Легенева гіпертензія: стан проблеми та аналіз роботи референтного центру (дані першого українського реєстру)

The aim – to analyze the structure of patients who were treated in specialized referential center; to evaluate Ukrainian reality of survival of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) who were treated at the referential center and to determine the predictors of death. Materials and methods. Data of 359 patients with pulmonary hypertension were included in the study. To assess survival, 281 patients (52 (18.5 %) with CTEPH and 229 (81.5 %)) with PAH were examined who were treated at the center of pulmonary hypertension of M.D. Strazhesko Institute of Cardiology of NAMS of Ukraine. The diagnosis of pulmonary hypertension was based on the data of the catheterization of the right heart. Survival was determined by the method of constructing the Kaplan – Meier curves. The observation period was 51 months. Predictors were determined using binary logistic regression and Cox regression analysis. Results and discussion. The survival rate of the overall patient cohort was 93.3 %, 86.8 % and 81.5 % at stages one, two, and three, respectively. The best survival rate was in patients with PAH associated with congenital heart diseases – CHD (92.7 %) compared to patients with idiopathic PAH (67.5 %, long rank р=0.002), PAH associated with connective tissue diseases (49.7 %, long rank р=0.001) and CTEPH (83.2 %, long rank р=0.04). According to the univariate Cox analysis, the predictors of death were: functional class IV according to the WHO (OR=4.94, 95 % CI 2.12–11.48), presence of ascites (OR=4.52, 95 % CI 2.21–9.24), PAH associated with connective tissue disease (OR=3.07, 95 % CI 1.07–8.87), PAH associated with CHD (OR=0.28, 95 % CI 0.11–0.68), heart rate at the background of treatment > 105 beats per minute (OR=7.85, 95 % CI 1.83–33.69), office systolic blood pressure 300 pg/ml (OR=4.98, 95 % CI 1.49–16.6), right atrial area > 22 cm2 (OR=14.2, 95 % CI 1.92–104.89), right ventricular area in diastole (OR=1.08, 95 % CI 1.03–1.14), right ventricular area in systole (OR=1.08, 95 % CI 1.02–1.11), 1 mm Hg increase of mean pressure in the right atrium (OR=1.02, 95 % CI 1.02–1.19). In multivariate Cox regression analysis, independent predictors of death were ascites, office systolic blood pressure 300 pg/ml. PAH associated with CHD reduced the likelihood of death. Conclusions. These are the first results of the study that was conducted at the only Ukrainian center for the diagnosis and treatment of pulmonary hypertension. They show that the structure of patients with PAH in Ukraine is significantly different from that in centers of other countries, though the survival rates are comparable. Quite simple indicators are found which can be evaluated in routine practice and which are independent predictors of death.