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.

The aim of the work is to compare vectorcardiographic (VCG) variables - spatial QRS-T angle and electrocardiographic ventricular gradient (VG) with echocardiography (EchoCG) data in patients with idiopathic pulmonary hypertension (IPH) and chronic thromboembolic pulmonary hypertension (CTEPH). Materials and methods. In 40 patients with IPH and 40 patients with CTEPH at the age of 45±12 years, systolic pulmonary artery pressure (SPAP); the sizes of heart chambers, parameters of RV systolic and diastolic function were evaluated with EchoCG. The QRS-T and VG angles were calculated on the VCG, derived from 12-lead digital ECG. Results. In all patients SPAP was greater than 40 mm Hg (mean 83±18 mm Hg), EchoCG data indicated hypertrophy and dilatation of RV, its systolic and diastolic function; dilatation of the right atrium (RA). Prognostically unfavorable changes in EchoCG were observed: the presence of pericardial effusion in 35 (44%) patients, RA area greater than 26 cm2 in 18 (23%) patients; TAPSE less than 1.5 cm in 37 (46%) patients. EchoCG and VCG variables had statistically significant differences in patients with III-IV functional class in comparison with I-II functional class. Statistically significant moderate correlations between VCG and EchoCG variables were revealed. VCG variables allowed to separate patient groups with the presence and absence of prognostically unfavorable changes in EchoCG with sensitivity from 54 to 78% and specificity from 66 to 87%. Conclusion. In patients with IPH and CTEPH, changes of QRS-T angle and VG correlate with SPAP, the size of RV and RA, parameters of RV systolic and diastolic function. The possibility of the use of QRS-T angle and VG for the detection of patients with prognostically unfavorable echocardiographic changes in the general group of patients with IPH and CTEPH has been shown.

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