Features of pulmonary arterial hypertension in the Russian register

The aim of the multicenter prospective open study: to assess demographic and clinical characteristics of PAH patients, regions of their accommodation, features of diagnostic and medical strategy, and survival. Materials and methods. In the study we included PAH patients (group 1) aged >18 years observed in 10 expert centers of the Russian Federation from 01.01.2012 to 30.04.2014. Patients'' data were brought in the electronic forms on the registry site www.pul-hyp.medibase.ru. Results. 198 patients (160 women/38 men) from 44 regions of the country were included in the study: 55.0% - IPAH, 35.9% - PAH associated with congenital heart disease, 7,6% - PAH associated with connective tissue disease, 1.0% - heritable PAH, 0.5% - PAH associated with portal hypertension. At the time of including in the register the patients'' age median was 38.1 (28.753.8) years, the disease duration median was 5.9 (2.3-13.7) years. The 6MW distance was 394.84 ± 111.10 meters, dyspnea Borg index - 3.34 ± 1.41 points. 9.2% had FC I, 43.7% of FC II, 36.2% of FC III, 10.9% of FC IV. According to RHC mean PAP was 55 (43.25-64.0) mm Hg, CV 3.5 (2.9-4.33) l/min, CI 2.0 (1.82.3) l/min/m 2, PVR 1079 (762-1424.8) dinхс/см 5. Positive acute vasodilator testing was found in 25% of patients. The spectrum of the most frequent associated diseases included gastrointestinal diseases (27%), arterial hypertension (14%), urolithiasis (9%) were. PAH-specific therapy was prescribed to 68% of patients (sildenafil - 75.4%, bosentan - 40.4%, iloprost - 9.5%). 9.6% of patients were observed in clinical trials. PAH monotherapy was prescribed to 66.7% of pts, the combined two-component therapy was recommended to 31.7% of pts, triple therapy- to 1.6% of pts. 51% of pts were treated with calcium channel blockers, 59% - diuretics, 82% - antitrombotic drugs. In PAH group the 1-y survival was 98%, the 2-y survival - 96%. Conclusion. Harmoniously built system network of expert centers will allow to improve early diagnostic of PAH, providing proper observation and lowering mortality rate.