Pulmonary arterial hypertension (PAH) is associated with significant symptoms and impaired health-related quality of life (HRQOL) and survival. Specialty palliative care (SPC), an intervention delivered by a multidiscipl...Pulmonary arterial hypertension (PAH) is associated with significant symptoms and impaired health-related quality of life (HRQOL) and survival. Specialty palliative care (SPC), an intervention delivered by a multidisciplinary team, is focused on improving HRQOL. We performed a randomized controlled trial (RCT) of SPC among adults with PAH and impaired HRQOL to determine the impact of SPC on HRQOL as assessed by PAH-SYMPACT. PAH-SYMPACT is a validated PAH disease-specific patient-reported outcome measure that assesses HRQOL across four domains: cardiopulmonary symptoms, cardiovascular symptoms, physical impact, and cognitive/emotional impact. Sixty-two patients were included (36 usual care, 26 SPC plus usual care), but only 58% of patients randomized to SPC completed their SPC visits. In the intention-to-treat analysis, SPC was associated with similar changes in total, symptom, and physical impact domain scores and increased cognitive/emotional impact scores, indicative of worse HRQOL. In the per-protocol analysis, SPC was not associated with significant changes in total or individual domain scores. According to a survey of patients who completed SPC visits, most were satisfied with their visit and were likely to recommend SPC for other patients with PAH. In conclusion, limited SPC visits in this single-center RCT were not associated with significant improvements in HRQOL among patients with PAH and impaired HRQOL, but the majority of patients who participated in a SPC intervention were satisfied and would recommend SPC to other PAH patients. Future studies of SPC in PAH are needed to determine the appropriate patient selection and timing for SPC. www.clinicaltrials.gov (NCT03905421).
Pulmonary arterial hypertension (PAH) is a complex progressive disease associated with high morbidity and mortality. Circulating serum biomarkers have the potential to optimize diagnosis and prognosis in PAH. The cellula...Pulmonary arterial hypertension (PAH) is a complex progressive disease associated with high morbidity and mortality. Circulating serum biomarkers have the potential to optimize diagnosis and prognosis in PAH. The cellular communication network (CCN) protein family is a group of similarly structured matricellular proteins with many roles ranging from fibrosis to malignancy. Individual CCN proteins have been associated with PAH in previous studies, but no study has evaluated multiple CCN proteins as potentially relevant biomarkers in PAH. This study sought to establish associations using the circulating concentrations of measurable CCN proteins and PAH diagnosis, severity, outcomes, and other biomarkers. Serum levels of CCN1, CCN2, CCN3, and CCN6 were measured utilizing 225 patients from the PAH Biobank (PAHBiobank) with available hemodynamic data and 40 control samples. Serum levels of CCN1, 2, 3, and 6 proteins were significantly increased in PAH compared to controls. CCN1, CCN2, and CCN3 were associated with a lower 6-min walk distance. CCN2 and CCN3 were also associated with worse New York Heart Association Functional Class. Higher CCN2 and CCN3 levels correlated with higher levels of Endostatin and NT-proBNP. CCN6 was not significantly associated with any hemodynamic or clinical variables in the PAH cohort. Our results suggest that multiple CCN proteins are increased in PAH and that CCN2 and CCN3 have the most potential as novel biomarkers in PAH.
Pulmonary arterial hypertension (PAH) is a life-threatening disease of the pulmonary arteries. Progressively increasing pulmonary artery pressure may lead to fatal failure of the right ventricle, necessitating aggressive...Pulmonary arterial hypertension (PAH) is a life-threatening disease of the pulmonary arteries. Progressively increasing pulmonary artery pressure may lead to fatal failure of the right ventricle, necessitating aggressive drug treatment. Sotatercept, a novel drug for PAH-targeted therapy, shows promising results in clinical trials. Nevertheless, efficacy in the heterogeneous PAH population and long-term outcomes have yet to be investigated. This case report illustrates the life-changing effects of sotatercept in a patient with heritable PAH associated with a TBX4 mutation. Our patient faced near immobility, oxygen dependency, and was listed for lung transplantation in November 2023. Following sotatercept administration from July 2024 onward, she started sporting activities and has been removed from the transplantation waiting list. Thereby, this case illustrates an exceptional response to sotatercept in a patient with TBX4-associated heritable PAH. Positive effects persist 1 year after initiation without significant side effects.
The identification of ubiquitous risk factors determining long-term morbidity is crucial in infants born prematurely when aiming to develop prevention strategies. In a cohort of deeply phenotyped infants born before 32 w...The identification of ubiquitous risk factors determining long-term morbidity is crucial in infants born prematurely when aiming to develop prevention strategies. In a cohort of deeply phenotyped infants born before 32 weeks gestational age, we successfully demonstrate that exposure to fine particulate matter during pregnancy is associated with increased odds of preeclampsia and altered birth weight percentiles, highlighting potential underlying effects on vascular and metabolic pathology in relation to the degree of immaturity.
Pulmonary arterial hypertension (PAH) in India is a substantial yet under-recognized contributor to cardiovascular morbidity and mortality. Its progressive course, nonspecific early manifestations, and heterogeneous aeti...Pulmonary arterial hypertension (PAH) in India is a substantial yet under-recognized contributor to cardiovascular morbidity and mortality. Its progressive course, nonspecific early manifestations, and heterogeneous aetiologies often lead to delayed diagnosis and fragmented care. The unique epidemiological profile of India, characterized by a higher prevalence of PAH due to factors such as uncorrected congenital heart disease, environmental exposure, and chronic infections, underscores the need for a management approach tailored to Indian settings. These realities intersect with systemic challenges, including late presentation, resource constraints, a lack of local guidelines, and the costs of diagnostic workups and therapies, which hinder the delivery of standardized care. Variabilty in clinical practice in PAH management underscores the need for guidelines that account for local context and resources. Despite robust international recommendations, including those from the European Society of Cardiology and the European Respiratory Society, implementation of standardized risk stratification and treatment algorithms remains inconsistent across Indian centers. This variability reflects disparities in infrastructure, expertise, and affordability rather than differences in disease biology. This consensus document reviews current guidelines and advances in the management of PAH, consolidating evidence with potential for adoption in India and other low- and middle-income countries (LMICs). This document also highlights the need to establish centers of excellence, develop specialized care protocols, and increase awareness to improve patient outcomes.
Red blood cell distribution width (RDW)-derived indicators have increasingly been recognized as biomarkers reflecting systemic inflammation and hematological disorders. However, the prognostic value of these indicators i...Red blood cell distribution width (RDW)-derived indicators have increasingly been recognized as biomarkers reflecting systemic inflammation and hematological disorders. However, the prognostic value of these indicators in critically ill patients with pulmonary hypertension (PH), especially the dynamic implications of their temporal changes, remains insufficiently elucidated. A total of 990 adult patients diagnosed with PH in the intensive care unit (ICU) based on ICD-9/10 codes were enrolled from the MIMIC-IV database. The association between baseline levels of three key indicators and the 1-year all-cause mortality risk was assessed using Cox proportional hazards models and restricted cubic spline (RCS) analysis, and the consistency of this association was assessed for external consistency in the eICU-CRD cohort ( = 298) and NWICU cohort ( = 182). In the subgroup of patients with ≥ 3 consecutive laboratory tests ( = 316), group-based multi-trajectory modeling (GBTM) was employed to identify the joint longitudinal trajectory patterns of these three indicators. The robustness of the prediction results was then evaluated using machine learning models as secondary exploratory analysis. In the MIMIC-IV cohort, higher baseline RAR (aHR = 1.17, 95% CI: 1.08-1.27) and RPR (aHR = 2.31, 95% CI: 1.38-3.86) were significantly associated with increased 1-year mortality risk, whereas higher HRR exerted a protective effect (aHR = 0.31, 95% CI: 0.17-0.58). Across the multiple cohorts, only RAR remained statistically significant in the multivariate model of the eICU-CRD cohort ( = 0.009); although RPR and HRR showed a consistent association, the results did not reach statistical significance. GBTM identified three distinct joint trajectory patterns. Compared with the reference group (Class 2), the high-risk trajectory group (Class 3, = 35) exhibited a significantly elevated mortality risk (aHR = 2.48). In the exploratory analysis, the support vector machine (SVM) demonstrated moderate discriminative ability (AUC = 0.782). RDW-derived indicators are independently associated with the mortality risk in critically ill patients with ICD-code defined PH. Both their baseline levels and short-term change trajectories may reflect the severity of the underlying disease and systemic stress response. These findings suggest that RDW-derived indicators play an important role in risk phenotyping; however, prospective studies are warranted to confirm their clinical significance.
Clinical worsening events are increasingly recognized as a meaningful outcome in pulmonary arterial hypertension (PAH). We applied machine-learning models to real-world data to identify clinical features that may predict...Clinical worsening events are increasingly recognized as a meaningful outcome in pulmonary arterial hypertension (PAH). We applied machine-learning models to real-world data to identify clinical features that may predict clinical worsening events in PAH. Data were obtained retrospectively from the electronic health records of adults diagnosed with PAH at Mayo Clinic locations (January 2015-December 2019). Machine-learning LASSO regularized logistic regression models were developed to analyze the association between 100 clinical features and occurrence of clinical worsening events. In total, 455 patients were included (mean age 62.1 years; 59.3% female). Of these, 232 (51.0%) experienced a clinical worsening event after a median (quartile 1, quartile 3) of 10.9 (4.1, 21.4) months. The best-performing model had an area under the curve of 0.78, sensitivity of 78%, and specificity of 59%. The model identified 11 clinical features associated with clinical worsening events in PAH with a non-zero coefficient: baseline age, body mass index (BMI), creatinine level, red cell (erythrocyte) distribution width (RDW), mean pulmonary arterial pressure, pulmonary vascular resistance, sodium concentration, and QT interval, along with dyspnea, number of clinic visits, and other forms of heart disease within a 1-month window before a clinical worsening event. Baseline RDW and BMI, and number of clinic visits within the 1-month window were statistically significantly associated with clinical worsening events. Our study used machine-learning models to identify clinical features associated with risk of clinical worsening events in PAH. Automation of risk prediction could lead to earlier therapeutic intervention to optimize patient outcomes.
Pulmonary hypertension (PH) is a major complication of interstitial lung disease (ILD) and is associated with increased morbidity and mortality. Early recognition of PH in ILD is crucial for guiding diagnostic evaluation...Pulmonary hypertension (PH) is a major complication of interstitial lung disease (ILD) and is associated with increased morbidity and mortality. Early recognition of PH in ILD is crucial for guiding diagnostic evaluation, initiating treatment, and considering lung transplantation. The previously developed PH-ILD Detection Tool by Parikh et al. incorporated eight variables to stratify patients into low-, intermediate-, and high-risk groups, and was subsequently validated in an independent cohort. While effective, the multi-component score may be difficult to implement in routine practice. The aim of this study was to develop a simplified assessment tool for PH in ILD that relies on easily obtainable clinical data. We retrospectively evaluated 154 ILD patients who underwent right heart catheterization. The abbreviated PH-ILD score assigned one point each for: (1) physical examination findings suggestive of PH, (2) diffusion capacity for carbon monoxide < 40% predicted, and (3) supplemental oxygen use (range 0-3). Diagnostic performance was assessed using sensitivity, specificity, predictive values, and an ROC curve. The mean age was 70.0 ± 12.1 years, and the overall prevalence of PH was 48.1%. PH prevalence increased with higher scores: 14.3% (score 0), 58.1% (score 1), 79.0% (score 2), and 87.2% (score 3). The abbreviated tool demonstrated good discriminatory ability (AUC 0.74; 95% CI 0.65-0.82; < 0.001). A threshold of ≥ 2 yielded a sensitivity of 90%, specificity of 58%, PPV of 78%, and NPV of 79%, while a score of 3 produced higher specificity (88%) with lower sensitivity (54%). This simplified 3-point PH-ILD assessment tool effectively stratifies PH risk in ILD using readily accessible clinical variables. A threshold of ≥ 2 provides high sensitivity for screening, whereas a score of 3 offers improved specificity. This tool may support early identification of PH in ILD and prompt timely referral for specialized evaluation and advanced therapies.
Pulmonary hypertension (PH) and diabetes frequently coexist, and metabolic dysregulation plays a role in PH pathogenesis. While preclinical studies indicate that certain antidiabetic therapies may beneficially impact the...Pulmonary hypertension (PH) and diabetes frequently coexist, and metabolic dysregulation plays a role in PH pathogenesis. While preclinical studies indicate that certain antidiabetic therapies may beneficially impact the pulmonary vasculature and right heart function in PH, clinical data remain limited. We conducted a retrospective cohort study of veterans with PH and diabetes using Veterans Health Affairs data (2003-2015). The associations between exposure to antidiabetic medications at the time of PH diagnosis and survival were analyzed using Cox proportional hazards regression. We identified 41,670 veterans with PH and diabetes, largely older men (median age 69, 97% male). Average survival from PH diagnosis was 3.5 years. After adjustment for demographics, comorbid conditions, renal function, and hemoglobin A1c, metformin and thiazolidinedione (TZD) exposure were associated with lower mortality (HR 0.828, 95% CI 0.799-0.857 and HR 0.838, 95% CI 0.793-0.885, respectively). Conversely, insulin use was associated with higher mortality. These associations remained consistent across multiple analytical approaches and were independent of baseline hemoglobin A1c levels. Interactions were observed between drug effects and both renal function and PH comorbidities, with metformin's protective effect enhanced in patients with lower eGFR but attenuated in those with lung disease. In conclusion, metformin and TZD use were associated with improved survival in veterans with PH and diabetes, while insulin use was associated with reduced survival. These findings suggest that metabolic modulation may represent a viable therapeutic strategy in PH, though could be context dependent. Prospective trials are needed to evaluate therapeutic approaches targeting the metabolic pathobiology of PH.
The revised definition of pulmonary hypertension (PH) reclassifies patients previously diagnosed with chronic thromboembolic pulmonary disease (CTEPD) without pre-capillary PH as having chronic thromboembolic pulmonary h...The revised definition of pulmonary hypertension (PH) reclassifies patients previously diagnosed with chronic thromboembolic pulmonary disease (CTEPD) without pre-capillary PH as having chronic thromboembolic pulmonary hypertension (CTEPH). Reclassified CTEPH patients demonstrated oxygen desaturation during exercise and reduced ventilatory efficiency, as patients with more severe CTEPH, indicating impaired exercise physiology. These findings support the data of prior studies and the clinical relevance of the newly defined hemodynamic thresholds in patients with CTEPH.
Pulmonary hypertension (PH) is a progressive condition associated with reduced physical activity and impaired quality of life. While exercise training is now recognised as a safe and beneficial adjunct to PH management,...Pulmonary hypertension (PH) is a progressive condition associated with reduced physical activity and impaired quality of life. While exercise training is now recognised as a safe and beneficial adjunct to PH management, opportunities for supervised programmes remain limited. There is a growing need for person-centred, acceptable interventions that enable patients to engage safely and meaningfully in physical activity within their own environment. The aim of this study was to explore the lived experiences of individuals with PH who participated in a 10-week, home-based exercise programme, and to evaluate its acceptability, utility, and perceived impact. Semi-structured interviews were conducted with participants ( = 13) diagnosed with precapillary PH, specifically pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), who completed the intervention. Data was analysed thematically to identify core themes reflecting patient experiences and perceived changes. Thematic analysis revealed four key themes: convenience and accessibility, development of exercise self-regulation skills, support and accountability, and perceived improvements in physical fitness and well-being. Person-centred, behaviourally informed home-based exercise interventions can help individuals with PH exercise safely, overcome fear, enhance self-efficacy, and re-engage with physical activity. These findings provide patient-driven insights to guide the design and implementation of scalable exercise models for the PH population.
Limited guidance exists for the management of methamphetamine-associated PAH (Meth-APAH) and the experiences of physicians treating this population are not well documented. This study explored how physicians approach dia...Limited guidance exists for the management of methamphetamine-associated PAH (Meth-APAH) and the experiences of physicians treating this population are not well documented. This study explored how physicians approach diagnosis and treatment and identified unmet needs in clinical care through structured interviews with 30 U.S. pulmonologists and cardiologists who manage patients with Meth-APAH. Physicians relied heavily on patient self-report to establish methamphetamine use; only 20% performed routine toxicology testing. Compared with patients with other forms of pulmonary arterial hypertension, those with methamphetamine-associated disease were younger, more likely to present in the emergency department, and more often diagnosed at advanced functional class based on physician perspectives. Physicians reported caution with initiating triple therapy and often preferred oral or subcutaneous prostacyclin agents due to concerns about adherence and risks of intravenous therapy. Socioeconomic instability and stigma were identified as major contributors to delayed diagnosis, nonadherence, and poor outcomes. Unmet needs included earlier recognition by frontline providers and greater access to addiction support resources. This study highlights critical challenges in diagnosing and managing Meth-APAH. Improving provider education, standardizing screening practices, and integrating addiction treatment into care pathways are essential to address unmet needs.
Pulmonary endarterectomy (PEA) is a potentially curative treatment for chronic thromboembolic pulmonary hypertension (CTEPH); however, decompensated right heart failure and concomitant cardiac procedures increase the ris...Pulmonary endarterectomy (PEA) is a potentially curative treatment for chronic thromboembolic pulmonary hypertension (CTEPH); however, decompensated right heart failure and concomitant cardiac procedures increase the risk of postoperative mortality. We report a case of operable CTEPH with severe mitral regurgitation causing right heart failure refractory to medical therapy. Emergent PEA combined with mitral valve plasty was considered unacceptably high risk. Although balloon pulmonary angioplasty (BPA) for CTEPH complicated by severe mitral regurgitation carries a risk of worsening pulmonary congestion, we assessed pulmonary artery wedge pressure by right heart catheterization and confirmed that it was not elevated after aggressive diuretic therapy. Therefore, a single BPA session was performed using undersized balloons, with treatment limited to a small number of lesions. This approach stabilized the patient's right heart failure without exacerbating pulmonary congestion and left-sided heart failure. Subsequently, elective PEA combined with mitral valve plasty was successfully performed, resulting in marked improvements in pulmonary hemodynamics and clinical symptoms. This case suggests that rescue BPA followed by elective PEA with mitral valve plasty may benefit selected patients with CTEPH complicated by severe mitral regurgitation and decompensated right heart failure refractory to medical therapy.
Gouteng Jiangya Jieyu Prescription (GTJYJYP) shows potential in regulating blood pressure and improving mood. This study aims to investigate GTJYJYP's neuroprotective effects against neuronal damage and its underlying me...Gouteng Jiangya Jieyu Prescription (GTJYJYP) shows potential in regulating blood pressure and improving mood. This study aims to investigate GTJYJYP's neuroprotective effects against neuronal damage and its underlying mechanisms in rats with hypertension complicated by depression (HD). Forty-eight male SHR were randomly divided into SHR group, HD group, GTJYJYP low-dose (6.34 g/kg), medium-dose (12.69 g/kg), high-dose group (25.38 g/kg), and a positive control group. And eight WKY rats were served as the control group. Blood pressure, depressive-like behaviors and the contents of ROS, GSH, MDA TNF-α, IL-1β, IL-6, and IL-18 were measured. Hippocampal neuronal morphology was observed using HE and Nissl staining. Protein expression of ROS/AKT/NF-κB pathway was detected by WB. Subsequently, ROS inducer HO and AKT inhibitor MK2206 were used for intervention, and the changes of the above indicators were observed. Compared to the control group, blood pressure in both SHR and HD group rats was significantly elevated. Compared to the control and SHR groups, rats in HD group exhibited depressive-like behavior significantly. Furthermore, the HD group showed elevated levels of TNF-α, IL-1β, IL-6, IL-18, ROS, and MDA, along with reduced GSH levels significantly. Besides, the ratios of p-NF-κB p65/NF-κB p65 and p-AKT/AKT in HD group were increased significantly. Obvious neuronal damage was observed by Nissl staining. After the treatment of different doses of GTJYJYP and the positive drug, the damage was ameliorated. The ROS/AKT/NF-κB pathway was significantly inhibited. H₂O₂ treatment reversed the protective effects of GTJYJYP, whereas MK2206 further enhanced the ameliorative effects of GTJYJYP on oxidative stress, inflammatory response, and neuronal damage. GTJYJYP ameliorates neuronal damage in rats with HD through ROS/AKT/NF-κB pathway.
Pulmonary hypertension (PH) is a progressive and life-threatening disease characterized by pulmonary vascular remodeling that leads to elevated pulmonary artery pressures, and subsequent right ventricular dysfunction. De...Pulmonary hypertension (PH) is a progressive and life-threatening disease characterized by pulmonary vascular remodeling that leads to elevated pulmonary artery pressures, and subsequent right ventricular dysfunction. Despite advances in understanding PH pathogenesis, treatment options remain limited, underscoring the need to define novel mechanisms that contribute to disease progression. Inflammation and oxidative stress are recognized drivers of pulmonary vascular injury, with extracellular superoxide dismutase (EC-SOD or ), a matrix-bound antioxidant enzyme, playing a role in multiple lung and vascular pathologies. A common human single nucleotide polymorphism (rs1799895) in the gene leads to an arginine to glycine amino acid substitution (R213G) and alters EC-SOD localization by reducing its affinity for the extracellular matrix, resulting in increased circulating but decreased lung EC-SOD content. Using a murine knock-in model of the R213G variant, we have previously demonstrated exacerbated chronic hypoxia-induced PH. In this study, we examined the impact of EC-SOD redistribution due to the R213G variant on the development of PH in the Sugen-hypoxia (SuHx) model, a more severe model of PH. We hypothesized that R213G mice would also have exaggerated hemodynamic changes, vascular remodeling, and inflammatory changes in this model. However, while SuHx increased pulmonary artery pressure and vascular remodeling in wild-type mice, the R213G variant unexpectedly attenuated SuHx-induced PH. Following SuHx, the increase in pulmonary artery pressures was attenuated in R213G mice. Early immune profiling revealed that SuHx triggered significant neutrophil and interstitial macrophage infiltration in the lungs of wild-type mice, which was markedly blunted in R213G mice. These findings suggest that the redistribution of EC-SOD into the extracellular fluid, though it lowed lung EC-SOD levels, attenuated early inflammatory responses and protected against the development of PH in SuHx. This work highlights a novel, compartment-specific role for EC-SOD in modulating immune-driven mechanisms of PH and may inform future therapeutic strategies targeting oxidative stress and inflammation in vascular disease.
Use of subcutaneous treprostinil (TRE) in children with pulmonary hypertension (PH) secondary to lung disease (group 3 PH) is not well described. We hypothesized that initiation of TRE is associated with improvement in P...Use of subcutaneous treprostinil (TRE) in children with pulmonary hypertension (PH) secondary to lung disease (group 3 PH) is not well described. We hypothesized that initiation of TRE is associated with improvement in PH severity and right ventricular (RV) function with minimal early side effects. We conducted a single-center retrospective analysis of children with group 3 PH treated with TRE between 2006 and 2022. We compared echocardiographic changes in PH severity and RV systolic function over the first 3-6 months of TRE and described adverse effects over the first 48 h of therapy. Forty-one patients (56% male), aged 6 days to 15 years old at the time of TRE initiation, were included. The most common associated diagnoses were bronchopulmonary dysplasia (63%), congenital diaphragmatic hernia (10%), and pulmonary hypoplasia (10%). TRE was associated with improvement in PH severity ( < 0.001), estimated by interventricular septal position at end-systole ( = 0.001), RV systolic pressure to systemic blood pressure ratio ( < 0.001), and flow direction across a simple shunt ( = 0.153). There were improvements in individual markers of RV function including tricuspid annular plane systolic excursion ( = 0.005), tricuspid annulus dilation ( = 0.004), RV global longitudinal strain ( < 0.001), RV free wall longitudinal strain ( < 0.001), and qualitative RV systolic function ( = 0.002). Over the first 48 h of TRE, the dose was reduced in 3 patients (7%) due to hypotension, hypoxemia, or emesis, all of whom tolerated re-escalation within 48 h. TRE is well tolerated and is associated with early improvement in PH severity and RV function in children with group 3 PH.
Pulmonary hypertension is a known complication of scimitar syndrome; however, its risk factors and outcomes are not well understood. This study aimed to assess the prevalence, clinical features, risk factors, and outcome...Pulmonary hypertension is a known complication of scimitar syndrome; however, its risk factors and outcomes are not well understood. This study aimed to assess the prevalence, clinical features, risk factors, and outcomes of pulmonary hypertension in patients with scimitar syndrome. In this retrospective cohort study, patients diagnosed with scimitar syndrome and treated at a tertiary center between June 2015 and June 2024 were reviewed. Pulmonary hypertension was defined as a mean pulmonary arterial pressure > 20 mmHg based on cardiac catheterization. PH developed in 77.5% of pediatric SS patients. PVR and PCWP differed significantly across hemodynamic characteristics ( = 0.038) ( = 0.001), respectively with 67.7% of PH patients exhibiting a pre-capillary phenotype. Patients selected for surgery demonstrated higher baseline severity, including greater Qp:Qs ratio (1.99 vs. 1.49, = 0.033) and more frequent ASDs (57.1% vs. 8.3%, = 0.005). Resolution was achieved in 74.2% of patients with longer resolution time found in surgical cases (median 8.1-12.3 years) compared to non-surgical cases (3.2 years). Of the 8 patients with persistent PH, 25% ( = 2) were found to have pathogenic variants associated with non-mechanical factors linked to PH in SS. PH is highly prevalent in patients with scimitar syndrome, particularly in those with infantile type and congenital heart defects. While the majority of patients achieve resolution, the extended timeline in surgical cases reflects the higher baseline severity and complexity, along with advanced vascular remodeling. 25% of persistent PH cases harbored pathogenic variants that may contribute to a biological "second hit" that is linked to therapy resistance. Further multicenter and prospective studies are warranted to expand on these findings.
Integrating routine mental health support in pulmonary hypertension care is not just a matter of efficiency but also of recognition, equity, and epistemic justice. We provide evidence supporting the Generalised Anxiety D...Integrating routine mental health support in pulmonary hypertension care is not just a matter of efficiency but also of recognition, equity, and epistemic justice. We provide evidence supporting the Generalised Anxiety Disorder-2 questionnaire and the Patient Health Questionnaire-2 as practical, psychometrically sound, and low-burden entry points for screening anxiety and depression.