Влияние демографических характеристик на клиническое течение и результаты терапии пациентов с прекапиллярной легочной гипертензией
Влияние демографических характеристик на клиническое течение и результаты терапии пациентов с прекапиллярной легочной гипертензией
Таран И.Н., Валиева З.С., Белевская А.А., Саидова М.А., Мартынюк Т.В. , Чазова И.Е. Влияние демографических характеристик на клиническое течение и результаты терапии пациентов с прекапиллярной легочной гипертензией. Терапевтический архив. 2021; 93 (3): 283–289. DOI: 10.26442/00403660.2021.03.200655
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Аннотация
Цель. Оценить клиническое течение заболевания и особенности достижения целевых показателей в лечении у лиц с идиопатической легочной гипертензией (ИЛГ) и неоперабельной хронической тромбоэмболической легочной гипертензией (ХТЭЛГ) в зависимости от пола и возраста на момент установления диагноза.
Материалы и методы. В исследование включены 88 пациентов с установленным диагнозом ИЛГ и 38 пациентов с неоперабельной ХТЭЛГ со давлением заклинивания легочной артерии <12 мм рт. ст. и длительностью наблюдения на фоне специфической терапии ≥12 мес. Пациенты с ИЛГ/ХТЭЛГ были разделены на 2 группы в зависимости от возраста на момент установления диагноза: <50 лет (n=69) и >50 лет (n=57) – и в зависимости от пола: 106 женщин и 20 мужчин.
Результаты. У пациентов в возрасте 50 лет и старше на момент установления диагноза выявлен достоверно более тяжелый функциональный класс (ВОЗ). Пациенты мужского пола характеризовались более тяжелыми параметрами центральной гемодинамики (по данным катетеризации правых отделов сердца) и высоким уровнем NT-proBNP на момент верификации диагноза. Пациенты мужского пола, а также в возрасте 50 лет и более имели более выраженное нарушение систолической функции правого желудочка по данным эхокардиографии. Пациенты с ИЛГ моложе 50 лет, по сравнению с более старшими, достоверно чаще к медиане 26,5 мес достигали целей лечения (21% vs 6,45%). Мужчины с ИЛГ/ХТЭЛГ достоверно чаще исходно имели высокий риск летального исхода (90%) при сравнении с женщинами (61%).
Заключение. Принадлежность когорты пациентов с ИЛГ/ неоперабельной ХТЭЛГ к мужскому полу, а также наличие возраста 50 лет и старше, по сравнению с более молодыми, на момент верификации диагноза ассоциируются с менее благоприятным течением заболевания.
Ключевые слова: прекапиллярная легочная гипертензия, идиопатическая легочная гипертензия, хроническая тромбоэмболическая легочная гипертензия, фенотипы, демографический статус
Materials and methods. The study included 88 patients with IPAH and 38 patients with inoperable CTEPH with a PAWP <12 mm Hg and the duration of PAH-specific therapy treatment more than 12 months. IPAH/CTEPH patients were divided into groups depending on age at the time of diagnosis verification: age <50 years (n=69)/ >50 years (n=57), and gender: 106 women/20 men.
Results. Patients with age >50 years at the time of diagnosis verification have significantly more severe functional class (WHO). In IPAH/CTEPH male patients a significant hemodynamic disorder also as significantly higher level of NT-proBNP at the time of diagnosis verification were observed. In men with IPAH/CTEPH and patients aged >50 years more pronounced deterioration of right ventricular systolic function was observed (ECHO). Patients with IPAH younger than 50 years were significantly more likely to achieve the treatment goals by the median 26.5 months of treatment compared to the patients aged >50 years (21% vs 6.45%). Men with IPAH/CTEPH were significantly more likely to have a high risk of death (90%) at baseline compared to the women (61%).
Conclusion. IPAH/inoperable CTEPH patients with male sex, as well as the age 50 years and older at the time of diagnosis verification, compared with younger ones, are associated with a less favorable course of the disease.
Keywords: precapillary pulmonary hypertension, idiopathic pulmonary hypertension, chronic thromboembolic pulmonary hypertension, phenotypes, demographic status
Материалы и методы. В исследование включены 88 пациентов с установленным диагнозом ИЛГ и 38 пациентов с неоперабельной ХТЭЛГ со давлением заклинивания легочной артерии <12 мм рт. ст. и длительностью наблюдения на фоне специфической терапии ≥12 мес. Пациенты с ИЛГ/ХТЭЛГ были разделены на 2 группы в зависимости от возраста на момент установления диагноза: <50 лет (n=69) и >50 лет (n=57) – и в зависимости от пола: 106 женщин и 20 мужчин.
Результаты. У пациентов в возрасте 50 лет и старше на момент установления диагноза выявлен достоверно более тяжелый функциональный класс (ВОЗ). Пациенты мужского пола характеризовались более тяжелыми параметрами центральной гемодинамики (по данным катетеризации правых отделов сердца) и высоким уровнем NT-proBNP на момент верификации диагноза. Пациенты мужского пола, а также в возрасте 50 лет и более имели более выраженное нарушение систолической функции правого желудочка по данным эхокардиографии. Пациенты с ИЛГ моложе 50 лет, по сравнению с более старшими, достоверно чаще к медиане 26,5 мес достигали целей лечения (21% vs 6,45%). Мужчины с ИЛГ/ХТЭЛГ достоверно чаще исходно имели высокий риск летального исхода (90%) при сравнении с женщинами (61%).
Заключение. Принадлежность когорты пациентов с ИЛГ/ неоперабельной ХТЭЛГ к мужскому полу, а также наличие возраста 50 лет и старше, по сравнению с более молодыми, на момент верификации диагноза ассоциируются с менее благоприятным течением заболевания.
Ключевые слова: прекапиллярная легочная гипертензия, идиопатическая легочная гипертензия, хроническая тромбоэмболическая легочная гипертензия, фенотипы, демографический статус
________________________________________________
Materials and methods. The study included 88 patients with IPAH and 38 patients with inoperable CTEPH with a PAWP <12 mm Hg and the duration of PAH-specific therapy treatment more than 12 months. IPAH/CTEPH patients were divided into groups depending on age at the time of diagnosis verification: age <50 years (n=69)/ >50 years (n=57), and gender: 106 women/20 men.
Results. Patients with age >50 years at the time of diagnosis verification have significantly more severe functional class (WHO). In IPAH/CTEPH male patients a significant hemodynamic disorder also as significantly higher level of NT-proBNP at the time of diagnosis verification were observed. In men with IPAH/CTEPH and patients aged >50 years more pronounced deterioration of right ventricular systolic function was observed (ECHO). Patients with IPAH younger than 50 years were significantly more likely to achieve the treatment goals by the median 26.5 months of treatment compared to the patients aged >50 years (21% vs 6.45%). Men with IPAH/CTEPH were significantly more likely to have a high risk of death (90%) at baseline compared to the women (61%).
Conclusion. IPAH/inoperable CTEPH patients with male sex, as well as the age 50 years and older at the time of diagnosis verification, compared with younger ones, are associated with a less favorable course of the disease.
Keywords: precapillary pulmonary hypertension, idiopathic pulmonary hypertension, chronic thromboembolic pulmonary hypertension, phenotypes, demographic status
Список литературы
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3. Dweik RA, Rounds S, Erzurum SC, et al. An official American Thoracic Society Statement: pulmonary hypertension phenotypes. Am J Respir Crit Care Med. 2014;189(3):345-55. doi: 10.1164/rccm.201311-1954ST
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6. Kramm T, Wilkens H, Fuge J, et al. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clin Res Cardiol. 2018;107(7):548-553. doi: 10.1007/s00392-018-1215-5
7. 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;41:462-8. doi: 10.1183/09031936.00049312
8. 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 [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 Russ.)].
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doi: 10.17116/terarkh201688990-101
12. Zamanian RT, Hansmann G, Snook S, et al. Insulin resistance in pulmonary arterial hypertension. Eur Respir J. 2009;33(2):318-24. doi: 10.1016/j.healun.2014.02.016
13. Hjalmarsson C, Radegran G, Kylhammar D, et al. Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5).
doi: 10.1183/13993003.02310-2017
14. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010;122(2):156-63. doi: 10.1161/CIRCULATIONAHA.109.911818
15. Shigeta A, Tanabe N, Shimizu H, et al. Gender differences in chronic thromboembolic pulmonary hypertension in Japan. Circ J. 2008;72(12):2069-74.
16. Simon MA, Deible C, Mathier MA, et al. Phenotyping the right ventricle in patients with pulmonary hypertension. Clin Transl Sci. 2009;2(4):294-9.
doi: 10.1111/j.1752-8062.2009.00134.x
17. Nagueh SF, Smiseth OA, Appleton CP, et al. Recommendations for the Evaluation of Left Ventricular Diastolic Function by Echocardiography: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2016;29(4):277-314. doi: 10.1016/j.echo.2016.01.011
18. Kawut SM, Al-Naamani N, Agerstrand C, et al. Determinants of right ventricular ejection fraction in pulmonary arterial hypertension. Chest. 2009;135(3):752-9. doi: 10.1378/chest.08-1758
19. Jacobs W, van de Veerdonk MC, Trip P, et al. The right ventricle explains sex differences in survival in idiopathic pulmonary arterial hypertension. Chest. 2014;145(6):1230-6. doi: 10.1378/chest.13-1291
20. Kerkhof PLM, Peace RA, Macfarlane PW. Sex- and Age-Related Reference Values in Cardiology, with Annotations and Guidelines for Interpretation. Adv Exp Med Biol. 2018;1065:677-706. doi: 10.1007/978-3-319-77932-4_41
21. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115(5):343-9.
22. Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142(2):448-56. doi: 10.1378/chest.11-1460
23. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50(2). doi: 10.1183/13993003.00740-2017.
24. Humbert M, Farber HW, Ghofrani HA et al. Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(6). doi: 10.1183/13993003.02004-2018
25. Hoeper MM, Pittrow D, Opitz C, et al. Risk assessment in pulmonary arterial hypertension. Eur Respir J. 2018;51(3). doi: 10.1183/13993003.02606-2017
26. Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated Chronic Thromboembolic Pulmonary Hypertension patients. Eur Respir J. 2018. doi: 10.1183/13993003.00248-2018
27. Hoeper MM, Boucly A, Sitbon O. Age, risk and outcomes in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5). doi: 10.1183/13993003.00629-2018
28. Klinger JR, Elliott G, Levine DJ et al. Therapy for Pulmonary Arterial Hypertension in Adults Update of the CHEST Guideline and Expert Panel Report. Chest. 2019;155(3):565-86. doi: 10.1016/j.chest.2018.11.030
29. Campean IA, Lang IM. Treating pulmonary hypertension in the eldery. Expert Opin Pharmacother. 2020;31:1-7. doi: 10.1080/14656566.2020.1739270
30. Barco S, Klok FA, Konstantinides SV et al. Sex-specific differences in chronic thromboembolic pulmonary hypertension. Results from the European CTEPH registry. J Thromb Haemost. 2020;18(1):151-61. doi: 10.1111/jth.14629
2. 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. doi: 10.1016/j.ijcard.2012.10.026
3. Dweik RA, Rounds S, Erzurum SC, et al. An official American Thoracic Society Statement: pulmonary hypertension phenotypes. Am J Respir Crit Care Med. 2014;189(3):345-55. doi: 10.1164/rccm.201311-1954ST
4. McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol. 2013;62(25):51-9. doi: 10.1016/j.jacc.2013.10.023
5. 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
6. Kramm T, Wilkens H, Fuge J, et al. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clin Res Cardiol. 2018;107(7):548-553. doi: 10.1007/s00392-018-1215-5
7. 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;41:462-8. doi: 10.1183/09031936.00049312
8. 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 Russ.) doi: 10.26442/00403660.2019.09.000343
9. 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: 10.1183/13993003.51032-2015
10. Eurasian clinical guidelines on diagnosis and treatment of pulmonary hypertension. Eurasian Heart Journal. 2020;1:78-122 (In Russ.) doi: 10.24411/2076-4766-2020-10002
11. Chazova IE, Martynyuk TV on behalf of the Working Group on Text Preparation for russian Guidelines for the Diagnosis and Treatment of CTEPH. Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1). Terapevticheskii Arkhiv (Ter. Arkh.). 2016;88(9):90-101 (In Russ.)
doi: 10.17116/terarkh201688990-101
12. Zamanian RT, Hansmann G, Snook S, et al. Insulin resistance in pulmonary arterial hypertension. Eur Respir J. 2009;33(2):318-24. doi: 10.1016/j.healun.2014.02.016
13. Hjalmarsson C, Radegran G, Kylhammar D, et al. Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5).
doi: 10.1183/13993003.02310-2017
14. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010;122(2):156-63. doi: 10.1161/CIRCULATIONAHA.109.911818
15. Shigeta A, Tanabe N, Shimizu H, et al. Gender differences in chronic thromboembolic pulmonary hypertension in Japan. Circ J. 2008;72(12):2069-74.
16. Simon MA, Deible C, Mathier MA, et al. Phenotyping the right ventricle in patients with pulmonary hypertension. Clin Transl Sci. 2009;2(4):294-9.
doi: 10.1111/j.1752-8062.2009.00134.x
17. Nagueh SF, Smiseth OA, Appleton CP, et al. Recommendations for the Evaluation of Left Ventricular Diastolic Function by Echocardiography: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2016;29(4):277-314. doi: 10.1016/j.echo.2016.01.011
18. Kawut SM, Al-Naamani N, Agerstrand C, et al. Determinants of right ventricular ejection fraction in pulmonary arterial hypertension. Chest. 2009;135(3):752-9.
doi: 10.1378/chest.08-1758
19. Jacobs W, van de Veerdonk MC, Trip P, et al. The right ventricle explains sex differences in survival in idiopathic pulmonary arterial hypertension. Chest. 2014;145(6):1230-6. doi: 10.1378/chest.13-1291
20. Kerkhof PLM, Peace RA, Macfarlane PW. Sex- and Age-Related Reference Values in Cardiology, with Annotations and Guidelines for Interpretation. Adv Exp Med Biol. 2018;1065:677-706. doi: 10.1007/978-3-319-77932-4_41
21. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115(5):343-9.
22. Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142(2):448-56. doi: 10.1378/chest.11-1460
23. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50(2). doi: 10.1183/13993003.00740-2017.
24. Humbert M, Farber HW, Ghofrani HA et al. Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(6). doi: 10.1183/13993003.02004-2018
25. Hoeper MM, Pittrow D, Opitz C, et al. Risk assessment in pulmonary arterial hypertension. Eur Respir J. 2018;51(3). doi: 10.1183/13993003.02606-2017
26. Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated Chronic Thromboembolic Pulmonary Hypertension patients. Eur Respir J. 2018. doi: 10.1183/13993003.00248-2018
27. Hoeper MM, Boucly A, Sitbon O. Age, risk and outcomes in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5). doi: 10.1183/13993003.00629-2018
28. Klinger JR, Elliott G, Levine DJ et al. Therapy for Pulmonary Arterial Hypertension in Adults Update of the CHEST Guideline and Expert Panel Report. Chest. 2019;155(3):565-86. doi: 10.1016/j.chest.2018.11.030
29. Campean IA, Lang IM. Treating pulmonary hypertension in the eldery. Expert Opin Pharmacother. 2020;31:1-7. doi: 10.1080/14656566.2020.1739270
30. Barco S, Klok FA, Konstantinides SV et al. Sex-specific differences in chronic thromboembolic pulmonary hypertension. Results from the European CTEPH registry. J Thromb Haemost. 2020;18(1):151-61. doi: 10.1111/jth.14629
2. 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. doi: 10.1016/j.ijcard.2012.10.026
3. Dweik RA, Rounds S, Erzurum SC, et al. An official American Thoracic Society Statement: pulmonary hypertension phenotypes. Am J Respir Crit Care Med. 2014;189(3):345-55. doi: 10.1164/rccm.201311-1954ST
4. McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol. 2013;62(25):51-9. doi: 10.1016/j.jacc.2013.10.023
5. 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
6. Kramm T, Wilkens H, Fuge J, et al. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clin Res Cardiol. 2018;107(7):548-553. doi: 10.1007/s00392-018-1215-5
7. 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;41:462-8. doi: 10.1183/09031936.00049312
8. 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 [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 Russ.)].
doi: 10.26442/00403660.2019.09.000343
9. 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: 10.1183/13993003.51032-2015
10. Евразийские клинические рекомендации по диагностике и лечению легочной гипертензии. Евразийский кардиологический журнал. 2020;1:78-122 [Eurasian clinical guidelines on diagnosis and treatment of pulmonary hypertension. Eurasian Heart Journal. 2020;1:78-122 (In Russ.)]. doi: 10.24411/2076-4766-2020-10002
11. Чазова И.Е., Мартынюк Т.В. от имени рабочей группы по разработке подготовке текста Российских рекомендаций по диагностике и лечению ХТЭЛГ. Клинические рекомендации по диагностике и лечению хронической тромбоэмболической легочной гипертензии (I часть). Терапевтический архив. 2016;88(9):90-101 (Chazova IE, Martynyuk TV on behalf of the Working Group on Text Preparation for russian Guidelines for the Diagnosis and Treatment of CTEPH. Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1). Terapevticheskii Arkhiv (Ter. Arkh.). 2016;88(9):90-101 (In Russ.)].
doi: 10.17116/terarkh201688990-101
12. Zamanian RT, Hansmann G, Snook S, et al. Insulin resistance in pulmonary arterial hypertension. Eur Respir J. 2009;33(2):318-24. doi: 10.1016/j.healun.2014.02.016
13. Hjalmarsson C, Radegran G, Kylhammar D, et al. Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5).
doi: 10.1183/13993003.02310-2017
14. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010;122(2):156-63. doi: 10.1161/CIRCULATIONAHA.109.911818
15. Shigeta A, Tanabe N, Shimizu H, et al. Gender differences in chronic thromboembolic pulmonary hypertension in Japan. Circ J. 2008;72(12):2069-74.
16. Simon MA, Deible C, Mathier MA, et al. Phenotyping the right ventricle in patients with pulmonary hypertension. Clin Transl Sci. 2009;2(4):294-9.
doi: 10.1111/j.1752-8062.2009.00134.x
17. Nagueh SF, Smiseth OA, Appleton CP, et al. Recommendations for the Evaluation of Left Ventricular Diastolic Function by Echocardiography: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2016;29(4):277-314. doi: 10.1016/j.echo.2016.01.011
18. Kawut SM, Al-Naamani N, Agerstrand C, et al. Determinants of right ventricular ejection fraction in pulmonary arterial hypertension. Chest. 2009;135(3):752-9. doi: 10.1378/chest.08-1758
19. Jacobs W, van de Veerdonk MC, Trip P, et al. The right ventricle explains sex differences in survival in idiopathic pulmonary arterial hypertension. Chest. 2014;145(6):1230-6. doi: 10.1378/chest.13-1291
20. Kerkhof PLM, Peace RA, Macfarlane PW. Sex- and Age-Related Reference Values in Cardiology, with Annotations and Guidelines for Interpretation. Adv Exp Med Biol. 2018;1065:677-706. doi: 10.1007/978-3-319-77932-4_41
21. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115(5):343-9.
22. Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142(2):448-56. doi: 10.1378/chest.11-1460
23. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50(2). doi: 10.1183/13993003.00740-2017.
24. Humbert M, Farber HW, Ghofrani HA et al. Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(6). doi: 10.1183/13993003.02004-2018
25. Hoeper MM, Pittrow D, Opitz C, et al. Risk assessment in pulmonary arterial hypertension. Eur Respir J. 2018;51(3). doi: 10.1183/13993003.02606-2017
26. Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated Chronic Thromboembolic Pulmonary Hypertension patients. Eur Respir J. 2018. doi: 10.1183/13993003.00248-2018
27. Hoeper MM, Boucly A, Sitbon O. Age, risk and outcomes in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5). doi: 10.1183/13993003.00629-2018
28. Klinger JR, Elliott G, Levine DJ et al. Therapy for Pulmonary Arterial Hypertension in Adults Update of the CHEST Guideline and Expert Panel Report. Chest. 2019;155(3):565-86. doi: 10.1016/j.chest.2018.11.030
29. Campean IA, Lang IM. Treating pulmonary hypertension in the eldery. Expert Opin Pharmacother. 2020;31:1-7. doi: 10.1080/14656566.2020.1739270
30. Barco S, Klok FA, Konstantinides SV et al. Sex-specific differences in chronic thromboembolic pulmonary hypertension. Results from the European CTEPH registry. J Thromb Haemost. 2020;18(1):151-61. doi: 10.1111/jth.14629
________________________________________________
2. 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. doi: 10.1016/j.ijcard.2012.10.026
3. Dweik RA, Rounds S, Erzurum SC, et al. An official American Thoracic Society Statement: pulmonary hypertension phenotypes. Am J Respir Crit Care Med. 2014;189(3):345-55. doi: 10.1164/rccm.201311-1954ST
4. McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol. 2013;62(25):51-9. doi: 10.1016/j.jacc.2013.10.023
5. 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
6. Kramm T, Wilkens H, Fuge J, et al. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clin Res Cardiol. 2018;107(7):548-553. doi: 10.1007/s00392-018-1215-5
7. 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;41:462-8. doi: 10.1183/09031936.00049312
8. 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 Russ.) doi: 10.26442/00403660.2019.09.000343
9. 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: 10.1183/13993003.51032-2015
10. Eurasian clinical guidelines on diagnosis and treatment of pulmonary hypertension. Eurasian Heart Journal. 2020;1:78-122 (In Russ.) doi: 10.24411/2076-4766-2020-10002
11. Chazova IE, Martynyuk TV on behalf of the Working Group on Text Preparation for russian Guidelines for the Diagnosis and Treatment of CTEPH. Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1). Terapevticheskii Arkhiv (Ter. Arkh.). 2016;88(9):90-101 (In Russ.)
doi: 10.17116/terarkh201688990-101
12. Zamanian RT, Hansmann G, Snook S, et al. Insulin resistance in pulmonary arterial hypertension. Eur Respir J. 2009;33(2):318-24. doi: 10.1016/j.healun.2014.02.016
13. Hjalmarsson C, Radegran G, Kylhammar D, et al. Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5).
doi: 10.1183/13993003.02310-2017
14. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010;122(2):156-63. doi: 10.1161/CIRCULATIONAHA.109.911818
15. Shigeta A, Tanabe N, Shimizu H, et al. Gender differences in chronic thromboembolic pulmonary hypertension in Japan. Circ J. 2008;72(12):2069-74.
16. Simon MA, Deible C, Mathier MA, et al. Phenotyping the right ventricle in patients with pulmonary hypertension. Clin Transl Sci. 2009;2(4):294-9.
doi: 10.1111/j.1752-8062.2009.00134.x
17. Nagueh SF, Smiseth OA, Appleton CP, et al. Recommendations for the Evaluation of Left Ventricular Diastolic Function by Echocardiography: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2016;29(4):277-314. doi: 10.1016/j.echo.2016.01.011
18. Kawut SM, Al-Naamani N, Agerstrand C, et al. Determinants of right ventricular ejection fraction in pulmonary arterial hypertension. Chest. 2009;135(3):752-9.
doi: 10.1378/chest.08-1758
19. Jacobs W, van de Veerdonk MC, Trip P, et al. The right ventricle explains sex differences in survival in idiopathic pulmonary arterial hypertension. Chest. 2014;145(6):1230-6. doi: 10.1378/chest.13-1291
20. Kerkhof PLM, Peace RA, Macfarlane PW. Sex- and Age-Related Reference Values in Cardiology, with Annotations and Guidelines for Interpretation. Adv Exp Med Biol. 2018;1065:677-706. doi: 10.1007/978-3-319-77932-4_41
21. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115(5):343-9.
22. Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142(2):448-56. doi: 10.1378/chest.11-1460
23. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50(2). doi: 10.1183/13993003.00740-2017.
24. Humbert M, Farber HW, Ghofrani HA et al. Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(6). doi: 10.1183/13993003.02004-2018
25. Hoeper MM, Pittrow D, Opitz C, et al. Risk assessment in pulmonary arterial hypertension. Eur Respir J. 2018;51(3). doi: 10.1183/13993003.02606-2017
26. Delcroix M, Staehler G, Gall H, et al. Risk assessment in medically treated Chronic Thromboembolic Pulmonary Hypertension patients. Eur Respir J. 2018. doi: 10.1183/13993003.00248-2018
27. Hoeper MM, Boucly A, Sitbon O. Age, risk and outcomes in idiopathic pulmonary arterial hypertension. Eur Respir J. 2018;51(5). doi: 10.1183/13993003.00629-2018
28. Klinger JR, Elliott G, Levine DJ et al. Therapy for Pulmonary Arterial Hypertension in Adults Update of the CHEST Guideline and Expert Panel Report. Chest. 2019;155(3):565-86. doi: 10.1016/j.chest.2018.11.030
29. Campean IA, Lang IM. Treating pulmonary hypertension in the eldery. Expert Opin Pharmacother. 2020;31:1-7. doi: 10.1080/14656566.2020.1739270
30. Barco S, Klok FA, Konstantinides SV et al. Sex-specific differences in chronic thromboembolic pulmonary hypertension. Results from the European CTEPH registry. J Thromb Haemost. 2020;18(1):151-61. doi: 10.1111/jth.14629
Авторы
И.Н. Таран*1, З.С. Валиева2, А.А. Белевская2, М.А. Саидова2, Т.В. Мартынюк2,3, И.Е. Чазова2
1 ФГБНУ «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний», Кемерово, Россия;
2 Институт клинической кардиологии им. А.Л. Мясникова ФГБУ «Национальный медицинский исследовательский центр кардиологии» Минздрава России, Москва, Россия;
3 ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России, Москва, Россия
*zaviirina@mail.ru
1 Research Institute for Complex Issues of Cardiovascular Diseases, Kemerovo, Russia;
2 Myasnikov Scientific Research Institute of Clinical Cardiology, National Medical Research Center of Cardiology, Moscow, Russia;
3 Pirogov Russian National Research Medical University, Moscow, Russia
*zaviirina@mail.ru
1 ФГБНУ «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний», Кемерово, Россия;
2 Институт клинической кардиологии им. А.Л. Мясникова ФГБУ «Национальный медицинский исследовательский центр кардиологии» Минздрава России, Москва, Россия;
3 ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России, Москва, Россия
*zaviirina@mail.ru
________________________________________________
1 Research Institute for Complex Issues of Cardiovascular Diseases, Kemerovo, Russia;
2 Myasnikov Scientific Research Institute of Clinical Cardiology, National Medical Research Center of Cardiology, Moscow, Russia;
3 Pirogov Russian National Research Medical University, Moscow, Russia
*zaviirina@mail.ru
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