BACKGROUND AND AIMS: Adults with congenital heart disease (ACHD) knowledge regarding their heart condition is crucial for optimal long-term outcome. Previous studies from North-Western Europe showed that important gaps in ACHD knowledge still exist. This study evaluates ACHD patients' knowledge in Central and South-eastern Europe (CESEE) and aims to identify opportunities for improving life-long ACHD care and outcomes in this region. METHODS: A structured survey regarding the baseline heart condition knowledge was prospectively distributed to stable ACHD patients over a one-year period (2021-2022). Patients' responses were verified by their ACHD physicians to ensure accurate background information. RESULTS: Among 1650 patients (age 34.5 ±14) across 14 CESEE countries the majority 1023(62.0%) had simple congenital heart disease with at least one previous heart procedure performed 1201(72.8%); 1060(64.2%) were asymptomatic and 875(53.8%) had secondary school education. Overall, 576(34.9%) did not have basic knowledge regarding their congenital heart disease and 146(12.2%) did not have basic understanding regarding their previous heart procedure/s. Patients considered their life expectancy similar to the general population (p=0.039). Encouragingly, 962(59.5%) expressed a desire to learn more, and 929(58.1%) favoured technological integration in their care. CONCLUSIONS: Significant knowledge gaps exist amongst CESEE ACHD patients regarding their heart condition. Better ACHD patient education on current health and prospects is urgently needed. The results of this study should serve for developing congenital heart disease structured transitional and educational programmes in CESEE incorporating technology for their ACHD care and education to enhance patients' health knowledge and healthy life-behaviours to positively influence their life-long prospects.

Adults with congenital heart disease (ACHD) represent one of the fastest-growing patient groups in cardiology globally, yet the current study shows significant knowledge gaps regarding their condition exist among ACHD patients in Central and South-Eastern European region, despite the crucial role that adequate health understanding plays in making informed life choices, adhering to medical care, and ultimately achieving the full life-long potential.In Central and South-Eastern Europe 34.9% of ACHD patients do not have basic knowledge regarding their congenital heart disease, whereas 12.2% do not have any basic understanding of their previous heart procedure/s.Interestingly, patients across different congenital heart disease complexity groups considered their life expectancy similar to the general population.

• Klíčová slova
• adult congenital heart disease, congenital heart disease, patient education,
• Publikační typ
• časopisecké články MeSH

Pulmonary arterial hypertension (PAH) is a severe and progressive disease with limited survival prospects under currently available therapies. Since the 2022 edition of the European Society of Cardiology and European Respiratory Society guidelines on pulmonary hypertension, substantial clinical evidence has emerged, supporting a new treatment algorithm for PAH as presented at the 7th World Symposium on Pulmonary Hypertension 2024 and the following proceeding papers. Key updates include the introduction of sotatercept as a second-line therapy leading to a revised definition of maximal medical therapy now encompassing agents from four therapeutic groups (phosphodiesterase-5 inhibitors/soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin pathway agents, and sotatercept), instead of three (phosphodiesterase-5 inhibitors/soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin pathway agents). Other novelties include the elimination of a distinct pathway for patients with cardiopulmonary comorbidities in favor of an individualized approach, a reduction in the initial patient assessment risk categories from three to two, and a follow-up interval shortened from 3-6 months to 3-4 months post-treatment initiation. This review presents these advancements and emphasizes the need for their widespread implementation in clinical practice. At the end, we present new opportunities and challenges in the treatment of pulmonary arterial hypertension in eight Central and Eastern European countries.

• Klíčová slova
• activin signaling inhibitors, novel therapies, risk assessment, sotatercept, treatment strategy,
• MeSH
• antihypertenziva terapeutické užití   MeSH
• inhibitory fosfodiesterasy 5 terapeutické užití   MeSH
• lidé MeSH
• plicní arteriální hypertenze * farmakoterapie   MeSH
• plicní hypertenze farmakoterapie   terapie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Názvy látek
• antihypertenziva MeSH
• inhibitory fosfodiesterasy 5 MeSH

Chronic thromboembolic pulmonary hypertension (CTEPH) is successfully treatable with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty, and medical therapy. Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management risk score (RRS) is able to predict long-term outcome in inoperable patients or in patients with residual PH after surgery. We performed a post hoc analysis of RRS in patients who were enrolled in the CTREPH study (NCT01416636), a randomized, double-blind clinical trial comparing high-dose and low-dose subcutaneous (SC) treprostinil in patients with severe CTEPH that was classified by an interdisciplinary CTEPH team as nonoperable, or as persistent or recurrent pulmonary hypertension after PEA. Baseline mean RRS was similar in both treatment groups (8.7 in high-dose arm vs. 8.6 in low-dose arm), but mean RRS change from baseline to Week 24 was greater in the high-dose treprostinil group than in the low-dose treprostinil group (-0.88 vs. -0.17). The difference in RRS change from baseline to Week 24 between high dose versus low dose was statistically significant with mean difference of -0.70 (95% confidence interval: -1.36 to -0.05, p = 0.0352), and was driven mainly by improvement of World Health Organization functional class and N-terminal pro-brain natriuretic peptide concentration. SC treprostinil therapy administered in standard dose had positive effect on the risk profile measured by RRS in patients with inoperable or persistent/recurrent severe CTEPH. Although our study was limited by the small sample size and post hoc nature, assessment of risk profile is of great importance to this particular patient population with very poor prognosis.

• Klíčová slova
• REVEAL risk score, chronic thromboembolic pulmonary hypertension, treprostinil,
• Publikační typ
• časopisecké články MeSH

OBJECTIVES: To determine the long-term and transplantation-free survival of all patients after atrial correction of transposition of the great arteries (TGA) in the Czech and Slovak republics, including its preoperative and perioperative determinants. METHODS: Retrospective analysis of all 454 consecutive patients after atrial correction of TGA was performed. Of these, 126 (27.8%) were female, median age at operation was 7.4 months (Q1 5.3; Q3 13.3) and 164 (36.1%) underwent the Mustard procedure. The relationships between age, weight, the complexity of TGA, operative technique, additional surgical procedures, immediate postoperative presence of tricuspid regurgitation and revision procedures during follow-up to major composite outcome, as such defined as long-term and transplantation-free survival, were tested. RESULTS: Early 30-day mortality did not differ between the Mustard (9.76%) and Senning (8.97%) cohorts (p=0.866). The long-term and transplantation-free survival, which differed between the Mustard and Senning cohorts in favour of the Senning procedure (HR 0.43; 95% CI 0.21 to 0.87), was shorter in complex TGA (HR 2.4; 95% CI 1.59 to 3.78) and in complex surgical interventions (HR 3.51; 95% CI 2.31 to 5.56). The immediate presence of at least moderate tricuspid regurgitation after correction was associated with a shorter long-term and transplantation-free survival in the univariate but not in the multivariable model. CONCLUSIONS: The lower long-term survival of patients after an atrial switch operation of TGA in the Czech and Slovak republics is associated with greater complexity of TGA, complex surgical interventions and application of the the Mustard operative procedure.

• Klíčová slova
• heart defects, congenital, transposition of great vessels, tricuspid valve insufficiency,
• MeSH
• arterie MeSH
• kojenec MeSH
• korekce transpozice velkých arterií * škodlivé účinky   MeSH
• lidé MeSH
• následné studie MeSH
• retrospektivní studie MeSH
• transpozice velkých cév * MeSH
• trikuspidální insuficience * chirurgie   MeSH
• výsledek terapie MeSH
• Check Tag
• kojenec MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

Background and Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) is a chronic progressive disease, resulting from persistent arterial obstruction combined with small-vessel remodeling. Central and peripheral CTEPH are distinguished, according to the dominant lesion's location. This is important for surgical or percutaneous interventional assessment or for medical treatment. Material and Methods: Eighty-one patients (51 male/30 female) with confirmed CTEPH were analyzed, while the CENTRAL type included 51 patients (63%) and the PERIPHERAL type 30 patients (37%). Results: A significant difference in CENTRAL type vs. PERIPHERAL type was determined in gender (male 72.5% vs. 46.7%; p = 0.0198). No difference was found in age, functional status, or echocardiographic parameters. Invasive hemodynamic parameters showed a significant difference in mean pulmonary arterial pressure (46 vs. 58 mmHg; p = 0.0002), transpulmonary gradient (34 vs. 47 mmHg; p = 0.0005), and cardiac index (2.04 vs. 2.5 L.min.m2; p = 0.02) but not in pulmonary vascular resistance. Risk factors showed a significant difference only in acute pulmonary embolism (93.8% vs. 60%; p = 0.0002) and malignancy (2% vs. 13.3%; p = 0.0426). Conclusions: Our study showed hemodynamic differences between CENTRAL type vs. PERIPHERAL type CTEPH with a worse hemodynamic picture in CENTRAL form. This may indicate a different pathophysiological response and/or possible additional influences contributing especially to the peripheral pulmonary bed affection.

• Klíčová slova
• central CTEPH, chronic thromboembolic pulmonary hypertension (CTEPH), hemodynamic evaluation, peripheral CTEPH, risk factors,
• MeSH
• cévní rezistence MeSH
• chronická nemoc MeSH
• hemodynamika MeSH
• lidé MeSH
• plíce MeSH
• plicní embolie * komplikace   MeSH
• plicní hypertenze * komplikace   farmakoterapie   MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

AIMS: To examine the current status of care and needs of adult congenital heart disease (ACHD) services in the Central and South Eastern European (CESEE) region. METHODS AND RESULTS: We obtained data regarding the national ACHD status for 19 CESEE countries from their ACHD representative based on an extensive survey for 2017 and/or 2018. Thirteen countries reported at least one tertiary ACHD centre with a median year of centre establishment in 2007 (interquartile range 2002-2013). ACHD centres reported a median of 2114 patients under active follow-up with an annual cardiac catheter and surgical intervention volume of 49 and 40, respectively. The majority (90%) of catheter or surgical interventions were funded by government reimbursement schemes. However, all 19 countries had financial caps on a hospital level, leading to patient waiting lists and restrictions in the number of procedures that can be performed. The median number of ACHD specialists per country was 3. The majority of centres (75%) did not have ACHD specialist nurses. The six countries with no dedicated ACHD centres had lower Gross Domestic Product per capita compared to the remainder (P = 0.005). CONCLUSION: The majority of countries in CESEE now have established ACHD services with adequate infrastructure and a patient workload comparable to the rest of Europe, but important gaps still exist. ACHD care is challenged or compromised by limited financial resources, insufficient staffing levels, and reimbursement caps on essential procedures compared to Western Europe. Active advocacy and increased resources are required to address the inequalities of care across the continent.

• Klíčová slova
• Adult congenital heart disease, Central and South Eastern Europe, Tertiary centre,
• MeSH
• dospělí MeSH
• kardiologie * MeSH
• konsensus MeSH
• lidé MeSH
• srdeční selhání * MeSH
• vrozené srdeční vady * MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa MeSH

OBJECTIVE: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice. METHODS: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat. Patients were followed for at least 1 year and up to 4 years from enrollment or until 30 days after stopping riociguat treatment. Primary safety outcomes were adverse events (AEs) and serious adverse events (SAEs) coded using Medical Dictionary for Regulatory Activities preferred terms and System Organ Classes version 21.0, collected during routine clinic visits and collated via case report forms. RESULTS: In total, 956 patients with CTEPH were included in the analysis. The most common AEs in these patients were peripheral edema/edema (11.7%), dizziness (7.5%), right ventricular (RV)/cardiac failure (7.7%), and pneumonia (5.0%). The most common SAEs were RV/cardiac failure (7.4%), pneumonia (4.1%), dyspnea (3.6%), and syncope (2.5%). Exposure-adjusted rates of hemoptysis/pulmonary hemorrhage and hypotension were low and comparable to those in the long-term extension study of riociguat (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase-Stimulator Trial [CHEST-2]). CONCLUSION: Data from EXPERT show that in patients with CTEPH, the safety of riociguat in routine practice was consistent with the known safety profile of the drug, and no new safety concerns were identified.

• Klíčová slova
• Chronic thromboembolic pulmonary hypertension, Clinical practice, Real-world, Registry, Riociguat, Safety,
• MeSH
• analýza dat * MeSH
• bezpečnost MeSH
• časové faktory MeSH
• chronická nemoc MeSH
• lidé středního věku MeSH
• lidé MeSH
• multicentrické studie jako téma MeSH
• plicní embolie komplikace   farmakoterapie   MeSH
• plicní hypertenze farmakoterapie   etiologie   MeSH
• prospektivní studie MeSH
• pyrazoly škodlivé účinky   terapeutické užití   MeSH
• pyrimidiny škodlivé účinky   terapeutické užití   MeSH
• randomizované kontrolované studie jako téma MeSH
• recidiva MeSH
• registrace * MeSH
• senioři MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• pyrazoly MeSH
• pyrimidiny MeSH
• riociguat MeSH Prohlížeč

OBJECTIVE: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice. METHODS: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat. Patients were followed for at least 1 year and up to 4 years from enrollment or until 30 days after stopping riociguat treatment. Primary safety outcomes were adverse events (AEs) and serious adverse events (SAEs) coded using Medical Dictionary for Regulatory Activities preferred terms and System Organ Classes version 21.0, collected during routine clinic visits (usually every 3-6 months) and collated via case report forms. RESULTS: In total, 326 patients with PAH were included in the analysis. The most common AEs in these patients were dizziness (11.7%), right ventricular (RV)/cardiac failure (10.7%), edema/peripheral edema (10.7%), diarrhea (8.6%), dyspnea (8.0%), and cough (7.7%). The most common SAEs were RV/cardiac failure (10.1%), pneumonia (6.1%), dyspnea (4.0%), and syncope (3.4%). The exposure-adjusted rate of hemoptysis/pulmonary hemorrhage was 2.5 events per 100 patient-years. CONCLUSION: Final data from EXPERT show that in patients with PAH, the safety of riociguat in clinical practice was consistent with clinical trials, with no new safety concerns identified and a lower exposure-adjusted rate of hemoptysis/pulmonary hemorrhage than in the long-term extension of the Phase 3 trial in PAH.

• Klíčová slova
• Clinical practice, Pulmonary arterial hypertension, Pulmonary hypertension, Registry, Riociguat, Safety,
• Publikační typ
• časopisecké články MeSH

AIMS: Long-term persistence of perfusion defect after pulmonaryembolism (PE) may lead to the development of chronic thromboembolic pulmonary hypertension. Identification of patients at risk of such a complication using a scoring system would be beneficial in clinical practice. Here, we aimed to derive a score for predicting persistence of perfusion defects after PE. METHODS: 83 patients after PE were re-examined 6, 12 and 24 months after the PE episode. Data collected at the time of PE and perfusion status during follow-ups were used for modelling perfusion defects persistence using the Cox proportional hazards model and validated using bootstrap method. RESULTS: A simple scoring system utilizing two variables (hemoglobin levels and age at the time of PE) was developed. Patients with hemoglobin levels over 140 g/L who were older than 65 years were at the highest risk of perfusion defects; in patients with the same hemoglobin levels and age <65 years, the risk was reduced by 79%, and by 89% in patients with hemoglobin <140 g/L. CONCLUSION: The proposed scoring system may be useful in clinical practice for identifying patients with high risk of persisting perfusion defects, flagging them for closer follow up, thus improving the effectiveness of long-term treatment of patients after PE.

• Klíčová slova
• classification, perfusion defects, prediction score, pulmonary embolism, reperfusion, risk score,
• MeSH
• antikoagulancia terapeutické užití   MeSH
• dospělí MeSH
• echokardiografie MeSH
• hemoglobiny analýza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• plicní embolie komplikace   farmakoterapie   MeSH
• plicní hypertenze farmakoterapie   etiologie   MeSH
• pravidla klinického rozhodování * MeSH
• proporcionální rizikové modely MeSH
• rizikové faktory MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• věkové faktory MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• srovnávací studie MeSH
• Názvy látek
• antikoagulancia MeSH
• hemoglobiny MeSH

BACKGROUND: Treprostinil, a prostacyclin analogue, is effective for the treatment of pulmonary arterial hypertension. However, information is scarce regarding treprostinil for treatment of chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to examine the efficacy and safety of subcutaneous treprostinil in this setting. METHODS: In this 24-week, randomised, double-blind controlled trial, we enrolled patients with CTEPH, classified as non-operable, or with persistent or recurrent pulmonary hypertension after pulmonary endarterectomy, in six European expert centres in Austria, Czech Republic, Germany, and Poland. Patients in WHO functional class III or IV with a 6-min walk distance of 150-400 m were randomly assigned at a 1:1 allocation ratio to continuous high-dose subcutaneous treprostinil (target dose around 30 ng/kg per min at week 12) or low-dose subcutaneous treprostinil (target dose around 3 ng/kg per min at week 12). The primary endpoint was the change from baseline in 6-min walk distance at week 24. All patients who received at least one dose of the study drug were included in the intention-to-treat efficacy and safety analyses based on assessment of adverse events. The trial was registered at ClinicalTrialsRegister.eu EudraCT number 2008-006441-10 and ClinicalTrials.gov, number NCT01416636. FINDINGS: From March 9, 2009, to June 9, 2016, 105 patients were enrolled with 53 (50%) patients randomly assigned to high-dose and 52 (50%) patients to low-dose subcutaneous treprostinil. At week 24, marginal mean 6-min walk distance improved by 44·98 m (95% CI 27·52 to 62·45) in the high-dose group, and by 4·29 m (95% CI -13·34 to 21·92) in the low-dose group (treatment effect 40·69 m, 95% CI 15·86 to 65·53, p=0·0016). 12 serious adverse events were reported in ten (19%) of 52 patients from the low-dose group and 16 serious adverse events were reported in nine (17%) of 53 patients from the high-dose group. The most common treatment-related adverse events in both groups were infusion site pain and other infusion site reactions. INTERPRETATION: Treatment with subcutaneous treprostinil was safe, and improved exercise capacity in patients with severe CTEPH. Subcutaneous treprostinil provides a parenteral treatment option for patients of WHO functional class III or IV and those who do not tolerate other therapies or need combination treatment. FUNDING: SciPharm Sàrl.

• MeSH
• antihypertenziva aplikace a dávkování   škodlivé účinky   MeSH
• dvojitá slepá metoda MeSH
• epoprostenol aplikace a dávkování   škodlivé účinky   analogy a deriváty   MeSH
• injekce subkutánní MeSH
• lidé středního věku MeSH
• lidé MeSH
• monitorování léčiv metody   MeSH
• plicní embolie komplikace   MeSH
• plicní hypertenze * diagnóza   farmakoterapie   etiologie   patofyziologie   MeSH
• stupeň závažnosti nemoci MeSH
• test chůzí metody   MeSH
• tolerance zátěže účinky léků   MeSH
• výsledek terapie MeSH
• vztah mezi dávkou a účinkem léčiva MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinické zkoušky, fáze III MeSH
• multicentrická studie MeSH
• práce podpořená grantem MeSH
• randomizované kontrolované studie MeSH
• Názvy látek
• antihypertenziva MeSH
• epoprostenol MeSH
• treprostinil MeSH Prohlížeč