Anxiety and Depression in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension after the Removal of COVID-19 Pandemic Restrictions.

Background: There was increased risk of mental disturbances during the COVID-19 pandemic. Patients with chronic diseases, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), were particularly vulnerable. Our previous study showed high levels of fear of COVID-19 (FCV-19S), anxiety (HADS-A), and depression (HADS-D) in the second year of the pandemic among PAH/CTEPH patients. The aim of the present study was to assess changes in the levels of FCV-19S, HADS-A, and HADS-D after removing restrictions related to the COVID-19 pandemic. Methods: In this prospective, single-center study, 141 patients (62% females, 64% PAH) with a median age of 60 (range 42-72) years were included. Patients completed appropriate surveys in the second year of the pandemic, and then, after the restrictions were lifted in Poland (after 28 March 2022). Results: FVC-19S decreased significantly from 18 (12-23) to 14 (9-21), p < 0.001. The levels of anxiety (HADS-A ≥ 8 points) and depression (HADS-D ≥ 8 points) were abnormal in 26% and 16% of patients, respectively; these did not change at follow-up (p = 0.34 for HADS-A and p = 0.39 for HADS-D). Conclusions: Among PAH/CTEPH patients, fear of COVID-19 decreased significantly after the COVID-19 pandemic restrictions were removed, but anxiety and depression remained high, indicating that the COVID-19 pandemic was not a major factor in causing these disorders.

Safety and Outcomes of Inferior Vena Cava Filter Placement in Oncology Patients: A Single-Centre Experience.

The risk of venous thromboembolism (VTE) in the oncology population is significantly higher than in non-cancer patients. Inferior vena cava (IVC) filters may, therefore, be an important part of VTE treatment. In this study, we address the outcomes of placing IVC filters in the oncology population. This single-centre, observational, retrospective study included 62 patients with active malignancy and acute VTE who underwent an IVC filter implantation due to contraindications to anticoagulation during the period 2012-2023. The control group consisted of 117 trauma patients. In both groups, an urgent surgical procedure requiring temporary cessation of anticoagulation was the most noted reason for IVC filter placement-76% in the oncology group vs. 100% in the non-oncology group (p < 0.001). No complications were reported during the IVC filter implantation procedures. There was no recurrence of pulmonary embolism or deep venous thrombosis in the oncology group after filter implantation. The rate of successful filter explantation, median time to retrieval, and abnormal findings during retrieval were not significantly different between both subgroups (64.3% vs. 76.5%, p = 0.334; 77 days vs. 84 days, p = 0.764; 61.5% vs. 54.2%, p = 0.672; respectively). The study showed that IVC filter placement is a safe and effective method of preventing PE in cancer patients with contraindications to anticoagulation. The complication rate following IVC filter implantation in cancer patients is low and similar to that in non-oncology patients.

The Utility of a Resting Electrocardiogram (ECG-PH Index) in Evaluating the Efficacy of Pulmonary Endarterectomy in Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: The ECG-PH index (PH-ECG score) has been proposed as a valuable ECG-derived method of evaluating the effectiveness of balloon pulmonary angioplasty (BPA) in chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary endarterectomy (PEA) is the main form of therapy for CTEPH with a proximal clot location. The objective of this study was to assess the clinical utility of a resting electrocardiogram (ECG-PH index) in assessing the effectiveness of PEA in CTEPH patients. METHODS: The retrospective analysis included 73 patients who underwent PEA. Their ECG-PH index values were calculated using four ECG parameters: R-wave amplitude V1 + S-wave amplitude V5/V6 > 10.5 mm, QRS-wave axis > 110 degrees, R-wave amplitude V1 > S-wave amplitude V1, and SIQIII pattern. PH-ECG scores were assessed after a median time of 13 months (IQR: 8-31 months) had passed since the PEA procedures. RESULTS: The current analysis documented that ECG-PH index = 0 is a good reflection of mPAP < 25mmHg (sensitivity 76.1%; specificity 66.7%; positive predictive value 79.5%; negative predictive value 62.1%) or mPAP ≤ 20 mmHg (sensitivity 69.6%; specificity 70.6%; positive predictive value 88.6%; negative predictive value 41.4%) after PEA. The values of the area under the ROC curve for ECG-PH index were 0.772 (95% CI: 0.676-0.867) and 0.743 (95% CI: 0.637-0.849) for the mPAP < 25 mmHg and mPAP ≤ 20 mmHg patient groups, respectively. CONCLUSION: The ECG-PH index may be useful for monitoring the haemodynamic effect of PEA in CTEPH patients.

Emerging Role of Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Disease-Insights from the 2022 ESC Guidelines.

In this article, we discuss the topic of chronic thromboembolic pulmonary disease (CTEPD) and the growing role of balloon pulmonary angioplasty (BPA) in its treatment. We present the pathophysiology of CTEPD which arises from an incomplete resolution of thrombi in the pulmonary arteries and leads to stenosis and occlusion of the vessels. The article focuses mainly on the chronic thromboembolic pulmonary hypertension (CTEPH) subpopulation for which prognosis is very poor when left untreated. We describe a multimodal approach to treating CTEPH, including pulmonary endarterectomy (PEA), BPA, and pharmacological therapies. Additionally, the benefits of pharmacological pre-treatment before BPA and the technical aspects of the procedure itself are outlined. It is emphasized that BPA does not replace PEA but serves as a complementary treatment option for eligible patients. We summarized efficacy and treatment goals including an improvement in functional and biochemical parameters before and after BPA. Patients who received pre-treatment with riociguat prior to BPA exhibited a notable reduction in the occurrence of less severe complications. However, elderly patients are still perceived as an especially vulnerable group. It is shown that the prognosis of patients undergoing BPA is similar to PEA in the first years after the procedure but the long-term prognosis of BPA still remains unclear. The 2022 ESC/ERS guidelines highlight the significant role of BPA in the multimodal treatment of CTEPH, emphasizing its effectiveness and recommending its consideration as a therapeutic option for patients with CTEPD, both with and without pulmonary hypertension. This review summarizes the available evidence for BPA, patient selection, procedural details, and prognosis and discusses the potential future role of BPA in the management of CTEPH.

Long COVID syndrome after SARS-CoV-2 survival in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients have a more severe COVID-19 course than the general population. Many patients report different persistent symptoms after SARS-CoV-2 infection. The aim of our study is to analyze the prevalence of long COVID-19 symptoms and assess if COVID-19 affects pulmonary hypertension (PH) prognosis. PAH/CTEPH patients who survived COVID-19 for at least 3 months before visiting the PH centers were included in the study. The patients were assessed for symptoms in acute phase of SARS-CoV-2 infection and persisting in follow-up visit, WHO functional class, 6-min walk distance, NT-proBNP concentration. The COMPERA 2.0 model was used to calculate 1-year risk of death due to PH at baseline and at follow-up. Sixty-nine patients-54 (77.3%) with PAH and 15 (21.7%) with CTEPH, 68% women, with a median age of 47.5 years (IQR 37-68)-were enrolled in the study. About 17.1% of patients were hospitalized due to COVID-19 but none in an ICU. At follow-up (median: 155 days after onset of SARS-CoV-2 symptoms), 62% of patients reported at least 1 COVID-19-related symptom and 20% at least 5 symptoms. The most frequently reported symptoms were: fatigue (30%), joint pain (23%), muscle pain (17%), nasal congestion (17%), anosmia (13%), insomnia (13%), and dyspnea (12%). Seventy-two percent of PH patients had a low or intermediate-low risk of 1-year death due to PH at baseline, and 68% after COVID-19 at follow-up. Over 60% of PAH/CTEPH patients who survived COVID-19 suffered from long COVID-19 syndrome, but the calculated 1-year risk of death due to PH did not change significantly after surviving mild or moderate COVID-19.

Outcomes of COVID-19 in patients vaccinated and unvaccinated against SARS-CoV-2 and suffering from pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.

INTRODUCTION: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) lead to progressive right heart failure. The mortality rates in PAH and CTEPH patients due to COVID­19 are high, and vaccination against COVID­19 is recommended in this group. OBJECTIVES: We analyzed the incidence and outcomes of COVID­19in the PAH/CTEPH patients for 2 years of the pandemic, as well as the predictors of worse outcomes of COVID­19 in this group. PATIENTS AND METHODS: PAH/CTEPH patient data for this observational, cohort study were obtained from 3 pulmonary hypertension centers between March 11, 2020 and March 11, 2022. RESULTS: A total of 364 consecutive patients with PAH/CTEPH (248/122; 232 women [64%]; median [interquartile range] age, 61 years [18-92]) were included in the study. All the patients had advanced pulmonary hypertension at baseline. Eighty­five patients (23%) suffered from COVID­19. Seven of them (8%), all of whom were unvaccinated, died of COVID­19. The unvaccinated patients suffered from COVID­19 more often than the vaccinated ones (46% vs 9%; P <0.001). As many as 31% of the PAH/CTEPH patients with COVID­19 needed hospitalization, in 8% of cases in the intensive care unit. Age equal to or above 65 years and severe pulmonary hypertension defined as a World Health Organization functional class 3 or 4 were associated with severe COVID­19 in the PAH/CTEPH patients. CONCLUSIONS: The vaccinated PAH/CTEPH patients suffered from COVID­19 less frequently than the unvaccinated ones. The mortality rate and hospitalization due to COVID­19 were higher in the PAH/CTEPH patients than in the general population. All efforts should be made to convince the PAH/CTEPH patients to vaccinate against COVID­19.

Effect of Statins on All-Cause Mortality in Adults: A Systematic Review and Meta-Analysis of Propensity Score-Matched Studies.

Statins are lipid-lowering medications used for the prevention of cardiovascular disease (CVD), but the pleiotropic effects of statins might be beneficial in other chronic diseases. This meta-analysis investigated the association between statin use and mortality in different chronic conditions. Eligible studies were real-world studies that compared all-cause mortality over at least 12 months between propensity score-matched statin users and non-users. Overall, 54 studies were included: 21 in CVD, 6 in chronic kidney disease, 6 in chronic inflammatory diseases, 3 in cancer, and 18 in other diseases. The risk of all-cause mortality was significantly reduced in statin users (hazard ratio: 0.72, 95% confidence interval: 0.66−0.76). The reduction in mortality risk was similar in CVD studies (0.73, 0.66−0.76) and non-CVD studies (0.70, 0.67−0.79). There were no significant differences in the risk reduction between cohorts with different diseases (p = 0.179). The greatest mortality reduction was seen in studies from Asia (0.61, 0.61−0.73) and the lowest in studies from North America (0.78, 0.73−0.83) and Australia (0.78, 0.62−0.97). There was a significant heterogeneity (I2 = 95%, tau2 = 0.029, p < 0.01). In conclusion, statin use was associated with a significantly reduced risk of all-cause mortality in real-world cohorts with CVD and non-CVD.

Physical Activity in Pulmonary Arterial Hypertension during Pandemic COVID-19 and the Potential Impact of Mental Factors.

One of the non-pharmacological recommendations for stable patients with pulmonary arterial hypertension (PAH) is to increase physical activity. The study aimed to analyze the degree of physical activity of PAH patients and check if mental factors may have a potential negative impact during the COVID-19 pandemic. Forty patients with stable PAH were included in the study. Physical activity was assessed by pedometer (Omron HJ-321-E) for four weeks. At baseline, in addition to the 6 min walk test (6MWT) and functional assessment, patients completed the quality-of-life questionnaire SF-36, fear of COVID-19 scale, and hospital anxiety and depression scale (HADS). The mean age of the study group was 45.5 years, 80% were women, and 62.5% had idiopathic/heritable PAH. Low physical activity defined as <5000 steps/day had 19 (47.5%), and moderate/high physical activity (≥5000 steps/day) had 21 (52.5%) patients. Patients with low physical activity less frequently worked compared with the moderate−high-activity sub-group, 42% vs. 81%, p = 0.03, and had the shorter distance in 6-6MWT, p = 0.03. There was no significant correlation between steps/day and different mental factors. Almost half of the study group had low activity during the pandemic. Mental factors did not impact physical activity in PAH patients during the pandemic.

Impact of the COVID-19 Pandemic on Pulmonary Hypertension Patients: Insights from the BNP-PL National Database.

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.

Characteristics and Outcomes of Patients Consulted by a Multidisciplinary Pulmonary Embolism Response Team: 5-Year Experience.

(1) Background: Pulmonary embolism (PE) is the third most frequent acute cardiovascular condition worldwide. PE response teams (PERTs) have been created to facilitate treatment implementation in PE patients. Here, we report on the 5-year experience of PERT operating in Warsaw, Poland, with regard to the characteristics and outcomes of the consulted patients. (2) Methods: Patients diagnosed with PE between September 2017 and December 2021 were included in the study. Clinical and treatment data were obtained from medical records. Patient outcomes were assessed in-hospital, at a 1- and 12-month follow-up. (3) Results: There were 235 PERT activations. The risk of early mortality was low in 51 patients (21.8%), intermediate-low in 83 (35.3%), intermediate-high in 80 (34.0%) and high in 21 (8.9%) patients. Anticoagulation alone was the most frequently administered treatment in all patient subgroups (altogether 84.7%). Systemic thrombolysis (47.6%) and interventional therapy (52%) were the prevailing treatment options in high-risk patients. The in-hospital mortality was 6.4%. The adverse events during 1-year follow-up included five deaths, two recurrent VTE and two minor bleeding events. (4) Conclusions: Our initial 5-year experience showed that the activity of the local PERT facilitated patient-tailored decision making and the access to advanced therapies, with subsequent low overall mortality and treatment complication rates, confirming the benefits of PERT implementation.

An unusual case of CTEPH treated by BPA in a patient with a single lung after cancer surgery.

A 46-year-old man 1 year after left-sided pneumonectomy for squamous cell lung cancer presented with severely limited exercise tolerance and dyspnea corresponding to World Health Organization functional class IV (WHO Class IV). After right heart catheterization (RHC), mean pulmonary artery pressure (mPAP) was 43 mmHg and pulmonary vascular resistance (PVR) was 10.2 Wood units (WU). Arteriography revealed organized clots located at the proximal level of the right pulmonary artery, leading to a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). The CTEPH team disqualified the patient from surgical treatment due to high perioperative risk and referred him for balloon pulmonary angioplasty (BPA) together with pulmonary hypertension-specific pharmacotherapy (sildenafil). The patient underwent a cycle of nine BPA sessions and completed treatment without complications. Follow-up showed sustained hemodynamic improvement in RHC (mPAP 23 mmHg, PVR 2.6 WU), improved physical capacity (WHO Class II), and relief of dyspnea symptoms.

Circulating Blood-Based Biomarkers in Pulmonary Hypertension.

Pulmonary hypertension (PH) is a serious hemodynamic condition, characterized by increased pulmonary vascular resistance (PVR), leading to right heart failure (HF) and death when not properly treated. The prognosis of PH depends on etiology, hemodynamic and biochemical parameters, as well as on response to specific treatment. Biomarkers appear to be useful noninvasive tools, providing information about the disease severity, treatment response, and prognosis. However, given the complexity of PH, it is impossible for a single biomarker to be adequate for the broad assessment of patients with different types of PH. The search for novel emerging biomarkers is still ongoing, resulting in a few potential biomarkers mirroring numerous pathophysiological courses. In this review, markers related to HF, myocardial remodeling, inflammation, hypoxia and tissue damage, and endothelial and pulmonary smooth muscle cell dysfunction are discussed in terms of diagnosis and prognosis. Extracellular vesicles and other markers with complex backgrounds are also reviewed. In conclusion, although many promising biomarkers have been identified and studied in recent years, there are still insufficient data on the application of multimarker strategies for monitoring and risk stratification in PH patients.

COVID-19 Vaccination in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: Safety Profile and Reasons for Opting against Vaccination.

The incidence of COVID-19 infection in patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) is similar to that in the general population, but the mortality rate is much higher. COVID-19 vaccination is strongly recommended for PAH/CTEPH patients. The aim of our cross-sectional study was to identify reasons why PAH/CTEPH patients refused vaccination against COVID-19. Moreover, we assessed the safety profile of approved COVID-19 vaccines in PAH/CTEPH patients. We examined 261 patients (164 PAH patients and 97CTEPH patients) with a median age of 60 (18-92) years, 62% of which were female. Sixty-one patients (23%) refused to be vaccinated. The main reason for unwillingness to be vaccinated was anxiety about adverse events (AEs, 61%). Age and fear of COVID-19 in the univariate analysis and age ≥60 years in the multivariate regression analysis were factors that impacted willingness to be vaccinated (OR = 2.5; p = 0.005). AEs were reported in 61% of vaccinated patients after the first dose and in 40.5% after the second dose (p = 0.01). The most common reported AEs were pain at the injection site (54.5%), fever (22%), fatigue (21%), myalgia (10.5%), and headache (10%). A lower percentage of AEs was reported in older patients (OR = 0.3; p = 0.001). The COVID-19 vaccines are safe for PAH/CTEPH patients. The results obtained in this study may encourage patients of these rare but severe cardio-pulmonary diseases to get vaccinated against COVID-19.

Predictive value of chest HRCT for survival in idiopathic pulmonary arterial hypertension.

BACKGROUND: Little attention has been paid to chest high resolution computed tomography (HRCT) findings in idiopathic pulmonary arterial hypertension (IPAH) patients so far, while a couple of small studies suggested that presence of centrilobular ground-glass opacifications (GGO) on lung scans could have a significant negative prognostic value. Therefore, the aims of the present study were: to assess frequency and clinical significance of GGO in IPAH, and to verify if it carries an add-on prognostic value in reference to multidimensional risk assessment tool recommended by the 2015 European pulmonary hypertension guidelines. METHODS: Chest HRCT scans of 110 IPAH patients were retrospectively analysed. Patients were divided into three groups: with panlobular (p)GGO, centrilobular (c)GGO, and normal lung pattern. Association of different GGO patterns with demographic, functional, haemodynamic, and biochemical parameters was tested. Survival analysis was also performed. RESULTS: GGO were found in 46% of the IPAH patients: pGGO in 24% and cGGO in 22%. Independent predictors of pGGO were: positive history of haemoptysis, higher number of low-risk factors, and lower cardiac output. Independent predictors of cGGO were: positive history of haemoptysis, younger age, higher right atrial pressure, and higher mixed venous blood oxygen saturation. CGGO had a negative prognostic value for outcome in a 2-year perspective. This effect was not seen in the longer term, probably due to short survival of cGGO patients. CONCLUSIONS: Lung HRCT carries a significant independent prognostic information in IPAH, and in patients with cGGO present on the scans an early referral to lung transplantation centres should be considered.

Assessment of Clinical Usefulness of Resting Electrocardiogram (PH-ECG Score) in Monitoring the Efficacy of Balloon Pulmonary Angioplasty (BPA) in Patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH).

BACKGROUND: Balloon pulmonary angioplasty (BPA) is a form of therapy for chronic thromboembolic pulmonary hypertension (CTEPH). The study objective is to assess the clinical usefulness of resting ECG (PH-ECG score) in monitoring the efficacy of BPA in CTEPH patients. METHODS AND RESULTS: Ninety-four (n = 94) CTEPH patients were included in the analysis. A standard 12-lead-ECG was performed before the first BPA session and after completion of treatment. The whole analysed population (n = 94) was divided into the following two groups: derivation cohort (n = 41) and validation cohort (n = 53). The derivation cohort was divided into the following two subgroups: patients with mean pulmonary artery pressure (mPAP) after the completion of therapy < 25 mmHg (n = 21) and patients with mPAP after the completion of therapy ≥ 25 mmHg (n = 20). In the first subgroup, four (R-wave V1 + S-wave V5/V6 > 10.5 mm, QRS-wave axis > 110 degrees, R-wave V1 > S-wave V1, SIQIII pattern) of the six ECG parameters of overload of the right cardiac chambers showed statistically significant differences (p < 0.005). That was followed by a determination of the sensitivity and specificity, positive (PPV) and negative predictive value (NPV), and ROC curve (AUC 0.9; 95% CI: 0.792-1.000) for the variable that was a sum of the above four ECG parameters (PH-ECG score). The absence of all of the four ECG parameters at rest (PH-ECG score = 0) well reflected patients with mPAP < 25 mmHg (sensitivity, 100%; specificity, 80%; PPV, 84%; NPV, 100%). In the validation cohort with mPAP < 25 mmHg and PH-ECG score = 0, sensitivity, specificity, PPV, and NPV were 86%, 77%, 73%, and 89%, respectively. CONCLUSIONS: Resting ECG trace is clinically useful in the monitoring of therapeutical effects of BPA in CTEPH patients.

Fear of COVID-19, Anxiety and Depression in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension during the Pandemic.

The COVID-19 pandemic has affected the physical and mental health of people around the world. This may be particularly true for patients with life-threatening diseases. We analyzed the level of fear of COVID-19 (FCV-19S), the prevalence of anxiety (HADS-A) and depression (HADS-D) in pulmonary arterial and chronic thromboembolic pulmonary hypertension (PAH and CTEPH) patients during the COVID-19 pandemic. In this multicenter prospective study, 223 patients (63% females, 66% PAH) with age range 18-90 years were included. The fear of COVID-19 was high, at a mean level of 18.9 ± 7.4 points. Anxiety (HADS-A ≥ 8 points) was diagnosed in 32% of all patients, depression (HADS-D ≥ 8 points) in 21%, and anxiety or depression in 38%. FCV-19S was higher in woman and in elderly people (p = 0.02; p = 0.02, respectively). In the multivariate analysis, FCV-19S higher than the median increased the odds ratio of anxiety, but not of depression (R 6.4 (95%CI 2.0-20.0), p = 0.002; OR 1.9 (0.9-3.9), p = 0.06, respectively). History of COVID-19 increased risk of both HADS-A and HADS-D. Patients with PAH and CTEPH, especially woman over 65 years and those who had been infected with COVID-19, may need additional psychological support due to fear of COVID-19, anxiety or depression.

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease.

Systemic connective tissue diseases (CTDs) comprise a large group of diseases that are auto-immune in nature and characterized by the involvement of multiple systems and organs. Pul-monary hypertension (PH) of various etiologies may develop in the course of CTD, including pulmonary arterial hypertension (PAH), PH secondary to the lung disease, postcapillary PH in the course of left heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH). In addition, the different forms of PH may coexist with each other. Among patients with CTD, PAH occurs most commonly in those with systemic sclerosis, where it affects ap-proximately 8%-12% of patients. The prognosis in patients with untreated PAH is very poor. It is particularly important to identify the high-risk CTD-PAH population and to perform effi-cient and accurate diagnostics so that targeted therapy of the pulmonary arteries can be intro-duced. Echocardiography is used to screen for PH, but clinical and echocardiographic suspicion of PH always requires confirmation by right heart catheterization. Confirmation of PAH ena-bles the initiation of life-prolonging pharmacological treatment in this group of patients, which should be administered in referral centers. Drugs available for pharmacological management include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins.

Soluble ST2 as a Biomarker for Early Complications in Patients with Chronic Thromboembolic Pulmonary Hypertension Treated with Balloon Pulmonary Angioplasty.

BACKGROUND: The aim of the study was to assess soluble ST2 (sST2) concentration and its dynamic changes in the periprocedural period in patients with chronic thromboembolic pulmonary hypertension (CTEPH) treated with balloon pulmonary angioplasty (BPA). METHODS: We prospectively analyzed 57 procedures of BPA performed in 37 patients with CTEPH. Biomarkers, such as N-terminal pro B-type natriuretic peptide (NT-proBNP), troponin T (TnT), and sST2 were assessed at four time points: Before the BPA procedure, 24 h and 48 h after the procedure, and at the discharge from hospital. Each postprocedural period was assessed for complications. RESULTS: Before the BPA procedure, median sST2 concentration was 26.56 ng/mL (IQR: 16.66-40.83 ng/mL). sST2 concentration was significantly higher 24 h and 48 h after the BPA compared to the baseline measurements (33.31 ng/mL (IQR: 20.81-62.56), p = 0.000 and 27.45 ng/mL (IQR: 17.66-54.45), p = 0.028, respectively). sST2 level 24 h after the BPA procedure was significantly higher in the group with complications compared to the group without complications in the postprocedural period (97.66 ng/mL (IQR: 53.07-126.18) vs. 26.86 ng/mL (IQR: 19.10-40.12), p = 0.000). CONCLUSIONS: sST2 concentration in patients with CTEPH treated with BPA changes significantly in the postprocedural period and is significantly higher in the group with complications in postprocedural period.

Prediction of Prognostic Hemodynamic Indices in Pulmonary Hypertension Using Non-Invasive Parameters.

Effective targeted therapy of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) requires regular risk stratification. Among many prognostic parameters, three hemodynamic indices: right atrial pressure, cardiac index, and mixed venous saturation are considered critically important for correct risk classification. All of them are measured invasively and require right heart catheterization (RHC). The study was aimed to verify assumption that a model based on non-invasive parameters is able to predict hemodynamic profile described by the mentioned invasive indices. A group of 330 patients with pulmonary hypertension was used for the selection of the best predictors from the set of 17 functional, biochemical, and echocardiographic parameters. Multivariable logistic regression models for the prediction of low-risk and high-risk profiles were created. The cut-off points were determined and subsequent validation of the models was conducted prospectively on another group of 136 patients. The ROC curve analysis showed the very good discrimination power of the models (AUC 0.80-0.99) in the prediction of the hemodynamic profile in the total validation group and subgroups: PAH and CTEPH. The models indicated the risk profiles with moderate sensitivity (57-60%) and high specificity (87-93%). The method enables estimation of the hemodynamic indices when RHC cannot be performed.

Assessment of electrocardiographic markers of acute and long-term hemodynamic improvement in patients with pulmonary hypertension.

BACKGROUND: The remodeling of the right heart in patients with chronic pulmonary hypertension (cPH) is associated with the appearance of electrocardiographic (ECG) abnormalities. We investigated the resolution of ECG markers of right ventricular hypertrophy (RVH) caused by acute and long-term hemodynamic improvement. METHODS: Twenty-nine (29) patients with chronic thromboembolic pulmonary hypertension (CTEPH) and seven patients with pulmonary arterial hypertension (PAH) were included in the analysis. Patients with CTEPH achieved a significant long-term hemodynamic improvement following the treatment with balloon pulmonary angioplasty (BPA); all the patients with PAH reported significant acute hemodynamic relief after a single inhalation of iloprost, fulfilling the criteria of responder. Standard 12-lead ECG was performed before and after intervention. RESULTS: The interval between baseline and control ECG in CTEPH and PAH groups was 28 (IQR: 17-36) months and 15 min (IQR: 11-17), respectively. Despite similar hemodynamic improvement in both groups, only the CTEPH group presented significant changes in most analyzed ECG parameters: T-wave axis (p = .002), QRS-wave axis (p = .012), P-wave amplitude (p < .001) and duration in II (p = .049), R-wave amplitude in V1 (p = .017), R:S ratio in V1 (p = .046), S-wave amplitude in V5 (p = .004), R-wave amplitude in V5 (p = .044), R:S ratio in V5 (p = .004), S-wave amplitude in V6 (p = .026), R-wave amplitude in V6 (p = .01), and R-wave amplitude in aVR (p = .031). In patients with PAH, significant differences were found only for P wave in II (duration: p = .035; amplitude: p = .043) and QRS axis (p = .018). CONCLUSIONS: The effective treatment of cPH ensures improvement in ECG parameters of RVH, but it requires extended time.