Oxidative stress is regarded to play a crucial role in the pathophysiology of pulmonary arterial hypertension (PAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH). This study evaluated the prognostic value of serum oxidative stress markers (malondialdehyde (MDA), total antioxidant capacity (TAC), catalase activity (CAT), and superoxide activity (SOD)) in patients with PAH and CTEPH (). During 13 months of follow-up (median 9 months), clinical deterioration occurred in 14 patients (including 2 deaths). On the Cox regression analysis, MDA, TAC, and CAT were associated with clinical deterioration (,, 95% CI: 1.10-1.82; ,, 95% CI: 0.0032-0.33; and ,, 95% CI: 0.04-0.98, respectively). There was no significant difference in SOD (,, 95% CI: 0.87-1.08). The cut-off value derived from ROC curve analysis was 3.79 μM (,, 95% CI: 0.62-0.91) for MDA, 0.49 mM (,, 95% CI: 0.18-0.47) for TAC, and 1.34 U/L (,, 95% CI: 0.55-0.86) for CAT. MDA in the group with deterioration was higher (), while TAC as well as CAT were lower ( and , respectively) when compared to stable patients. Survival without clinical deterioration was significantly longer in patients with lower MDA (,, 95% CI: 0.12-1.14, log-rank), higher TAC (,, 95% CI: 0.06-0.60, log-rank), and higher CAT (, 95% CI: 0.11-0.88, log-rank). Markers of oxidative stress such as MDA, TAC, and CAT were associated with adverse clinical outcomes in patients with PAH and inoperable or residual CTEPH.

We aimed to evaluate the clinical course and impact of the SARS-CoV-2 pandemic on the rate of diagnosis and therapy in the complete Polish population of patients (pts) with pulmonary arterial hypertension (PAH-1134) and CTEPH (570 pts) treated within the National Health Fund program and reported in the national BNP-PL database. Updated records of 1704 BNP-PL pts collected between March and December 2020 were analyzed with regard to incidence, clinical course and mortality associated with COVID-19. Clinical characteristics of the infected pts and COVID-19 decedents were analyzed. The rates of new diagnoses and treatment intensification in this period were studied and collated to the proper intervals of the previous year. The incidence of COVID-19 was 3.8% (n = 65) (PAH, 4.1%; CTEPH, 3.2%). COVID-19-related mortality was 28% (18/65 pts). Those who died were substantially older and had a more advanced functional WHO class and more cardiovascular comorbidities (comorbidity score, 4.0 ± 2.1 vs. 2.7 ± 1.8; p = 0.01). During the pandemic, annualized new diagnoses of PH diminished by 25–30% as compared to 2019. A relevant increase in total mortality was also observed among the PH pts (9.7% vs. 5.9% pre-pandemic, p = 0.006), whereas escalation of specific PAH/CTEPH therapies occurred less frequently (14.7% vs. 21.6% pre-pandemic). The COVID-19 pandemic has affected the diagnosis and treatment of PH by decreasing the number of new diagnoses, escalating therapy and enhancing overall mortality. Pulmonary hypertension is a risk factor for worsened course of COVID-19 and elevated mortality.

Background: The pathophysiology of postoperative atrial fibrillation (POAF) is multifactorial. Inflammation and increased oxidative stress play a significant role in POAF development. Neopterin, a biomarker of cellular immune response that enhances oxidative stress and increases the cytotoxic potential of activated macrophages and dendritic cells, was recently found as an independent predictive biomarker of non-operative atrial fibrillation. However, as far as we know, neopterin has never been investigated in POAF. Aims: The study aimed to assess neopterin concentration as a prognostic biomarker of POAF following coronary artery bypass grafting (CABG). Methods: One hundred one patients (80.2% males, 85% off-pump, 15% on-pump) were included. Blood samples were taken from patients for analysis of serum neopterin and high-sensitive C-reactive protein (hs-CRP) at three time points: (1) before operation (NP0); (2) on the first day after operation (NP1); and (3) between the fifth and eighth day after the procedure (NP5-8). All factors (preoperative, echocardiographic, and surgical), significant in univariate analysis, were included in a multivariable logistic regression analysis. Results: POAF occurred in 30 patients (30%). In the analyzed multivariable logistic regression models, the independent predictors of POAF occurrence were: higher NP0 concentration (odds ratio [OR], 1.16; 95% confidence interval [CI], 1.02-1.38 for continuous and OR, 3.75; 95% CI, 1.39-10.1 for NP0 cut-off >8.7 nmol/l), higher body mass index (OR, 1.15; 95% CI 1.02-1.29), history of pulmonary disease (OR, 6.72; 95% CI 1.57-28), increased diastolic thickness of the interventricular septum (OR, 1.45; 95% CI, 1.14-1.83), and duration of operation (OR, 1.01; 95% CI, 1.03-1.36). Conclusions: We found that elevated neopterin concentration before CABG may be a predictive biomarker of POAF.

Background: Upregulation of the immune system is regarded to play an important role in the etiopathobiology of pulmonary arterial hypertension (PAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH). To the best of our knowledge, neopterin (NP) has never been investigated in patients with PAH and CTEPH. Objectives: The aim of the study was to evaluate the concentration of NP in blood in order to examine its impact on outcome and relationship with disease severity in that population. Methods: Serum concentration of NP was analysed prospectively in 50 patients (36 with PAH and 14 with CTEPH vs. 31 healthy controls) and assessed in relation to clinical parameters and outcome. Results: NP concentration in the PAH and CTEPH groups combined was significantly higher than in the control group (8.68, 6.39–15.03 vs. 5.14, 4.16–5.98 nmol/L, p < 0.0000001). During 9 months of follow-up, clinical deterioration occurred in 18 patients (including 8 deaths), and NP concentration in this group was higher when compared to stable patients (15.6, 8.52–25.13 vs. 7.87, 6.18–9.89, p = 0.002). The cutoff value of NP derived from ROC curve analysis was 15.3 nmol/L (p = 0.002, AUC 0.77, p = 0.0004, HR = 4.35, 95% CI 1.43–13.18, log-rank test). On Cox regression analysis, NP predicted clinical deterioration (p = 0.009, 95% CI 1.01–1.06). NP correlated positively with NT-proBNP (p < 0.001), red blood cell distribution width (p < 0.001), and right atrium area (p = 0.002) and inversely with 6-min walking test (p = 0.002) and peak oxygen consumption (p = 0.001). Conclusions: NP concentration is increased in patients with PAH and inoperable CTEPH. Elevated NP concentration is associated with adverse clinical outcomes and correlates with clinical parameters.

Pulmonary hypertension (PH) is a severe vascular complication of connective tissue diseases (CTD). Patients with CTD may develop PH belonging to diverse groups: (1) pulmonary arterial hypertension (PAH), (2) PH due to left heart disease, (3) secondary PH due to lung disease and/or hypoxia and (4) chronic thromboembolic pulmonary hypertension (CTEPH). PAH most often develops in systemic scleroderma (SSc), mostly in its limited variant. PAH-CTD is a progressive disease characterized by poor prognosis. Therefore, early diagnosis should be established. A specific treatment for PAH-CTD is currently available and recommended: prostacyclin derivative (treprostinil, epoprostenol, iloprost, selexipag), nitric oxide and natriuretic pathway: stimulators of soluble guanylate cyclase (sGC: riociguat) and phosphodiesterase-five inhibitors (PDE5i: sildenafil, tadalafil), endothelin receptor antagonists (ERA: bosentan, macitentan, ambrisentan). Moreover, novel drugs, e.g., sotatercept, have been intensively investigated in clinical trials. We aim to review the literature on recent advances in the treatment strategy and prognosis of patients with PAH-CTD. In this manuscript, we discuss the mechanism of action of PAH-specific drugs and new agents and the latest research conducted on PAH-CTD patients.