Abstract
The aims of the study were: (1) to evaluate the Ukrainian reality of survival in patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH); and (2) to determine predictors of death. A total of 281 patients were enrolled (52 [18.5%] with CTEPH, 229 [81.5%] with PAH). Long-term survival (Kaplan-Meier) and its predictors (Stepwise binary logistic regression and Cox's proportional hazards analyses) were evaluated in adult patients with PH (diagnosed by right heart catheterization [RHC]) within a prospective registry at a single referral center in Kyiv, Ukraine. Follow-up period was up to 51 months. The Kaplan-Meier survival rate for the total cohort was 93.3%, 86.8%, and 81.5% at one, two, and three years, respectively. Survival was better in patients with congenital heart diseases (CHD) in comparison with idiopathic PAH (long rank P = 0.002), connective tissue diseases (CTD; long rank P = 0.001) and CTEPH (long rank P = 0.04). Univariate Cox's predictors of death were: functional class IV (odds ratio [OR] = 4.94; 95% confidence interval [CI] = 2.12-11.48), presence of ascites (OR = 4.52; 95% CI = 2.21-9.24), PAH-CTD (OR = 3.07; 95% CI = 1.07-8.87), PAH-CHD (OR = 0.28; 95% CI = 0.11-0.68), HR on treatment > 105 beats per min (OR = 7.85; 95% CI = 1.83-33.69), office systolic BP < 100 mmHg (OR = 2.78; 95% CI = 1.26-6.1), 6MWT on treatment < 340 m (OR = 3.47; 95% CI = 1.01-12.35), NT-proBNP > 300 pg/mL (OR = 4.98; 95% CI = 1.49-16.6), right atrium square > 22 cm(2) (OR = 14.2; 95% CI = 1.92-104.89), right ventricular square in diastole (OR = 1.08; 95% CI = 1.03-1.14), right ventricular square in systole (OR = 1.08; 95% CI = 1.02-1.11), mean pressure in right atrium per each 1-mmHg increase (OR = 1.02; 95% CI = 1.02-1.19). In multivariate Cox regression analyses only presence of ascites, office systolic BP < 100 mmHg, CHD etiology of PH, and NT-proBNP > 300 pg/mL were associated with survival.