Search Results (217)

Non-invasive methods used for PH detection in clinical practice have several limitations. The combination of high spatiotemporal sensitivity magnetocardiography (MCG) and artificial intelligence algorithms may offer an accurate approach for PH detection. In this study, we develop a convolutional neural network (CNN) model based on the 64-channel MCG time-series data. This exploratory study enrolled patients undergoing 64-channel MCG, including right-heart-catheterization confirmed PH patients and symptomatic controls with low echocardiographic probability of PH. After data preprocessing, a temporal CNN integrating MCG signals with age, sex, and body mass index was developed and compared with conventional machine learning models. The CNN model achieved strong discrimination, with area under the curve (AUC) values of 0.939 (95% confidence interval [CI]: 0.913–0.961) in the development out-of-fold evaluation and 0.974 (95% CI: 0.944–0.994) in the hold-out test set, outperforming conventional machine learning models. Decision curve analysis showed the greatest net benefit at clinically relevant thresholds. Attribution analysis indicated that spatial QRS morphology redistribution contributed substantially to PH classification. The temporal CNN model based on raw 64-channel MCG signals showed promising performance for non-invasive PH detection and outperformed conventional machine learning approaches in this exploratory single-center cohort enriched for PAH and CTEPH. Full article

Background/Objectives: Right heart failure remains the leading cause of death in pulmonary hypertension. Early detection of right-sided congestion is crucial but relies largely on clinical signs with limited diagnostic accuracy. We performed a post hoc analysis of the CODOVEIN study to evaluate the diagnostic performance of clinical and non-invasive parameters for predicting elevated right atrial pressure (RAP). Methods: This post hoc analysis included patients from a prospective cross-sectional study who underwent right heart catheterization. Clinical signs, echocardiographic parameters, venous Doppler indices, and NT-proBNP levels were assessed within four hours before catheterization. Patients were stratified according to invasively measured RAP. Diagnostic performances were evaluated using sensitivity, specificity, predictive values, likelihood ratios, and ROC curves. Results: Several clinical and imaging parameters were associated with increasing RAP. Among non-invasive markers, the femoral venous stasis index (FVSI) and inferior vena cava (IVC) collapsibility assessed in transverse view showed the best discriminative ability for detecting RAP > 14 mmHg. FVSI demonstrated the highest positive likelihood ratio (>11) and an excellent negative predictive value (>0.98), outperforming other clinical and echocardiographic markers. Conclusions: In patients with pulmonary hypertension, FVSI provides robust non-invasive identification of right atrial pressure elevation and may complement traditional clinical assessment for the early detection of right-sided congestion. Full article

(1) Background: Left ventricular assist device (LVAD) implantation is a valuable alternative as a bridge to transplant but also as a destination therapy in ineligible patients. Right ventricular failure (RVF) is a major cause of short- and long-term mortality post-LVAD. We aimed to validate echocardiographic and hemodynamic parameters predictive of RVF and adverse outcomes post-LVAD; (2) Methods: We screened a population of patients with end-stage heart failure selected for LVAD implantation according to SIENA protocol and standard international indications, including right heart catheterization (RHC). Individuals were followed up for 1 year with different time points for the development of RVF (primary endpoint) or mortality and hospitalization (secondary endpoint); (3) Results: The population included 29 patients with a mean age of 63 ± 7 years with a mean ejection fraction of 23 ± 4%, mostly due to ischemic etiology. All the patients had a SIENA protocol score of 0–1 before LVAD, and none met the primary endpoint. Regarding the secondary endpoint, among all the tested clinical, laboratory, echo, and RHC indices, only a central venous pressure/wedge pressure (CVP/PCWP) ratio > 0.63 was significantly associated with adverse outcomes (ß = 2.99, p = 0.026); (4) Conclusions: Excluding a pre-implantation RV dysfunction according to SIENA protocol significantly reduces the risk of post-LVAD RVF. The CVP/PCWP ratio may be an additional prognostic marker for mortality and rehospitalization in LVAD patients. Full article

Introduction: The Melody transcatheter pulmonary valve (TPV) was the first percutaneous bioprosthetic valve approved for transcatheter pulmonary valve implantation (TPVI). We report our single-centre experience with Melody TPV implantation in patients with congenital heart disease (CHD). Methods: This retrospective observational single-centre study included all patients evaluated in the catheterization laboratory for Melody TPV implantation. Early outcomes included procedural failure, life-threatening adverse events, and mortality. Long-term outcomes assessed during follow-up included infective endocarditis, transcatheter reintervention, and surgical reintervention. Results: Between 2015 and 2025, 50 consecutive patients were evaluated for TPVI with the Melody TPV at our institution. In four patients (8%), the procedure was aborted because of coronary artery compression detected during balloon interrogation of the right ventricular outflow tract (RVOT). One patient (2%) died of septic shock following acute pulmonary oedema in the immediate post-procedural period. The remaining 45 patients (90%) underwent successful Melody TPV implantation and were discharged from hospital. In six patients, the Melody TPV was implanted off-label: in the tricuspid position (n = 2) and in small conduits (<16 mm) (n = 4). Mean follow-up duration was 5.8 ± 3.6 years. One patient was lost to follow-up. Among the remaining 44 patients, seven (15.9%; 2.7% per patient-year) developed infective endocarditis, seven (15.9%; 2.7% per patient-year) underwent transcatheter reintervention (six balloon dilatations of the Melody valve and one valve-in-valve implantation), and four (9.1%; 1.5% per patient-year) required surgical replacement of the Melody TPV. Conclusions: Transcatheter implantation of the Melody TPV is an effective treatment for RVOT dysfunction. At mid-term follow-up, the majority of implanted Melody valves demonstrated satisfactory function, and only a minority of patients required surgical valve replacement. Full article

A 40-year-old man with complex congenital heart disease (double-inlet left ventricle with transposition of the great arteries), previously treated with a Blalock–Taussig shunt in infancy and a modified Fontan procedure (including superior vena cava-to-pulmonary artery anastomosis, atriopulmonary connection, and tricuspid valve closure with a Dacron patch), presented to the emergency department with worsening dyspnea and hypoxemia (SpO₂ < 80%). Echocardiography suggested a shunt through the tricuspid patch, possibly related to prior atrial flutter ablation. Cardiac catheterization confirmed an approximately 10 mm fenestration in the calcified patch causing a significant bidirectional shunt, along with two fistulae between the innominate vein and the left atrium. The fenestration was successfully closed using a septal occluder via right femoral venous access under transesophageal echocardiographic guidance. The venous collaterals were occluded with vascular plugs via right femoral and left brachial approaches. Technical success of the closure of the intracardiac and the venous shunts was confirmed angiographically at the end of the procedure. Oxygen saturation improved immediately from 72% to 91% and remained stable at the 2-year follow-up. Similarly, NYHA functional class improved from IV to II and episodes of tachycardia became less frequent and better tolerated, with sustained benefit throughout follow-up. Full article

Pulmonary arterial hypertension (PAH) is a progressive, fatal disease of the pulmonary vasculature characterized by obliterative remodeling of small pulmonary arteries, leading to sustained elevation of pulmonary vascular resistance, right ventricular failure, and premature death. The diagnostic gold standard remains right heart catheterization, requiring a mean pulmonary artery pressure greater than 20 mmHg at rest, a pulmonary arterial wedge pressure of 15 mmHg or below, and a pulmonary vascular resistance exceeding 2 Wood units. PAH is an autosomal dominant disorder with markedly incomplete penetrance of approximately 20–30%, indicating that germline mutations alone are insufficient to cause disease. Disease manifestation requires additional “second hits”, including chronic hypoxia, systemic inflammation, hemodynamic stress, hormonal influences, and common genetic modifiers such as single-nucleotide polymorphisms (SNPs). This genetic and environmental complexity underpins the broad clinical heterogeneity observed across PAH subtypes, which include idiopathic PAH, heritable PAH, and disease associated with connective tissue disorders, HIV infection, portal hypertension, congenital heart disease, schistosomiasis, and drug or toxin exposure. This review provides a comprehensive and critical appraisal of the molecular-genetic architecture of PAH. Thirty genes have now been implicated in disease pathogenesis, spanning seven functional categories: receptors of the TGF-β/BMP signaling family (BMPR2, ACVRL1, ENG, BMPR1B); circulating BMP ligands (GDF2, BMP10); transcription factors (TBX4, SOX17, KLF4, FOXF1, SMAD1, SMAD4, SMAD9); membrane and polyamine transporters (ATP13A3, AQP1); potassium channel regulators (KCNA5, KCNK3, ABCC8); metabolic and mitochondrial genes (EIF2AK4, NFU1, GGCX); signaling receptors and structural proteins (NOTCH3, KDR, CAV1, PLEKHH2); vasoactive and extracellular matrix regulators (KLK1, CBLN2, CD248); and epigenetic regulators (TET2, TOPBP1). Among these, BMPR2 is the dominant contributor, accounting for 53–86% of heritable PAH and 14–35% of idiopathic cases. The remaining genes each account for fewer than 5% of cases individually, collectively reflecting a broad landscape of rare and ultra-rare genetic contributions. For each gene, we critically evaluate the strength of genetic evidence, pathogenic mechanisms, degree of mechanistic resolution, and clinical relevance. We further discuss the contribution of emerging technologies, including whole-genome sequencing, single-cell and spatial transcriptomics, multi-omics integration, iPSC-derived vascular models, and artificial intelligence, to expanding the PAH genetic architecture beyond single-gene discovery. A key theme across this landscape is convergence: despite mechanistic diversity at the gene level, most PAH-associated variants ultimately impair endothelial quiescence, promote smooth muscle proliferation, and drive apoptosis resistance through disruption of BMP signaling amplitude, transcriptional stability, ion channel homeostasis, metabolic integrity, or epigenetic regulation. This convergence supports both a unified therapeutic rationale and a precision medicine framework for genotype-stratified intervention in PAH. Full article

Pulmonary hypertension (PH) is associated with adverse outcomes after heart transplantation (HT). In 2022, the European Society of Cardiology introduced lower thresholds for pulmonary vascular resistance (PVR) to distinguish isolated post-capillary PH (IpcPH) and combined post-capillary PH (CpcPH). We conducted a single-center retrospective study on 357 patients who underwent HT between 1985 and 2020 and had right heart catheterization prior to transplant, investigating the ability of the new PVR threshold to predict one-year mortality. Overall, 65 patients had no PH, 84 had IpcPH, and 208 had CpcPH. One-year survival was higher in patients without PH and similar between IpcPH and CpcPH (p = 0.04). Reclassification under the 2022 guidelines did not improve risk prediction. Only mean pulmonary artery pressure (mPAP) >20 mmHg independently predicted 1-year mortality. In conclusion, elevated mPAP, rather than PVR, was associated with post-transplant outcomes. This finding opens up the possibility of rethinking indication for reversibility tests and mechanical circulatory support in HT recipients. Full article

Background and Objectives: Pulmonary hypertension (PH) remains associated with substantial mortality despite advances in treatment. Although prognostic factors have been widely described at sea level, their behavior in populations living at high altitude remains insufficiently characterized. This study aimed to identify factors associated with mortality during follow-up in patients with Group 1 PH residing at high altitude. Materials and Methods: A retrospective cohort study was conducted including patients with confirmed Group I PH diagnosed by right heart catheterization and treated between 2017 and 2022. Clinical, functional, and hemodynamic variables were analyzed. A penalized logistic regression model using Elastic Net methodology was applied to identify variables associated with five-year mortality. Results: A total of 165 patients were included, with a mean age of 41 years (SD 13.93), and 84.2% were women. Among PH etiologies, congenital heart disease was the most frequent cause (50.3%), followed by idiopathic PH (33.3%) and connective tissue disease-associated PH (12.7%). Five-year mortality was 13.3% (22/165). Idiopathic pulmonary hypertension was significantly more frequent among deceased patients compared to survivors (13/22 [59.1%] vs. 42/143 [29.4%], p = 0.025). Mortality was associated with acute pulmonary embolism, greater smoking burden, worse functional class, and adverse hemodynamic parameters. In multivariable analysis, acute pulmonary embolism (coefficient 0.196; OR 1.216; 95% CI 1.16–1.27; p < 0.001), ESC/ERS risk stratification (coefficient 0.158; OR 1.171; 95% CI 1.08–1.26; p < 0.001), pulmonary vascular resistance > 25 wood units (coefficient 0.180; OR 1.198; 95% CI 1.13–1.26; p < 0.001), and age ≥ 65 years (coefficient 0.171; OR 1.187; 95% CI 1.10–1.27; p < 0.001) were identified as risk factors, while female sex showed a protective effect (coefficient −1.041; OR 0.353; 95% CI 0.33–0.37; p < 0.001). Conclusions: In patients with Group 1 PH living at high altitude, several clinical, functional, and hemodynamic variables were associated with increased mortality, including acute pulmonary embolism, elevated pulmonary vascular resistance, advanced age, and intermediate-high risk stratification. Female sex was associated with lower mortality. Full article

Background: Pulmonary arterial hypertension is a rare but life-threatening condition in children, with hereditary forms often being linked to mutations in genes such as bone morphogenetic protein receptor type 2 (BMPR2), caveolin 1 (CAV1), and potassium channel subfamily K member 3 (KCNK3). Among these, CAV1 mutations are associated with severe disease phenotypes, though cases resulting from de novo heterozygous CAV1 mutations with multi-system involvement remain rarely reported. The CAV1 mutation (c.424C > T, p.Q142X) disrupts caveolin-1 function, leading to dysregulated pulmonary vascular remodeling and multi-system abnormalities. Methods: This was a retrospective case study of a pediatric patient with hereditary PAH. The patient was followed at our hospital from initial presentation until death. Clinical data were collected from medical records, including physical examinations, laboratory tests, echocardiography, chest X-ray, computed tomography pulmonary angiography (CTPA), and genetic analysis. The patient was treated sequentially with various PAH-targeted medications. This report also includes a review of the relevant literature on CAV1-associated PAH. Results: A female aged 3 years and 11 months was diagnosed with hereditary PAH associated with a de novo heterozygous CAV1 mutation (c.424C > T, p.Q142X). Both parents underwent genetic testing and were negative for the mutation, confirming its de novo origin. Clinical manifestations included special facial features, congenital telangiectasia, cutis marmorata (marbled skin), congenital cataract, hereditary lipodystrophy, and severe PAH. The patient presented with progressive exercise intolerance, syncope, and worsening dyspnea over nine years. Echocardiography revealed pulmonary hypertension with an estimated pulmonary artery systolic pressure of 69–105 mmHg, right heart enlargement, right ventricular hypertrophy, and moderate tricuspid regurgitation. Blood and urine metabolic screenings were normal. A chest X-ray showed progressive enlargement of the cardiac silhouette and bulging of the pulmonary artery segment. CTPA demonstrated pulmonary hypertension, secondary right heart dysfunction, decompensated right ventricular function, and mosaic perfusion in both lungs, suggestive of small arterial branch occlusion. Right heart catheterization was declined by the parents. Thus, the diagnosis of PAH was established based on clinical, echocardiographic, CTPA, and genetic findings. The patient was hospitalized four times and lost to follow-up from 2017 to 2023. She received sequential treatment with digoxin, hydrochlorothiazide, tadalafil, ambrisentan, selexipag, and treprostinil. Despite these therapies, pulmonary artery pressure continued to rise with progressive clinical deterioration. The patient ultimately died at 13 years of age due to a pulmonary hypertensive crisis and multiple organ failure following a severe episode of gastroenteritis. Conclusions: Despite aggressive treatment with multiple targeted reduced pulmonary artery pressure drug therapies, managing hereditary PAH caused by CAV1 mutations in children remains a significant challenge, with a high mortality rate. Early genetic diagnosis, regular follow-up, and individualized treatment are crucial. It requires the joint efforts of patients, parents, and healthcare providers. Full article

Background: Right ventricular (RV) dysfunction is a key contributor to morbidity and mortality in systemic sclerosis (SSc), emerging from the combined effects of microvascular disease, myocardial fibrosis, interstitial lung involvement, and increasing pulmonary vascular load. Conventional echocardiography frequently fails to detect early RV impairment, prompting growing interest in deformation-based parameters such as RV free-wall longitudinal strain (RV-FWS), global longitudinal strain (RV-GLS), and RV–pulmonary artery (PA) coupling indices. Although natriuretic peptides reflect myocardial stress and are widely used in cardiopulmonary diseases, their integration with advanced RV imaging has been inconsistently reported in SSc. This systematic review synthesizes available evidence on RV strain, RV–PA coupling, and their relationship with clinical outcomes and biomarkers in SSc. Methods: A systematic search was conducted to identify clinical studies evaluating RV strain (RV-FWS, RV-GLS), right atrial strain, or RV–PA coupling indices in adult patients with SSc or SSc-associated pulmonary arterial hypertension (SSc-PAH). Eligible studies included those using speckle-tracking echocardiography or cardiac magnetic resonance feature-tracking. Study selection and data extraction were performed in accordance with PRISMA guidelines. Results: Seven studies met the eligibility criteria. Across unselected SSc cohorts, early disease without pulmonary hypertension (PH), and right-heart-catheterization-confirmed SSc-PAH, RV strain consistently detected myocardial impairment even when conventional echocardiographic indices remained normal. RV-FWS and RV-GLS were commonly reduced, and longitudinal data demonstrated progressive deterioration independent of standard measures. Strain-derived RV–PA coupling, particularly RV-FWS/PASP, significantly improved prognostic stratification when added to established PAH risk models. Two studies identified impaired RV deformation as a predictor of mortality, and CMR-derived right atrial strain provided additional prognostic value. Biomarker integration was limited, with only one study reporting an association between natriuretic peptide elevation (NT-proBNP) and impaired RV–PA coupling suggesting that biomarkers may reflect the hemodynamic load, although evidence remains limited captured by strain abnormalities. Conclusions: RV strain and RV–PA coupling indices are more sensitive than conventional echocardiography for detecting early RV dysfunction, monitoring disease progression, and predicting adverse outcomes in SSc. Although biomarker evidence remains limited, available data suggest that natriuretic peptides may provide complementary information to deformation-based assessment, although current evidence remains limited by reflecting combined myocardial and pulmonary vascular load. Standardized prospective studies including both strain imaging and biomarkers are needed to clarify the integrated diagnostic and prognostic value of advanced RV assessment in SSc. Full article

Pulmonary hypertension (PH) can be defined as a mean pulmonary artery pressure (mPAP) greater than 20 mm Hg at rest during right heart catheterization (RHC). The reported prevalence of PH throughout the globe has been estimated to impact approximately 1% of the total population, with a majority of those afflicted being women more than men. Numerous etiologies give rise to the pathophysiology of PH, including heart disease (i.e., left-sided heart failure), lung diseases, and other unclear causes related to chronic stages and complications surrounding long-standing pulmonary thromboembolisms, side effects of certain medications, and genetic and environmental factors. Untreated PH can lead to severe morbidities such as cardio-renal syndrome and congestive hepatopathy (cardiac cirrhosis). Management of PH focuses on decreasing pulmonary pressures by using vasodilators such as prostanoids, and phosphodiesterase type 5 (PDE-5) inhibitors, as well as newer treatments such as sotatercept, which inhibits activin signaling, thereby inhibiting excessive cell growth in the pulmonary artery vasculature and down-regulating the pro-proliferative pathways. Full article

Tricuspid regurgitation (TR) is a prevalent cardiovascular disorder with significant clinical impact. TR is frequently silent and underdiagnosed and is estimated to impact over 70 million people globally. Characterized by retrograde blood flow from the right ventricle into the right atrium due to incomplete valve closure, TR leads to right heart dilation, systemic congestion, and eventually right-sided heart failure. Importantly, TR may contribute to the onset of atrial fibrillation (AF), the most common sustained arrhythmia, affecting approximately 59 million individuals worldwide. Despite its growing clinical importance, the pathophysiology of TR remains incompletely understood, and current animal models of TR, based on direct valve manipulation, limit translational applicability. We present a novel, minimally invasive porcine model of TR established via femoral/jugular vein catheterization with deployment of an inferior vena cava (IVC) filter. The filter partially impedes tricuspid valve closure, inducing TR without valvular injury. Validation was achieved through multimodal imaging, including fluoroscopy, echocardiography, and electrocardiography, confirming hallmark features of TR, including right atrial and ventricular enlargement and arrhythmic activity. This model provides a reproducible, minimally invasive platform for studying selected features of TR progression. Its minimally invasive nature and preservation of native valvular structure make it a useful preclinical platform for mechanistic and translational research. Full article

Background: Heart failure (HF) is a major public health challenge. Management at or transfer to advanced therapy centers (ATCs) is linked to greater procedural use and better outcomes for HF, however there is little data on the impact of patient sex on access to ATCs and transfer patterns. We evaluated sex-based differences in HF management and outcomes during admissions across center types and transfer status. Method: Adult HF admissions were identified in the 2016–19 Nationwide Readmissions Database. Centers performing ≥1 heart transplant or LVAD were classified as ATCs. Patients were stratified by sex and center type: (A) non-ATC admission, (B) ATC admission, (C) transfer to ATC. Multivariable regression adjusted for comorbidities and HF decompensations. Results: Among 2,872,268 weighted HF admissions (51.3% male), females were older, while males had more HF decompensations (cardiogenic shock, ventricular arrhythmias, mechanical ventilation, AKI). Females comprised only 39.6% of all transfers to ATCs (0.4% vs. 0.6%, OR 0.69, p < 0.001) and had a lower unadjusted mortality (2.6% vs. 2.8%, p < 0.001); however, rates of transfer and mortality were similar between sexes when adjusted for comorbidities and HF decompensations. Female patients were significantly less likely to receive invasive procedures (CRT/ICD, PCI, right heart catheterization, CABG, temporary mechanical support, ECMO, LVAD or heart transplant) across all hospital types and transfers. This disparity in procedural utilization persisted after multivariable adjustment and in sensitivity analysis of patients with severe HF. Conclusions: Females had lower frequency of transfer to ATCs. In-hospital mortality and transfer rates to ATCs were similar across patient sex when adjusted for comorbidities and HF decompensations. Females consistently underwent fewer diagnostic and therapeutic interventions across all center types and transfers. Full article

Background and Clinical Significance: Pulmonary hypertension (PH) is a recognized complication of chronic liver disease, most commonly manifesting as portopulmonary hypertension (POHP) prior to liver transplantation. While the natural history and management of pre-transplant PH are well described, the development of de novo pulmonary arterial hypertension (PAH) following liver transplantation remains exceedingly rare and poorly understood. In such cases, establishing true causality is challenging, and alternative explanations—including previously unrecognized or masked disease—must be carefully considered. This entity poses significant diagnostic and therapeutic challenges and may adversely affect post-transplant outcomes if not promptly recognized and treated. Case Presentation: We report the case of a 46-year-old man with end-stage liver disease secondary to alcohol use who underwent deceased donor liver transplantation without preoperative evidence of PH. His pre-transplant evaluation revealed preserved biventricular function and no measurable PH. Eight days postoperatively, he was readmitted with acute dyspnea, hypoxemia, and signs of right ventricular failure. Transthoracic echocardiography demonstrated severe right ventricular dilation and dysfunction with markedly elevated pulmonary artery systolic pressure. Right heart catheterization confirmed severe PAH. Secondary causes of PH were excluded. The patient was initiated on sildenafil and continuous intravenous epoprostenol, resulting in clinical, echocardiographic, and hemodynamic improvement. Subsequent follow-up demonstrated sustained response to therapy despite concurrent progression of coronary artery disease requiring complex percutaneous intervention. Conclusions: This case highlights a rare presentation of severe PAH occurring shortly after liver transplantation, in the absence of documented pre-transplant PH. While a causal relationship cannot be definitively established, the temporal association raises important clinical considerations. It underscores the need for heightened clinical vigilance for pulmonary vascular disease in post-transplant patients presenting with cardiopulmonary symptoms. Further research is warranted to elucidate the underlying mechanisms, risk factors, and optimal management strategies for PAH diagnosed after liver transplantation. Full article

Background/Objectives: Pulmonary arterial hypertension (PAH) impacts various aspects of patients’ lives. Some questionnaires assessing health-related quality of life are specific to PAH patients. The aims of the study were to translate and investigate the factor structure and psychometric properties of the Polish version of the EmPHasis-10 health-related quality of life questionnaire in a group of adults with PAH. Construct validity was explored by the relationship with results of the 36-Item Short Form Survey (SF-36) and non-invasive prognostic factors: WHO functional class, 6 min walk distance (6MWD) and NTproBNP level were measured. Methods: In a single-center study, PAH patients were included. The diagnosis of PAH was confirmed by right heart catheterization. The demographic and clinical data were obtained. The EmPHasis-10 and the SF-36 questionnaires were administered to all patients. Results: Data from 120 PAH patients, median age 57 (IQR 45–68.7) years, 88 (73%) women, were obtained. Most of the patients suffered from IPAH (73, 61%). Results revealed a unidimensional structure of the EmPHasis-10 questionnaire and demonstrated satisfactory reliability (Cronbach α = 0.94). The EmPHasis-10 showed an adequate relationship with both SF-36 dimensions and three non-invasive prognostic parameters, i.e., WHO functional class, 6MWD and NTproBNP level. Regression analysis indicated that the 6MWD was the only predictor of the EmPHasis-10. Conclusions: The obtained results showed very good psychometric properties and adequate internal consistency of the Polish version of EmPHasis-10 in PAH patients. The results showed a unidimensional structure and very good psychometric properties, including satisfactory internal consistency and external validity of the Polish version of the EmPHasis-10 scale in patients with PAH. Full article