IV functional class (FC) according to the World Health Organization classification at the time of diagnosis verification versus 61% of IPAH patients. According to echocardiography the level of mean pulmonary arterial pressure was comparable in two groups of PH patients. However in inoperable CTEPH patients the right atrial area was larger. The significantly higher value of mean pulmonary arterial pressure and lower value oxygen saturation of arterial blood according to the right heart catheterization were revealed. The 5-year survival in CTEPH patients, receiving initial dual combination therapy in 75% cases (in 40% – combination of riociguat and iloprost ) was 93% versus 86.5% in patients with IPAH.
Conclusion: It was revealed, that inoperable CTEPH patients were significantly older with severe functional and hemodynamics status at the time of diagnosis verification, although with higher level of 5-year survival compared to the IPAH patients according to the Russian registry.
Keywords: idiopathic pulmonary arterial hypertension, inoperable chronic thromboembolic pulmonary hypertension, riociguat, cardiopulmonary exercise test, national registry of pulmonary hypertension.
Список литературы
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21. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107:216-23.
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25. Irene M. Lang, Gérald Simonneau, Joanna W. Pepke-Zaba, et al. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. Thromb Haemost. 2013;110:83-91.
26. 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.
27. 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.
28. Baptista R, Meireles J, Agapito A, et al. Pulmonary hypertension in Portugal: first data from a nationwide registry. Biomed Res Int. 2013;2013:489574.
29. Austin C, Alassas K, Burger C, et al. Echocardiographic assessment of estimated right atrial pressure and size predicts mortality in pulmonary arterial hypertension. Chest. 2015;147:198-208.
30. 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 [Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 2). Chazova IE, Martynyuk TV on behalf of the Working Group on Text Preparation for Russian Guidelines for the Diagnosis and Treatment of CTEPH. Therapeutic Archive. 2016;88(10):63-73 (In Russ.)].
31. Ling Y, Johnson MK, Kiely DG, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790-6.
32. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary Arterial Hypertension: Baseline Characteristics From the REVEAL Registry. Chest. 2010;137:376-87.
33. Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2013;168(2):871-80.
34. 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.
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2. [Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1) Chazova IE, Martynyuk TV on behalf of the Working Group on Text Preparation for Russian Guidelines for the Diagnosis and Treatment of CTEPH. Therapeutic Archive. 2016;88(9):90-101 (In Russ.)].
3. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62:D92-D99.
4. Hoeper MM, Mayer E, Simonneau G, et al. Chronic thromboembolic pulmonary hypertension. Circulation. 2006;113:2011-20.
5. 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.
6. Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated Chronic Thromboembolic Pulmonary Hypertension patients. Eur Respir J. 2018;pii:1800248.
7. 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 May;45(5):1293-302.
8. Minai OA, Bonner N, Mathai SC, Busse D, et al. Assessment Of Patient-Reported Health Status In Chronic Thromboembolic Pulmonary Arterial Hypertension Patients Treated With Riociguat: 2-Year Results From The Chest-2 Extension Study. Value Health. 2015 Nov;18(7):A398-9.
9. Hoeper MM, McLaughlin VV, Dalaan AM, Satoh T, GaliП N. Treatment of pulmonary hypertension. Lancet Respir Med. 2016 Apr;4(4):323-36.
10. Olschewski H, Simonneau G, Galiè N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002;347(5):322-9.
11. 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.
12. Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2):pii: 1700889.
13. 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.
14. Hoeper MM, Huscher D, Pittrow D. Incidence and prevalence of pulmonary arterial hypertension in Germany. Int J Cardiol. 2016;203:612-3.
15. 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.
16. 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.
17. Lang IM, Pesavento R, Bonderman D, et al. Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2013;41(2):462-8.
18. Kramm T, et al. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clinical Research in Cardiology. 2018;1-6.
19. [Martynyuk TV, Nakonechnikov SN, Chazova IE. On the way of optimization of approaches to treatment of chronic thromboembolic pulmonary hypertension. Consilium Medicum. 2016;(5):53-8 (In Russ.)].
20. 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.
21. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107:216-23.
22. 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.
23. [Chazova IE, Arkhipova OA, Martynyuk TV. Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry. Therapeutic Archive. 2019;91(1):10-31 (In Russ.)]. doi: 10.26442/00403660.2019.01.000024
24. [Valieva ZS, Valeeva EG, Glukhova SI, Martynyuk TV, Chazova IE. The development of a screening questionnaire to improve the early detection of pulmonary arterial hypertension. Systemic hypertension 2014;11(4):62-7 (In Russ.)].
25. Irene M. Lang, Gérald Simonneau, Joanna W. Pepke-Zaba, et al. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. Thromb Haemost. 2013;110:83-91.
26. 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.
27. 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.
28. Baptista R, Meireles J, Agapito A, et al. Pulmonary hypertension in Portugal: first data from a nationwide registry. Biomed Res Int. 2013;2013:489574.
29. Austin C, Alassas K, Burger C, et al. Echocardiographic assessment of estimated right atrial pressure and size predicts mortality in pulmonary arterial hypertension. Chest. 2015;147:198-208.
30. [Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 2). Chazova IE, Martynyuk TV on behalf of the Working Group on Text Preparation for Russian Guidelines for the Diagnosis and Treatment of CTEPH. Therapeutic Archive. 2016;88(10):63-73 (In Russ.)].
31. Ling Y, Johnson MK, Kiely DG, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790-6.
32. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary Arterial Hypertension: Baseline Characteristics From the REVEAL Registry. Chest. 2010;137:376-87.
33. Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2013;168(2):871-80.
34. 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.
35. MCJ van Thor, L ten Klooster, RJ Snijder, et al. Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension. IJC Heart&Vasculature. 2019;(22):163-8.
2. Чазова И.Е., Мартынюк Т.В., Филиппов Е.В. Клинические рекомендации по диагностике и лечению хронической тромбоэмболической легочной гипертензии (1 часть). Терапевтический архив. 2016;88(9):90-101 [Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1) Chazova IE, Martynyuk TV on behalf of the Working Group on Text Preparation for Russian Guidelines for the Diagnosis and Treatment of CTEPH. Therapeutic Archive. 2016;88(9):90-101 (In Russ.)].
3. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62:D92-D99.
4. Hoeper MM, Mayer E, Simonneau G, et al. Chronic thromboembolic pulmonary hypertension. Circulation. 2006;113:2011-20.
5. 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.
6. Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated Chronic Thromboembolic Pulmonary Hypertension patients. Eur Respir J. 2018;pii:1800248.
7. 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 May;45(5):1293-302.
8. Minai OA, Bonner N, Mathai SC, Busse D, et al. Assessment Of Patient-Reported Health Status In Chronic Thromboembolic Pulmonary Arterial Hypertension Patients Treated With Riociguat: 2-Year Results From The Chest-2 Extension Study. Value Health. 2015 Nov;18(7):A398-9.
9. Hoeper MM, McLaughlin VV, Dalaan AM, Satoh T, GaliП N. Treatment of pulmonary hypertension. Lancet Respir Med. 2016 Apr;4(4):323-36.
10. Olschewski H, Simonneau G, Galiè N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002;347(5):322-9.
11. 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.
12. Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2):pii: 1700889.
13. 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.
14. Hoeper MM, Huscher D, Pittrow D. Incidence and prevalence of pulmonary arterial hypertension in Germany. Int J Cardiol. 2016;203:612-3.
15. 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.
16. 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.
17. Lang IM, Pesavento R, Bonderman D, et al. Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2013;41(2):462-8.
18. Kramm T, et al. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clinical Research in Cardiology. 2018;1-6.
19. Мартынюк Т.В., Наконечников С.Н., Чазова И.Е. На пути оптимизации подходов к лечению хронической тромбоэмболической легочной гипертензии. Consilium Medicum. 2016;(5):53-8 [Martynyuk TV, Nakonechnikov SN, Chazova IE. On the way of optimization of approaches to treatment of chronic thromboembolic pulmonary hypertension. Consilium Medicum. 2016;(5):53-8 (In Russ.)].
20. 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.
21. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107:216-23.
22. 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.
23. Чазова И.Е., Архипова О.А., Мартынюк Т.В. Легочная артериальная гипертензия в России: анализ шестилетнего наблюдения по данным Национального регистра. Терапевтический архив. 2019;91(1):10-31 [Chazova IE, Arkhipova OA, Martynyuk TV. Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry. Therapeutic Archive. 2019;91(1):10-31 (In Russ.)]. doi: 10.26442/00403660.2019.01.000024
24. Валиева З.С., Валеева Э.Г., Глухова С.И., Мартынюк Т.В., Чазова И.Е. Разработка скринингового опросника для улучшения ранней диагностики легочной артериальной гипертензии. Системные гипертензии. 2014;11(4):62-7. [Valieva ZS, Valeeva EG, Glukhova SI, Martynyuk TV, Chazova IE. The development of a screening questionnaire to improve the early detection of pulmonary arterial hypertension. Systemic hypertension 2014;11(4):62-7 (In Russ.)].
25. Irene M. Lang, Gérald Simonneau, Joanna W. Pepke-Zaba, et al. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. Thromb Haemost. 2013;110:83-91.
26. 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.
27. 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.
28. Baptista R, Meireles J, Agapito A, et al. Pulmonary hypertension in Portugal: first data from a nationwide registry. Biomed Res Int. 2013;2013:489574.
29. Austin C, Alassas K, Burger C, et al. Echocardiographic assessment of estimated right atrial pressure and size predicts mortality in pulmonary arterial hypertension. Chest. 2015;147:198-208.
30. 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 [Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 2). Chazova IE, Martynyuk TV on behalf of the Working Group on Text Preparation for Russian Guidelines for the Diagnosis and Treatment of CTEPH. Therapeutic Archive. 2016;88(10):63-73 (In Russ.)].
31. Ling Y, Johnson MK, Kiely DG, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790-6.
32. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary Arterial Hypertension: Baseline Characteristics From the REVEAL Registry. Chest. 2010;137:376-87.
33. Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2013;168(2):871-80.
34. 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.
35. MCJ van Thor, L ten Klooster, RJ Snijder, et al. Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension. IJC Heart&Vasculature. 2019;(22):163-8.
________________________________________________
2. [Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1) Chazova IE, Martynyuk TV on behalf of the Working Group on Text Preparation for Russian Guidelines for the Diagnosis and Treatment of CTEPH. Therapeutic Archive. 2016;88(9):90-101 (In Russ.)].
3. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62:D92-D99.
4. Hoeper MM, Mayer E, Simonneau G, et al. Chronic thromboembolic pulmonary hypertension. Circulation. 2006;113:2011-20.
5. 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.
6. Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated Chronic Thromboembolic Pulmonary Hypertension patients. Eur Respir J. 2018;pii:1800248.
7. 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 May;45(5):1293-302.
8. Minai OA, Bonner N, Mathai SC, Busse D, et al. Assessment Of Patient-Reported Health Status In Chronic Thromboembolic Pulmonary Arterial Hypertension Patients Treated With Riociguat: 2-Year Results From The Chest-2 Extension Study. Value Health. 2015 Nov;18(7):A398-9.
9. Hoeper MM, McLaughlin VV, Dalaan AM, Satoh T, GaliП N. Treatment of pulmonary hypertension. Lancet Respir Med. 2016 Apr;4(4):323-36.
10. Olschewski H, Simonneau G, Galiè N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002;347(5):322-9.
11. 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.
12. Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2):pii: 1700889.
13. 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.
14. Hoeper MM, Huscher D, Pittrow D. Incidence and prevalence of pulmonary arterial hypertension in Germany. Int J Cardiol. 2016;203:612-3.
15. 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.
16. 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.
17. Lang IM, Pesavento R, Bonderman D, et al. Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2013;41(2):462-8.
18. Kramm T, et al. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clinical Research in Cardiology. 2018;1-6.
19. [Martynyuk TV, Nakonechnikov SN, Chazova IE. On the way of optimization of approaches to treatment of chronic thromboembolic pulmonary hypertension. Consilium Medicum. 2016;(5):53-8 (In Russ.)].
20. 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.
21. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107:216-23.
22. 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.
23. [Chazova IE, Arkhipova OA, Martynyuk TV. Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry. Therapeutic Archive. 2019;91(1):10-31 (In Russ.)]. doi: 10.26442/00403660.2019.01.000024
24. [Valieva ZS, Valeeva EG, Glukhova SI, Martynyuk TV, Chazova IE. The development of a screening questionnaire to improve the early detection of pulmonary arterial hypertension. Systemic hypertension 2014;11(4):62-7 (In Russ.)].
25. Irene M. Lang, Gérald Simonneau, Joanna W. Pepke-Zaba, et al. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. Thromb Haemost. 2013;110:83-91.
26. 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.
27. 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.
28. Baptista R, Meireles J, Agapito A, et al. Pulmonary hypertension in Portugal: first data from a nationwide registry. Biomed Res Int. 2013;2013:489574.
29. Austin C, Alassas K, Burger C, et al. Echocardiographic assessment of estimated right atrial pressure and size predicts mortality in pulmonary arterial hypertension. Chest. 2015;147:198-208.
30. [Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 2). Chazova IE, Martynyuk TV on behalf of the Working Group on Text Preparation for Russian Guidelines for the Diagnosis and Treatment of CTEPH. Therapeutic Archive. 2016;88(10):63-73 (In Russ.)].
31. Ling Y, Johnson MK, Kiely DG, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790-6.
32. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary Arterial Hypertension: Baseline Characteristics From the REVEAL Registry. Chest. 2010;137:376-87.
33. Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2013;168(2):871-80.
34. 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.
35. MCJ van Thor, L ten Klooster, RJ Snijder, et al. Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension. IJC Heart&Vasculature. 2019;(22):163-8.
Авторы
И.Е. Чазова 1, З.С. Валиева 1, С.Н. Наконечников 1, И.Н. Таран 1,2, Т.В. Мартынюк 1
1 Отдел легочной гипертензии и заболеваний сердца Научно-исследовательского института клинической кардиологии им. А.Л. Мясникова ФГБУ «Национальный медицинский исследовательский центр кардиологии» Минздрава России, Москва, Россия;
2 ФГБНУ «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний», лаборатория реабилитации отделения мультифокального атеросклероза, Кемерово, Россия
1 National Medical Research Center of Cardiology, Myasnikov Scientific Research Institute of Clinical Cardiology, Department of Pulmonary Hypertension and Heart Diseases, Moscow, Russia;
2 Scientific Research Institute for Complex Issues of Cardiovascular Diseases, Department of Multifocal Atherosclerosis, laboratory of rehabilitation, Kemerovo, Russia
1 Отдел легочной гипертензии и заболеваний сердца Научно-исследовательского института клинической кардиологии им. А.Л. Мясникова ФГБУ «Национальный медицинский исследовательский центр кардиологии» Минздрава России, Москва, Россия;
2 ФГБНУ «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний», лаборатория реабилитации отделения мультифокального атеросклероза, Кемерово, Россия
________________________________________________
1 National Medical Research Center of Cardiology, Myasnikov Scientific Research Institute of Clinical Cardiology, Department of Pulmonary Hypertension and Heart Diseases, Moscow, Russia;
2 Scientific Research Institute for Complex Issues of Cardiovascular Diseases, Department of Multifocal Atherosclerosis, laboratory of rehabilitation, Kemerovo, Russia
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