Pulmonary hypertension in Russia: The first results of the national register
- Authors: Chazova IE1, Arkhipova OA1, Valieva ZS1, Nakonechnikov SN1, Martyniuk TV1
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Affiliations:
- Институт клинической кардиологии им. А.Л. Мясникова ФГБУ "Российский кардиологический научно-производственный комплекс" Минздрава России, Москва
- Issue: Vol 86, No 9 (2014)
- Pages: 56-64
- Section: Editorial
- URL: https://journals.rcsi.science/0040-3660/article/view/31560
- ID: 31560
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Abstract
AIM. To investigate the demographic and clinical characteristics of patients with pulmonary hypertension (PH) and chronic thromboembolic pulmonary hypertension (CTEPH), regions of their residence, the specific features of diagnosis and drug therapy, and the patients' survival within the framework of a multicenter open-label prospective study. MATERIALS AND METHODS. The study enrolled patients over 18 years of age with PH classified as Group 1 (PH) and Group 4 (CTEPH). The follow-up was made in 9 expert centers of Russia on January 1 to February 28, 2014. The data of patients with PH and CTEPH were electronically entered at the register website: www.pul-hyp.medibase.ru. RESULTS. The study included 242 patients (183 women and 59 men) from 44 regions of the Russian Federation. PH of different etiologies was present in 79.3%, including 43.8% with idiopathic PH, 28.5% with congenital heart disease-associated PH, 6.2% with diffuse connective tissue-associated PH, 0.8% with hereditary PH; 20.7% were diagnosed with CTEPH. The patients' mean age at the inclusion in the register was 43.2±15.8 years; the median PH duration was 4.4 years (1.4 to 10.6%). The distance covered during a 6-minute walk test was 395.2±111.1 m; the Borg dyspnea index was 3.4±1.4 scores and the functional class was 2.5±0.8. After right cardiac catheterization, the mean pulmonary artery pressure was 53.1±19.5 mm Hg; cardiac output, 3.8±1.2 l/min; cardiac index, 2.1±0.6 l/min/m2; pulmonary vascular resistance, 1105±677.6 dyn·sec/cm5. A positive acute pharmacological test was seen in 16.3% of the patients. The most common comorbidity was erosive ulcerative lesion of the gastrointestinal tract (23.5%), hypertension (16.9%), and obesity (16.5%). 66% of the patients received PH-specific therapy: sildenafil (51.6%), bosentan (20.9%), or iloprost (7%); 7.4% of the patients were observed within the framework of clinical trials, 17.7% received combined therapy for PH: 16.3 and 1.4% had dual- or triple-component therapy, respectively; 55.8% of the patients took calcium antagonists, 61.4% had diuretics, 85.6% had antithrombotic drugs. The survival rates were 98% in the first year of the follow-up and 90.1% by the end of 2013. CONCLUSION. The formation of a national register of patients with PH and CTEPH makes it possible to introduce current approaches to diagnosing and treating the patients of this category and to evaluate the efficiency and quality of rendered medical care and will contribute to the estimation of required health care volumes and the expenditure of public health resources.
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##article.viewOnOriginalSite##About the authors
I E Chazova
Институт клинической кардиологии им. А.Л. Мясникова ФГБУ "Российский кардиологический научно-производственный комплекс" Минздрава России, Москва
O A Arkhipova
Институт клинической кардиологии им. А.Л. Мясникова ФГБУ "Российский кардиологический научно-производственный комплекс" Минздрава России, Москва
Email: olga_ark@list.ru
121552 Москва, 3-я Черепковская, д. 15а
Z S Valieva
Институт клинической кардиологии им. А.Л. Мясникова ФГБУ "Российский кардиологический научно-производственный комплекс" Минздрава России, Москва
S N Nakonechnikov
Институт клинической кардиологии им. А.Л. Мясникова ФГБУ "Российский кардиологический научно-производственный комплекс" Минздрава России, Москва
T V Martyniuk
Институт клинической кардиологии им. А.Л. Мясникова ФГБУ "Российский кардиологический научно-производственный комплекс" Минздрава России, Москва
References
- Диагностика и лечение легочной гипертензии. Российские рекомендации. Кардиоваск тер и проф 2007; 6: Приложение 2.
- Simonneau G., Gatzoulis M.A., Ian Adatia I. et al. Updated Clinical Classification of Pulmonary Hypertension. J Am Coll of Cardiol 2013; 62: Suppl: 34-41.
- Guidelines for the diagnosis and treatment of pulmonary hypertension. The task force for the diagnosis and treatment of pulmonary hypertension of the ECS and ERS, endorsed by the ISHLT. Eur Heart J 2009; 30: 2493-2537.
- Effective HealthCare, DEcIDE: Registries for Evaluating Patient Outcomes. Agency for Healthcare Research and Quality 2006. http://effectivehealthcare.ahrq.gov/decide/registryOutline.cfm.
- 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: 1023-1030.
- 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): 1285-1293.
- D´Alonzo G.E., Barst R.J., Ayres S.M. et al. Survival in patients with primary pulmonary hypertension: results from a national prospective registry. Ann Intern Med 1991; 115: 343-349.
- 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; 186 (8): 790-796.
- Badesch D.B., Raskob G.E., Elliott C.G. et al. Pulmonary Arterial Hypertension: Baseline Characteristics From the REVEAL Registry. Chest 2010; 137: 376-387.
- Shapiro S., Traiger G. L., Turner M. et al. 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): 363-373.
- Демографический ежегодник России. 2010: Стат. сб. Росстат. M 2010; 525.
- 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.
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