Pulmonary Hypertension Patients Research Articles

Elevated Lp(a) and its association with cardiac fibrosis in group II pulmonary hypertension patients.

Group II Pulmonary Hypertension (PH) secondary to Heart Failure with preserved Ejection Fraction (HFpEF) is associated with significant morbidity and mortality. Lipoprotein(a) [Lp(a)] is a novel biomarker implicated in cardiovascular pathology, yet its role in myocardial fibrosis within this population remains underexplored. This study investigates the association between elevated Lp(a) levels and cardiac fibrosis to improve understanding of its prognostic and diagnostic utility. This retrospective cohort study included 100 patients with Group II PH secondary to HFpEF. Serum Lp(a) levels were quantified using enzymatic assays, and myocardial fibrosis was assessed using Cardiac Magnetic Resonance Imaging (CMR) techniques, including T1 mapping and late gadolinium enhancement (LGE). Statistical models adjusted for confounding factors. Elevated Lp(a) levels were significantly associated with increased myocardial extracellular volume (31% vs. 27%, p < 0.01), prolonged native T1 times, and increased odds of myocardial scar formation. Structural cardiac changes correlated with Lp(a) concentrations. Elevated Lp(a) is a key marker of myocardial fibrosis and structural remodeling in Group II PH secondary to HFpEF. Routine Lp(a) measurement may enhance risk stratification and inform therapeutic strategies.

Prognosis of pulmonary hypertension in patients with hypertrophic cardiomyopathy: A multicenter propensity score matching study

Prognosis of pulmonary hypertension in patients with hypertrophic cardiomyopathy: A multicenter propensity score matching study

Differentiation of lung tissue-resident c-Kit+ cells into microvascular endothelial cells alleviates pulmonary vascular remodeling.

Pulmonary vascular remodeling (PVR), encompassing microvascular loss and muscularization, contributes to multiple respiratory diseases. c-Kit+ cells exhibit differentiation potential into both endothelial cells (ECs) and smooth muscle cells. The potential role of lung c-Kit+ cell differentiation in PVR, however, remains unclear. Lung c-Kit+ cells increase in pulmonary hypertension patients and in the SU5416/hypoxia (SuHx)-induced PVR mouse model. Employing genetic lineage tracing and single-cell RNA sequencing (scRNA-seq), we elucidate that lung-resident c-Kit+ cells display an aerocyte and venular endothelial differentiation in the SuHx model. Ablation of tissue-resident c-Kit+ cells exacerbates PVR. We identify an Nr2f2-expressing c-Kit+ cell subgroup, which exhibitsvenous EC differentiation and increases during PVR. Notably, the elevation of Nr2f2 in c-Kit+ cells via AAV enhances differentiation and mitigates PVR. These findings underscore the protective role of lung tissue-resident c-Kit+ cells in PVR, achieved by differentiating into mature ECs. Targeting NR2F2 expression in c-Kit+ cells emerges as a promising strategy for reversing PVR.

171 A case of pulmonary embolism management complicated by unnecessary chest tube insertion in a pulmonary hypertension patient

171 A case of pulmonary embolism management complicated by unnecessary chest tube insertion in a pulmonary hypertension patient

Correlation of right heart catheterization with simultaneous echocardiography for the assessment of pulmonary hypertension in patients with severe tricuspid regurgitation

Abstract Background In severe tricuspid regurgitation (TR), it is important to accurately measure pulmonary artery pressure because pulmonary hypertension is a poor prognostic factor in severe TR. Therefore, we sought to investigate the correlation between PASP using simultaneously measured echocardiography and right heart catheterization (RHC). Method In this observational prospective study, a total of 33 patients with severe TR were enrolled between September 2021 and May 2024. Data was obtained by simultaneously measuring echocardiography and RHC. The correlation between the PASP by echocardiography and RHC was analyzed using Pearson correlation and Bland-Altman analysis. To properly evaluate right atrial pressure (RAP), additional parameters, including systolic flow reversal of the hepatic vein and the V wave cut-off sign, were incorporated. Result PASP by RHC was strongly correlated with PASP by simultaneous measured echocardiography, with a correlation coefficient (r) of 0.87. The bias was 1.5 mmHg, and the limits of agreement (LoA) ranged from -12.5 to 15.5 mmHg. Recategorizations with systolic flow reversal on hepatic vein Doppler and V wave cut-off sign as well as the size and respiratory collapsibility of inferior vena cava provide a more accurate estimate of RAP (eRAP) by echocardiography (p = 0.004). Using this approach for eRAP, a correlation between PASP by RHC and PASP by echocardiography was more prominent (r = 0.91, LoA -9.9 ~ 13.0 mmHg). Conclusion PASP simultaneously measured by echocardiography with systolic flow reversal on hepatic vein Doppler and V wave cut-off sign was highly correlated with PASP by RHC in patients with severe TR.

Effectiveness and safety of the use of sodium nitrite in patients with hypertension and pulmonary hypertension: a scoping review.

Evidence suggests that sodium nitrite may be effective in the treatment of hypertension and pulmonary hypertension. However, its use remains debated due to safety concerns. In response, a scoping review was conducted to map current knowledge on the efficacy and safety of sodium nitrite in patients with hypertension or pulmonary hypertension, addressing the question: What evidence supports the effectiveness and safety of using sodium nitrite in these patients? The databases MEDLINE (PubMed), EMBASE, Scopus, Web of Science, and LILACS were searched for clinical studies on hypertensive patients at any disease stage without restrictions on age, sex, ethnicity, publication date, or status. The primary outcomes analyzed were the efficacy and safety of sodium nitrite. A total of nine articles were included, eight from databases and one from gray literature. Sample sizes ranged from 5 to 36 patients, with sodium nitrite doses between 240µg/kg/h and 325mg. Five studies showed positive results for treating essential hypertension. Regarding safety, only one study raised concerns about adverse effects, probably due to the high dose that was used. In pulmonary hypertension patients classified as groups 2, 3, and 5, four studies suggested sodium nitrite was effective and safe, but it was ineffective in group 1. The studies included several significant limitations that should be considered. As such, the current evidence is insufficient to definitively confirm the efficacy and safety of sodium nitrite in hypertensive patients. Further research with larger sample sizes is necessary to obtain more conclusive results.

One-minute sit-to-stand test cut-offs for risk stratification based on six-minute walk test thresholds in pulmonary hypertension.

We aimed to establish one-minute sit-to-stand test (1-min STST) cut-off values that align with the guideline-recommended six-minute walk test (6MWT) thresholds (165m and 440m) for one-year mortality risk stratification in pulmonary hypertension (PH) patients. Furthermore, we aimed to compare clinical characteristics and long-term mortality among patients stratified by these proposed 1-min STST cut-offs. All patients performed the 1-min STST and 6MWT. Receiver operating characteristics analysis was performed to determine 1-min STST cut-offs corresponding to the 6MWT thresholds. Patients were stratified into three groups based on these cut-offs. Among 114 PH patients (mean age 66±14 years, 57% female), the mean number of 1-min STST repetitions was 17±6 and the mean 6MWT distance was 354±133m. The aligning 1-min STST cut-off for the <165m 6MWT threshold was 14 repetitions (sensitivity 69%, specificity 100%) with an AUC of 0.90 (95%-CI 0.84-0.97). For the >440m threshold, 20 repetitions (sensitivity 81%, specificity 84%) aligned with an AUC of 0.85 (95%-CI 0.75-0.94). Patients performing ≤14 repetitions had worse NT-proBNP levels (p<0.001), WHO-FC (p<0.001), mPAP (p=0.050), as well as health-related quality of life (HRQoL) (p<0.001) and a higher rate of mortality than those performing ≥20 repetitions (p=0.020). The 1-min STST cut-offs aligning with the 6MWT thresholds of 165m and 440m are 14 and 20 repetitions, respectively. Patients performing ≤14 repetitions demonstrated worse clinical parameters and higher mortality rates, making the 1-min STST a potential risk stratification tool in PH patients.

Performance of the FORD Versus Other Available Models for the Noninvasive Prediction of Pulmonary Hypertension in Patients with Interstitial Lung Disease

PurposePulmonary hypertension (PH) is associated with morbidity and mortality in patients with interstitial lung disease (ILD). Several prediction models have been proposed to predict PH in ILD patients. We sought to discern how previously described prediction models perform in predicting PH in patients with ILD.MethodsPatients with ILD who completed a baseline right heart catheterization, from Inova Fairfax Hospital, Northwestern Memorial Hospital, and Asan Medical Center in Korea were enrolled. The performance of various prediction models (FORD model, the FORD calculator, the PH‐ILD Detection tool, and the mean pulmonary artery pressure prediction model) were assessed using receiver operating characteristic (ROC) curves and area under the receiver operating characteristic curve (AUROC). There were four definitions of pulmonary hypertension against which the models were evaluated.ResultsThere were a total of 192 patients with ILD, of whom 32.8% (n = 63/192) met the modified 5th world symposium on PH definition of precapillary PH. Among the models assessed, the FORD calculator had an AUROC (0.733) that was marginally highest. Subgroup analysis revealed that the FORD index had the highest AUROC (0.817) in patients with idiopathic pulmonary fibrosis, while the FORD calculator had the highest AUROC (0.751) in patients with non-IPF ILD.ConclusionThe FORD model can be used to predict group 3 PH in both IPF patients and non-IPF ILD patients. It could serve as a tool for ILD patient selection for right heart catheterization as well as an enrichment tool for clinical trials targeting the pulmonary vasculature.

Insights into balloon pulmonary angioplasty and the WHO functional class of chronic thromboembolic pulmonary hypertension patients: findings from the CTEPH AC registry.

Advances in chronic thromboembolic pulmonary hypertension (CTEPH) treatment have improved prognosis, shifting focus towards symptom management. This study aimed to identify factors influencing the World Health Organization functional class (WHO-FC) in CTEPH patients. The CTEPH AC registry is a prospective, multicenter database from 35 Japanese institutions, analyzing data from August 2018 to July 2023. We examined factors associated with achieving WHO-FC I and WHO-FC changes over time in 1,270 patients. Significant factors for WHO-FC I achievement included male sex (odds ratio: 1.86, p = 0.019), age (0.98, p = 0.007), pulmonary vasodilator use (0.51, p = 0.001), post-balloon pulmonary angioplasty (BPA) (1.93, p = 0.010), lower mean pulmonary arterial pressure (0.94, p = 0.004), and lower pulmonary vascular resistance (PVR) (0.78, p = 0.006). Multivariate analysis showed that WHO-FC improvement correlated with male sex, baseline PVR, and BPA during follow-up. WHO-FC deterioration was associated with cancer, history of pulmonary endarterectomy and/or BPA at registration, bleeding risks, and thyroid disease or hormone therapy. BPA implementation is closely linked to symptomatic improvement and achieving WHO-FC I, while symptom worsening is often associated with patient-specific, difficult-to-control conditions.

Descriptive Analysis of Oxygen Dependency and Hemodynamic Changes Following Pulmonary Thromboendarterectomy in Chronic Thromboembolic Pulmonary Hypertension Patients

Descriptive Analysis of Oxygen Dependency and Hemodynamic Changes Following Pulmonary Thromboendarterectomy in Chronic Thromboembolic Pulmonary Hypertension Patients

The prediction value of serum anion gap for short-term mortality in pulmonary hypertension patients with sepsis: a retrospective cohort study.

The relationship between anion gap (AG) and short-term mortality of pulmonary hypertension (PH) patients with sepsis in the intensive care unit (ICU) remains unclear. This study involved a retrospective analysis of incident PH patients with sepsis first admitted to the ICU in the MIMIC IV database (2008 to 2019). Short-term outcomes include in-hospital mortality and 28-day mortality. According to the AG value (17.0 mmol/L), patients were divided into high-AG and low-AG groups. The Kaplan-Meier survival curve was used to compare the cumulative survival rates of the high and low groups using the log-rank test. Multivariable Cox regression analyses were constructed to assess the relationship between AG and short-term outcomes in PH patients with sepsis. A total of 2,012 sepsis patients with PH were included. The in-hospital mortality rates (11.4%) and 28-day mortality rates (12.8%) in the high-AG group were higher than those in the low-AG group (5.0% or 7.2%, respectively; P < 0.001). The Kaplan-Meier curve showed that the in-hospital and 28-day cumulative survival rates were lower in the high-AG group than in the low-AG group (P < 0.001). The multivariable Cox regression analysis confirmed that elevated AG was an independent risk factor of in-hospital mortality, 28-day mortality, and length of stay in the ICU and hospital. The relationship between elevated AG and in-hospital mortality remains stable after subgroup analyses. Elevated serum AG is associated with increased risk-adjusted short-term mortality in PH patients with sepsis, and it may aid clinicians in identifying patients with poor prognosis as early as possible.

Clinical Outcomes Of Pulmonary Hypertension In Patients With Chronic Kidney Disease. Insight From The 2020 Nationwide Inpatient Sample Database

Clinical Outcomes Of Pulmonary Hypertension In Patients With Chronic Kidney Disease. Insight From The 2020 Nationwide Inpatient Sample Database

Right Heart Catheterization Accurately Diagnoses Pulmonary Hypertension in Patients With Interstitial Lung Disease: Results From a Long-Term Cohort Study.

Interstitial lung disease (ILD) can lead to pulmonary hypertension (ILD-PH), worsening prognosis and increasing mortality. Diagnosing ILD-PH is challenging due to the limitations of imaging methods. Right heart catheterization (RHC) is the gold standard for diagnosing PH but is limited to ILD patients considered for lung transplantation. This study assessed the usefulness of RHC in diagnosing ILD-PH in a large cohort of 105 patients followed for at least 72 months, examining hemodynamic parameters for survival analysis. We conducted an ambispective cohort study, diagnosing PH as mean pulmonary artery pressure ≥ 20 mmHg, pulmonary arterial wedge pressure < 15 mmHg, and pulmonary vascular resistance > 2 Wood units by RHC. We registered demographic, biochemical, echocardiographic, respiratory, and hemodynamic parameters for survival analyses. Using RHC, we found a PH prevalence of 84.7% among ILD patients who previously exhibited an intermediate-to-high probability of PH by echocardiography. Thirty-nine ILD-PH patients died, yielding a 5-year survival rate of 35%, whereas ILD patients without PH had a survival rate of 100%. Connective tissue disease-associated ILD and interstitial pneumonia with autoimmune features were the predominant ILD subtypes in ILD-PH patients. The ILD-PH group had worse pulmonary function, lower forced vital capacity, and more severe hypoxemia. Kaplan-Meier analyses showed significantly lower survival rates in ILD-PH patients with a 6-min walking distance < 360 m, tricuspid annular plane systolic excursion/pulmonary artery systolic pressure ratio < 0.35 mm/mmHg, venous oxygen saturation < 65%, and pulmonary artery compliance < 2.2 mm/mmHg. RHC accurately characterizes ILD-PH and provides long-term survival predictors.

Disparities in Clinical Outcomes Observed Within Electronic Health Record Data From a Statewide Cohort of Pulmonary Arterial Hypertension Patients.

Health disparities in patients with pulmonary arterial hypertension (PAH) have not been extensively reported in the United States. The aim of this project was to characterize the extent of demographic and socioeconomic disparities in clinical outcomes within a large, diverse PAH patient population. A retrospective, population-based study of electronic health record data from the OneFlorida Data Trust was completed. Adult patients seeking care within one of the 12 OneFlorida Network partner healthcare systems with a documented diagnosis of any form of pulmonary hypertension (PH), including PAH, via ICD-10 code were included. Social deprivation index and healthcare provider access scores were calculated from population-based centroids derived from patient home addresses. The primary outcome was all-cause mortality, with secondary outcomes including hospitalization, emergency department (ED) visits, and similar clinical outcomes in a combined cohort of patients with other forms PH. A total of 6379 patients were included in the PAH cohort, and 37,412 patients were included in the nonspecific PH cohort. PAH patients with the greatest social deprivation exhibited increased rates of ED visits and hospitalizations. Despite having similar rates of ED visits and hospitalizations compared to non-Hispanics, Hispanic PAH patients had markedly lower mortality rates. Similar associations were also observed in the combined cohort of 37,412 patients with other forms of PH. In conclusion, healthcare disparities exist in PAH outcomes across both demographic and socioeconomic boundaries. Patients identifying as Hispanic appear to have decreased rates of mortality compared to other races/ethnicities.

Consensus recommendations for diagnosis and management of pulmonary arterial hypertension patients in Egypt

Background The burden of pulmonary arterial hypertension (PAH) in Egypt is quite comparable to the global burden. Nonetheless, there is a lack of clear national guidance on the screening, treatment, and diagnosis of PAH in Egypt. This consensus aimed at summarizing and adapting to the updated guidelines for the diagnosis and treatment of PAH by combining available evidence from the Egyptian practice according to Egyptian experts’ opinions. Methods A three-step modified Delphi method was adopted to develop the present consensus, which consisted of two rounds of voting and in-between virtual meeting. Seven specialized experts from Egyptian PH expert referral centers were invited. Results The consensus provided 71 statements for the screening, diagnosis, and management of PAH, which considered the resources availability and the infrastructure of the healthcare system in Egypt. Besides, the experts highlighted several unmet medical needs pertaining to the diagnosis and management of PAH in Egypt. Conclusion There is a need to establish a national registry that covers all Egyptian PAH centers as well as standardized patients’ databases in these centers. This should be coupled with the development of screening algorithms tailored to the Egyptian setting. To develop such national screening algorithms, cost-effectiveness studies should be conducted in Egypt to better understand optimal screening frequency and the best use of algorithms. Concerning the management of PAH in Egypt, experts emphasized the role of real-world evidence in investigating the current treatment options for PAH in Egypt to improve the understanding of using these therapies in the real world. Likewise, educational programs and workshops should ensure effective physician–patient communication to promote greater patient satisfaction and higher adherence to medications.

Assessment of right ventricular hemodynamic force in pediatric pulmonary hypertension patients following initiation of oral treprostinil therapy

Assessment of right ventricular hemodynamic force in pediatric pulmonary hypertension patients following initiation of oral treprostinil therapy

Hypoxia Combined With Interleukin-17 Regulates Hypoxia-Inducible Factor-1α/Endothelial Nitric Oxide Synthase Expression in Pulmonary Artery Endothelial Cells.

The pathogenesis of chronic thromboembolic pulmonary hypertension may be multifactorial and requires further studies. We explored alterations in pulmonary artery endothelial cells under the hypoxic and elevated interleukin-17 conditions that are commonly present in patients with chronic thromboembolic pulmonary hypertension. We measured the serum interleukin-17 levels in 10 chronic thromboembolic pulmonary hypertension patients and 10 healthy control persons. The expressions and localisations of hypoxia-inducible factor-1α and endothelial nitric oxide synthase were detected in tissues. The levels of hypoxia-inducible factor-1α, endothelial nitric oxide synthase, nitric oxide, and reactive oxygen species in cultured pulmonary artery endothelial cells were examined under hypoxia and/or interleukin-17 treatment. The serum interleukin-17 level was increased in chronic thromboembolic pulmonary hypertension patients. Hypoxia-inducible factor-1α was increased, and endothelial nitric oxide synthase was decreased in chronic thromboembolic pulmonary hypertension pulmonary vascular tissue. After receiving the hypoxia combined with interleukin-17 treatment, pulmonary artery endothelial cells showed increased levels of hypoxia-inducible factor-1α and phospho-endothelial nitric oxide synthase (Thr495) (p = 0.001 and 0.063, respectively) and a decreased level of endothelial nitric oxide synthase (p < 0.001). In addition, the nitric oxide level was significantly decreased (p = 0.001), whereas the reactive oxygen species level was insignificantly increased in pulmonary artery endothelial cells. Chronic thromboembolic pulmonary hypertension patients might experience increased inflammation and hypoxia due to dysregulation of the hypoxia-inducible factor-1α/endothelial nitric synthase pathway in pulmonary artery endothelial cells under inflammation and hypoxia, contributing to the pathogenesis of chronic thromboembolic pulmonary hypertension.

Treatment pathways in Finnish patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH).

Treatment patterns of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Finland are unknown. Guidelines now recommend early escalation of treatment for PAH. We evaluated how well Finnish practice follows guidelines, and how treatment initiations and outcomes are related. The pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension patients in Finland cohort includes all PAH and CTEPH patients diagnosed between 2008 and 2020 in all Finnish university hospitals. Drug therapy was analysed in patients with medical/procedural history available, and changes in the 4-tier comparative, prospective registry of newly initiated therapies for pulmonary hypertension (COMPERA) 2.0 risk score were evaluated. PAH patients (n = 268) were initially treated with monotherapy (52%) or double therapy (24%). After year 2015, double therapy use increased to 39%. PAH treatment at 1 year after diagnosis included phosphodiesterase 5 inhibitors (71%), endothelin-receptor antagonist (48%), prostacyclin analogue (7%), calcium channel blocker (12%) and selexipag (1%). 35% achieved low risk at 1 year, increasing to 44% for patients diagnosed after 2015. Those remaining at intermediate-high (IH) or high risk (H) (28%) were not treated less aggressively than others but were older, had more comorbidities, and often history of smoking. CTEPH patients (n = 189) were treated with pulmonary endarterectomy (PEA) (27%), balloon pulmonary angioplasty (BPA) (11%) and medical therapy only (41%) within 1 year from diagnosis. 45% achieved low risk at 1 year. We present additional results on treatment of IH and H patients, patient characteristics preceding death, and treatment persistence. We found less treatment of PAH patients with double or triple therapies and of CTEPH patients with PEA and BPA than expected but with good results. Patients not reaching low or intermediate COMPERA 2.0 were old and had comorbidities.

Clinicolaboratory characteristics and 1-year survival among group 1 pulmonary hypertension in a university hospital

Abstract Background Pulmonary arterial hypertension is a life-threatening disorder of the pulmonary vascular system that can lead to right-sided heart failure, respiratory failure, and death. Still, more knowledge is needed to understand this complex disease. The study objective is to evaluate the clinicolaboratory characteristics of group 1 pulmonary hypertension patients and their survival in the Assiut University pulmonary hypertension unit throughout the study time. Patients and methods Consecutive patients were referred to the Assiut University Chest Department Pulmonary Hypertension unit in collaboration with the cardiology department for diagnosis and management. Patients were diagnosed as group 1 pulmonary hypertension (PH) included in the study. Patient demographic data, clinical presentation, electrocardiography, laboratory data, and invasive and noninvasive hemodynamics at presentation were recorded. The patients were followed through the study time to identify their survival. Results Out of 40 patients referred to the pulmonary hypertension unit throughout the study period, 29 patients have group 1 PH and were included. Their mean age was 44.10 ± 15.14 years, most of them were females (86.2%), 44.8% of patients presented with WHO functional class II, 48.3% of patients with FC III, and 6.9% of patients with FC IV. The mean 6 min walking distance was 256.97 ± 89.46 m. Pulmonary artery catheterization hemodynamic parameters were mean pulmonary artery pressure 52.48 ± 18.24 mmHg, pulmonary vascular resistance 13.32 ± 7.31 WU, cardiac index 2.32 ± 0.83 L/min/m2, and mixed venous oxygen saturation 59.6 ± 7%. The mean follow-up duration is 12.6 ± 8.8 months, and their survival at the end of study time was 82.35%. Serum uric acid, pulmonary vascular resistance, pulmonary artery systolic pressure, and age are predictors of disease progression and mortality. Conclusion Group 1 pulmonary hypertension is an uncommon complex disease with a difficult early diagnosis. Its clinical presentation varies from insidious breathlessness, syncope, palpitation, and obvious right heart failure. Some factors as serum uric acid, pulmonary vascular resistance, pulmonary artery systolic pressure, and age are associated with prognosis. One-year survival was 82.35%. Trial registration ClinicalTrials.gov ID: NCT0479145.

Determining pulmonary artery diameter on CT scans as basis for performing transthoracic echocardiography to screen for pulmonary hypertension in patients with pulmonary artery enlargement

Determining pulmonary artery diameter on CT scans as basis for performing transthoracic echocardiography to screen for pulmonary hypertension in patients with pulmonary artery enlargement