Email this article Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry
- Authors: Chazova IE1, Arkhipova OA1, Martynyuk TV1
-
Affiliations:
- A.L. Myasnikov Research Institute of Cardiology of National Medical Research Center of Cardiology of the Ministry of Health of the Russian Federation
- Issue: Vol 91, No 1 (2019)
- Pages: 25-31
- Section: Editorial article
- URL: https://ogarev-online.ru/0040-3660/article/view/32882
- DOI: https://doi.org/10.26442/00403660.2019.01.000024
- ID: 32882
Cite item
Full Text
Abstract
Aim. Assess the prevalence, clinical course, current therapy, and mortality in patients with pulmonary arterial hypertension (PAH) in the National Registry. Materials and methods. In the prospective study we included patients over 18 years of age with diagnosed PAH [idiopathic PAH (IPAH); Drug - and Toxin-Induced Pulmonary Hypertension; inherited PAH; PAH associated with congenital heart disease (PAH-CHD); PAH associated with systemic connective tissue disease (PAH-CTD); PAH associated with HIV infection (PAH-HIV); with portal pulmonary hypertension (portoPAH)]. The observation was carried out in 15 expert centers of Russia from 01.01.2012 to 31.12.2017. Results. Our registry included 470 patients with PAH: IPAH - 41.5%, PAH-CHD - 36%, PAH-CTD - 19.5%, inherited PAH - 0.4%, portoPAH - 1.9%, PAH-HIV - 0.4%, Drug - and Toxin-Induced PAH - 0.4%. The prevalence among women was 84%. The mean age at the time of patient enrollment in the registry for the overall group of PAH was 42.7±15.3 years. The distance in the 6-minute walking test was 361.3±129.3 m. Among all patients with PAH, 65% had functional class (FC) III/IV at the time of diagnosis, among IPAH - 62%. 69.9% received PAH-specific therapy, of which 62.1% - monotherapy, 32.7% - dual combination therapy, and 5.2% triple therapy. Sildenafil is the most commonly prescribed drug in the regimen of monotherapy. 31.6% of patients were treated with bosentan, 6.4% - riociguat, 3.4% - ambrisentan, 2.1% - macitentan and 2.0% iloprost. Survival of patients with PAH was 98.9% at 1 year of follow-up, 94.1% at 3 years and 86.0% at 5 years. Conclusion. The registry data indirectly indicates the need to increase efforts aimed at improving the diagnosis of systemic connective tissue diseases in adults, as well as congenital heart defects in children for timely surgical treatment. In recent years, PAH-specific drugs of the new generation have been introduced into clinical practice, but currently in Russia there are no parenteral prostanoids, which are recommended for the most severe patients.
Full Text
##article.viewOnOriginalSite##About the authors
I E Chazova
A.L. Myasnikov Research Institute of Cardiology of National Medical Research Center of Cardiology of the Ministry of Health of the Russian Federationкад. РАМН, проф., д.м.н., директор Института клинической кардиологии им. А.Л. Мясникова ФГБУ «НМИЦ кардиологии» Минздрава России, руководитель отд. гипертонии; ORCID: 0000-0002-9822-4357 Moscow, Russia
O A Arkhipova
A.L. Myasnikov Research Institute of Cardiology of National Medical Research Center of Cardiology of the Ministry of Health of the Russian Federation
Email: olga_ark@list.ru
к.м.н., н.с. отд. легочной гипертензии и заболеваний сердца Института клинической кардиологии им. А.Л. Мясникова ФГБУ «НМИЦ кардиологии» Минздрава России; тел.: 8(495)414-68-33; ORCID: 0000-0002-8520-2183 Moscow, Russia
T V Martynyuk
A.L. Myasnikov Research Institute of Cardiology of National Medical Research Center of Cardiology of the Ministry of Health of the Russian Federationд.м.н., руководитель отд. легочной гипертензии и заболеваний сердца Института клинической кардиологии им. А.Л. Мясникова ФГБУ «НМИЦ кардиологии» Минздрава России Moscow, Russia
References
- Galiè N, Humbert M, Vachiery J.L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2015;46:903-75. doi: 10.1093/eurheartj/ehv317
- Rich S, Dantzker D.R, Ayres S.M, et al. Primary pulmonary hypertension: a national prospective study. Ann Intern Med. 1987;107:216-23. doi: 10.7326/0003-4819-107-2-216
- Kane G.C, Maradit-Kremers H, Slusser J, et al. Integration of Clinical and Hemodynamic Parameters in the Prediction of Long - term Survival in Patients With Pulmonary Arterial Hypertension. Chest. 2011;139(6):1285-93. doi: 10.1378/chest.10-1293
- Badesch D.B, Raskob G.E, Elliott C.G, et al. Pulmonary Arterial Hypertension: Baseline Characteristics from the REVEAL Registry. Chest. 2010;137:376-87. doi: 10.1378/chest.09-1140
- Shapiro S, Traiger G.L, Michelle Turner M, et al. Sex Differences in the Diagnosis, Treatment, and Outcome of Patients With Pulmonary Arterial Hypertension Enrolled in the Registry to Evaluate Early and Long - term Pulmonary Arterial Hypertension Disease Management. Chest. 2012;141(2):363-73. doi: 10.1378/chest. 10-3114
- Ling Y, Johnson M.K, Kiely D.G, 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 Oct 15;186(8):790-6. doi: 10.1164/rccm.201203-0383OC
- Humbert 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
- Mc Goon M.D, Benza R.L, Escribano-Subias P, et al. Pulmonary Arterial Hypertension: Epidemiology and Registries. J Am College of Cardiology. 2013;62(25,Suppl D):51-9. doi: 10.1016/j.jacc.2013.10.023
- Архипова О.А., Валиева З.С., Мартынюк Т.В., Наконечников С.Н., Чазова И.Е. Особенности легочной артериальной гипертензии по данным Российского регистра. Евразийский кардиологический журнал. 2014;(4):44-53.
- Чазова И.Е., Архипова О.А., Валиева З.С., Наконечников С.Н., Мартынюк Т.В. Легочная гипертензия в России: первые результаты Национального Регистра. Терапевтический архив. 2014;86(9):56-64.
- Таран И.Н., Архипова О.А., Валиева З.С., Выборов О.Н., Мартынюк Т.В., Наконечников С.Н., Чазова И.Е. Гемодинамические и функциональные характеристики пациентов с различными формами легочной артериальной гипертензии согласно данным Российского регистра больных легочной гипертензией. В кн.: Кардиология, 2017: Лечить не болезнь, а больного. Сб. тезисов Всероссийской научно - практической конференции, 57-й ежегодной сессии Российского кардиологического научно - производственного комплекса. 2017;с. 24-5.
- Грацианская С.Е., Таран И.Н., Архипова О.А., Валиева З.С., Выборов О.Н., Мартынюк Т.В., Наконечников С.Н., Чазова И.Е. Особенности различных форм легочной артериальной гипертензии по данным Российского регистра. В кн.: Сб. тезисов V юбилейного евразийского конгресса кардиологов Евразийская ассоциация кардиологов. 2017;с. 89.
- Nakonechnikov S, Arkhipova O, Valieva Z, Martynyuk T, Chazova I. First date of Russian National Registry of Pulmonary Arterial Hypertension. J Hypertension. June 2015;33:e202.
- Юдкина Н.Н., Валеева Э.Г., Таран И.Н., Николаева Е.В., Парамонов В.М., Курмуков И.А., Валиева З.С., Архипова О.А., Мартынюк Т.В., Волков А.В., Насонов Е.Л., Чазова И.Е. Легочная артериальная гипертензия, ассоциированная с системной склеродермией, и идиопатическая легочная гипертензия: сравнительный анализ клинико - демографических особенностей и выживаемости по данным Российского национального регистра. Системные гипертензии. 2016;13(2):65-72.
- Легочная гипертензия. Под ред. И.Е. Чазовой и Т.В. Мартынюк. М.: Практика, 2015. 928 с.
- Opitz C.F, Rubin L.J. Acute vasodilator testing in idiopathic pulmonary arterial hypertension: must we take NO for the answer? European Respiratory J. 2009;33:1247-49. doi: 10.1183/09031936.00033809
- Ogawaa А, Ejirib К, Matsubara Н. Long - term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan. Life Sciences. 2014;118:414-9. doi: 10.1016/j.lfs.2014.01.077
Supplementary files
