Prognostic Significance of Systemic Arterial Stiffness Evaluated by Cardio-Ankle Vascular Index in Patients with Idiopathic Pulmonary Hypertension.

BACKGROUND: In a previous study, the cardio-ankle vascular index (CAVI) was increased significantly in idiopathic pulmonary arterial hypertension (IPAH) patients compared to the healthy group and did not much differ from one in systemic hypertensives. In this study the relations between survival and CAVI was evaluated in patients with IPAH. PATIENTS AND METHODS: We included 89 patients with new-diagnosed IPAH without concomitant diseases. Standard examinations, including right heart catheterization (RHC) and systemic arterial stiffness evaluation, were performed. All patients were divided according to CAVI value: the group with CAVI ≥ 8 (n = 18) and the group with CAVI < 8 (n = 71). The mean follow-up was 33.8 ± 23.7 months. Kaplan-Meier and Cox regression analysis were performed for the evaluation of our cohort survival and the predictors of death. RESULTS: The group with CAVI≥8 was older and more severe compared to the group with CAVI< 8. Patients with CAVI≥8 had significantly reduced end-diastolic (73.79±18.94 vs 87.35±16.69 mL, P<0.009) and end-systolic (25.71±9.56 vs 33.55±10.33 mL, P<0.01) volumes of the left ventricle, the higher right ventricle thickness (0.77±0.12 vs 0.62±0.20 mm, P < 0.006), and the lower TAPSE (13.38±2.15 vs 15.98±4.4 mm, P<0.018). RHC data did not differ significantly between groups, except the higher level of the right atrial pressure in patients with CAVI≥ 8-11.38±7.1 vs 8.76±4.7 mmHg, P<0.08. The estimated overall survival rate was 61.2%. The CAVI≥8 increased the risk of mortality 2.34 times (CI 1.04-5.28, P = 0.041). The estimated Kaplan-Meier survival in the patients with CAVI ≥ 8 was only 46.7 ± 7.18% compared to patients with CAVI < 8 - 65.6 ± 4.2%, P = 0.035. At multifactorial regression analysis, the CAVI reduced but saved its relevance as death predictor - OR = 1.13, CI 1.001-1.871. SUMMARY: We suggested the CAVI could be a new independent predictor of death in the IPAH population and could be used to better risk stratify this patient population if CAVI is validated as a marker in a larger multicenter trial.

Challenges and Special Aspects of Pulmonary Hypertension in Middle- to Low-Income Regions: JACC State-of-the-Art Review.

Challenges and special aspects related to the management and prognosis of pulmonary hypertension (PH) in middle- to low-income regions (MLIRs) range from late presentation to comorbidities, lack of resources and expertise, cost, and rare options of lung transplantation. Expert consensus recommendations addressing the specific challenges for prevention and therapy of PH in MLIRs with limited resources have been lacking. To date, 6 MLIR-PH registries containing mostly adult patients with PH exist. Importantly, the global prevalence of PH is much higher in MLIRs compared with high-income regions: group 2 PH (left heart disease), pulmonary arterial hypertension associated with unrepaired congenital heart disease, human immunodeficiency virus, or schistosomiasis are highly prevalent. This consensus statement provides selective, tailored modifications to the current PH guidelines to address the specific challenges faced in MLIRs, resulting in the first pragmatic and cost-effective consensus recommendations for PH care providers, patients, and their families.

Analysis of pulmonary hypertension patient survival after treatment in referral center (data of first Ukrainian register).

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 cm2 (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.

Influence of fixed-dose combination perindopril/amlodipine on target organ damage in patients with arterial hypertension with and without ischemic heart disease (results of EPHES trial).

BACKGROUND: The EPHES trial (Evaluation of influence of fixed dose combination Perindo-pril/amlodipine on target organ damage in patients with arterial HypErtension with or without iSchemic heart disease) compared the dynamics of target organ damage (TOD) in hypertensive patients with and without ischemic heart disease (IHD) treated with the fixed-dose combination (FDC) perindopril + amlodipine. METHODS: The analysis included 60 hypertensive patients (aged >30 years): 30 without IHD and 30 with IHD. At randomization, FDC was administered at a daily baseline dose of 5/5 mg with uptitration to 10/10 mg every two weeks. If target blood pressure (BP<140/90 mmHg) was not achieved after six weeks, indapamide 1.5 mg was added to the regimen. All patients underwent body mass index measurements, office and ambulatory BP measurements, pulse wave velocity (PWVe) and central systolic BP evaluation, augmentation index adjusted to heart rate 75 (Aix@75) evaluation, biochemical analysis, ECG, echocardiography with Doppler, ankle-brachial index measurement, and intima-media thickness measurement. The follow-up period was 12 months. RESULTS: Therapy based on FDC perindopril/amlodipine was effective in lowering BP (office, ambulatory, central) in both groups. We noted significant decrease in Aix@75 with the therapy in both groups, but ΔAix@75 was lesser in the group with IHD than the group without IHD. FDC provided significant improvement in PWVe and left ventricular diastolic function, and decrease in albuminuria, left ventricular hypertrophy (LVH), and left atrium size. ΔPWVe was significantly (P<0.005) less in patients without IHD than those with IHD (2.5±0.2 vs 4.4±0.5 m/s, respectively). In spite of almost equal LVH regression, the positive dynamics of ΔE/A and ΔE/E´ were more in patients with IHD than those without IHD (64.4% and 54.1% vs 39.8 and 23.2%, respectively; P<0.05 for both comparisons). Adverse reactions were in 2 (6.5%) patients without IHD and 3 (10%) with IHD (P=NS). In the group with IHD, we noted significant decrease in angina episode rate - from 2.5±0.4 to 1.2±0.2 (P<0.01) per week. CONCLUSION: Thus, treatment based on FDC was effective in decreasing BP and TOD regression in both patients with and without IHD. However, the dynamics of changes in TOD were different between the two groups, which should be taken into consideration during management of patients with and without IHD.