title: [Pulmonary hypertension in Russia: the first results of the national register].
abstract: OBJECTIVE
  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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.forename: I E
authors.initials: IE
authors.lastname: Chazova
authors.forename: O A
authors.initials: OA
authors.lastname: Arkhipova
authors.forename: Z S
authors.initials: ZS
authors.lastname: Valieva
authors.forename: S N
authors.initials: SN
authors.lastname: Nakonechnikov
authors.forename: T V
authors.initials: TV
authors.lastname: Martyniuk
mesh_terms: D015897:Comorbidity; D019468:Disease Management; D004452:Echocardiography / Q000379:methods; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D008297:Male; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011446:Prospective Studies; D011655:Pulmonary Embolism / Q000150:complications / Q000517:prevention & control; D012042:Registries; D012426:Russia / Q000453:epidemiology; D015996:Survival Rate
chemical_list: D065128:Endothelin Receptor Antagonists; D005343:Fibrinolytic Agents; D058986:Phosphodiesterase 5 Inhibitors