Pulmonary Circulation最新文献

{"title":"Outcomes of Multidisciplinary Care at a Chronic Thromboembolic Pulmonary Hypertension Center.","authors":"S Christopher Malaisrie, Stephen Chiu, Daniel Schimmel, Maanasi Samant, Ryan Avery, Amir Rahsepar, Bradley Allen, Yasmin Raza, Benjamin Freed, Ruben Mylvaganam, Michael J Cuttica","doi":"10.1002/pul2.70085","DOIUrl":"https://doi.org/10.1002/pul2.70085","url":null,"abstract":"<p><p>Recent international guidelines recommend a multidisciplinary evaluation and care model for patients with chronic thromboembolic pulmonary hypertension (CTEPH), but there is a paucity of supporting data. The aim of this study was to describe the outcomes of a multidisciplinary team approach to the comprehensive care of CTEPH patients. This single-center cohort study enrolled 166 consecutive adult patients undergoing CTEPH treatment evaluation from 2016 to 2022 at a tertiary care, academic regional referral and comprehensive CTEPH center with pulmonary thromboendarterectomy (PTE) and balloon pulmonary angioplasty (BPA) capabilities. Patients underwent PTE, BPA, or medical management after consensus evaluation by a multidisciplinary team including pulmonary hypertension physicians, surgeons, interventional cardiologists, and radiologists. 86% (142/166) of patients underwent interventional therapies; 100 (60%) underwent PTE and 42 (25%) BPA. Of the 24 (14%) medically treated patients, 13 patients were offered but deferred intervention; 11 patients had non-intervenable disease. 30-day mortality in both PTE and BPA was 0%. 1- and 3-year survival was 99% and 96% for PTE, 100% and 93% for BPA, 79% and 79% for medical management. Patients who underwent PTE had the best hemodynamic response (∆PVR: PTE -278.8 ± 366.9 dyne/sec/cm⁵; BPA -15.9 ± 171.8 dyne/sec/cm⁵; medical -60.2 ± 233.1 dyne/sec/cm⁵; <i>p</i> = 0.001), largest improvement in Borg Dyspnea Scale; [PTE -1.0 (-2.8 to 0.0), BPA + 0.5 (-0.8 to 5.0), medical +1.0 (0.75 to 3.0), <i>p</i> = 0.01], and most improvement in NYHA functional class [% improving at least 1 functional class: PTE 64% (47/73), BPA 18% (5/28), medical 21% (4/19), <i>p</i> = 0.0004].</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70085"},"PeriodicalIF":2.2,"publicationDate":"2025-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12011997/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144045971","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Societal Costs Associated With Pulmonary Arterial Hypertension Subgroups: A Study Utilizing Linked National Registries.","authors":"Barbro Kjellström, Bodil Ivarsson, Magnus Husberg, Lars-Åke Levin, Lars Bernfort","doi":"10.1002/pul2.70074","DOIUrl":"10.1002/pul2.70074","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a heterogenic diagnosis including idiopathic and hereditary PAH (IPAH/HPAH) and groups associated to connective tissue disease (APAH-CTD) and congenital heart disease (APAH-CHD). Pre- and post-diagnosis societal costs in PAH subgroups are not well known. By linking Swedish national databases, societal costs in a national PAH cohort 5 years before and 5 years after diagnosis were estimated and compared to an age, sex, and geographically matched control group (1:5 match). Incident patients diagnosed 2008-2019 were included (patient/control; IPAH/HPAH = 393/1965, APAH-CTD = 261/1305, APAH-CHD = 89/445). Pre-diagnosis mean societal costs were 2.9, 3.4, and 4.3 times higher for IPAH/HPAH, APAH-CTD and APAH-CHD patients, respectively, than controls. Post-diagnosis, mean costs had increased 3.1, 2.0, and 1.6 times further for IPAH/HPAH, APAH-CTD and APAH-CHD respectively, while it decreased in all control groups. Main cost driver pre-diagnosis were indirect costs (productivity loss) in both patient and control groups, however, 2.7-4.5 times higher in the patient groups. Post-diagnosis, the main cost driver for all groups were health care costs (in- and outpatient-care, drugs) that had increased 7.8, 5.4 and 6.8 times for IPAH/HPAH, APAH-CTD and APAH-CHD, respectively. Corresponding increase for controls were 17%-48%. For the PAH groups, drug treatment accounted for 70%-81% of the direct costs, while hospitalizations were the main driver for the control groups. In conclusion, PAH was associated with large societal costs. Pre-diagnosis, APAH-CHD had the highest societal costs, both in relation to their control group and compared to the other patient groups. Post-diagnosis, highest societal costs were seen in IPAH/HPAH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70074"},"PeriodicalIF":2.2,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12005055/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144012428","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Alterations in ECG and Right Heart Catheterization Data in PAH Patients Who Died From Sudden Death Compared With Right Heart Failure.","authors":"Alexandra B Flemington, Jeffery Annis, Evan L Brittain, Anna R Hemnes","doi":"10.1002/pul2.70082","DOIUrl":"10.1002/pul2.70082","url":null,"abstract":"<p><p>A meaningful number of patients with PAH die suddenly, and there is little data to understand the events surrounding sudden death in PAH. We tested the hypothesis that sudden death is associated with pre-mortem ECG or hemodynamics changes compared to those who died of RHF. We extracted data from the Vanderbilt University Medical Center Synthetic Derivative. Patients 18 years of age and older with Group 1 PAH secondary to any etiology who died between 2009 and 2017 with both ECG and RHC data from the inpatient and outpatient setting were included in the study. Continuous variables were compared using the Wilcoxon rank-sum test while categorical variables were compared using the <i>χ</i> ² test. Logistic regression models, adjusted for age and sex, were then used to evaluate the association between death and specific ECG or RHC measurements. Comparing the final ECG before death, those who died of SD had significantly shorter terminal 40 ms interval of the QRS than those who died of RHF, which became nonsignificant when adjusted for age and sex. We observed differences in baseline RHC data between SD and RHF including higher RV systolic pressure which remained significant when adjusted for age and sex. Using this data, we hope to find clinical data that can be used to predict increased risk of sudden death and aid in stratifying Group I PAH patients to earlier and more aggressive interventions.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70082"},"PeriodicalIF":2.2,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12005591/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143993762","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence and Economic Burden of Pulmonary Hypertension and Pulmonary Arterial Hypertension Among the Medicaid Population.","authors":"Marshaleen Henriques King, Chaohua Li, Vincent C Bond, Dimitri Ford, Peter Baltrus, Harrison W Farber","doi":"10.1002/pul2.70060","DOIUrl":"10.1002/pul2.70060","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is defined hemodynamically as a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg, measured at right heart catheterization (RHC). Pulmonary arterial hypertension (PAH) is defined as a mPAP ≥ 20 mmHg with a pulmonary capillary wedge pressure (PCWP) or left ventricular end-diastolic pressure (LVEDP) of ≤ 15 mmHg and a pulmonary vascular resistance (PVR) > 2 Woods Units (WU). The reported prevalence of PAH in the general population is 0.03-0.05 per 1000 population. However, several studies suggest that the prevalence may be higher among specific sub-populations. Using Medicaid Analytic Extract (MAX) files, we identified Medicaid beneficiaries who were diagnosed with PH or PAH between 2009 and 2012. The prevalence of PH and PAH was calculated for the overall study population and subgroups based on demographics or co-morbidities. We used one-way analysis of variance (ANOVA) tests to compare the differences in hospital bed days and total Medicaid cost across racial subgroups among those with PH and those without PH; Tukey post hoc tests were performed to calculate p-values for comparing White and Black subpopulations. Prevalence rates ranged between 1.7 and 1.8 per 1000 persons, and the PAH prevalence ranged between 0.4 and 0.5 per 1000 persons for the years reviewed. Significant racial/ethnic disparity in PH and PAH prevalence was observed (<i>p</i>-value < 0.001), with Black patients having the highest prevalence and Asian patients having the lowest prevalence. Prevalence of PH and PAH were noted to be higher for the Medicaid population than for the general population for all years reviewed. PH and PAH prevalence was noted to be higher among Blacks compared to Non-Hispanic Whites, while it was significantly lower in Hispanics and Asians. PH/PAH Medicaid patients were noted to account for a greater economic burden compared to the general Medicaid population. Stratifying economic burden by race revealed that American Indian and Alaska Natives with PH had the highest total Medicaid cost for all years reviewed.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70060"},"PeriodicalIF":2.2,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12006030/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143980595","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnostic Accuracy of the Malnutrition Universal Screening Tool and Mini Nutritional Assessment Short-Form in Outpatients With Pulmonary Hypertension.","authors":"E Grimbergen, S P M van Aarssen, D P Staal, J Peper, J J Mager, S Boerman, B J M Mulder, M C Post","doi":"10.1002/pul2.70075","DOIUrl":"10.1002/pul2.70075","url":null,"abstract":"<p><p>Several disease related factors of pulmonary hypertension (PH) can negatively impact the nutritional status, leading to an increased risk of malnutrition. However, there are no studies on the best method for nutritional screening in PH patients. Therefore, the aim of this study was to determine the diagnostic accuracy of two screening tools: the Malnutrition Universal Screening Tool (MUST) and the Mini Nutritional Assessment Short-Form (MNA-SF). This cross-sectional single center study included PH outpatients. Cut-off values MUST ≥ 1 and MNA-SF ≤ 11 were used for state of (risk of) malnutrition. The diagnostic criteria of the Global Leadership Initiative on Malnutrition (GLIM) were used as reference for diagnosing malnutrition. Diagnostic accuracy was determined by sensitivity, specificity, predictive positive value, negative predictive value, Cohen's Kappa- value (K) and area under the curve. Out of the 103 PH patients (age 67 years (SD 11.5), 66% female), 27% were malnourished according to the GLIM criteria. Both MUST and MNA-SF had an insufficient sensitivity (60.7% [CI: 41%-97%] vs. 64.3% [CI: 44%-81%]). The MUST had a specificity of 100% [CI: 95%-100%], PPV 100% [CI:94%-100%] and NPV 87.2% [CI:79%-93%]. The specificity of the MNA-SF was 81.3% [CI:70%-89%], PPV 56.3% [CI: 39%-73%] and NPV 85.9% [CI: 77%-93%]. The MUST had a higher K-value 0.692 and AUC (0.804) compared to the K-value 0.437 and AUC (0.728) of the MNA-SF. This study indicated that both MUST and MNA-SF are inaccurate to detect (risk of) malnutrition in PH outpatients. Future studies are needed to strive for a more sensitive screening tool.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70075"},"PeriodicalIF":2.2,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12004392/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144051018","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mas1 Receptor Activation is Necessary and Sufficient to Transduce ACE2 Effect in PAH, But Ang(1-7) Alone is Insufficient.","authors":"James West, Megha Talati, Erica Carrier, Anandharajan Rathinasabapathy, Ibragim Gaidarov, Benjamin Vigl, Ying Cai, Hongpeng Jia, Tom Blackwell, Santhi Gladson, Christie Moore, Sheila Shay, Ethan Sevier, Anna Hemnes","doi":"10.1002/pul2.70083","DOIUrl":"10.1002/pul2.70083","url":null,"abstract":"<p><p>ACE2 has shown effectiveness in treating pulmonary hypertension in multiple animal models and has some promise in early human trials. The key barrier to translation is that enzymatically active ACE2 is difficult to manufacture and exhibits a short half-life in humans, making chronic administration challenging. Understanding the mechanism of effect is thus key to finding ways to bypass ACE2 while still reproducing therapeutic effects. In this study, we test the hypotheses that ACE2 produces its therapeutic effect through increased Mas1 signaling and that Ang(1-7) is sufficient as the Mas1 ligand. We found that the ACE2 effect is blocked in Mas1 knockout mice and that the Mas1 agonist AR234960 reproduces the ACE2 effect, indicating that Mas1 activation is necessary and sufficient for the ACE2 therapeutic effect. However, neither AlbudAb-stabilized Ang(1-7) nor Ang(1-7) stabilized through the use of protease inhibitors were capable of reproducing ACE2 effectiveness, indicating that Ang(1-7) alone does not activate Mas1 in this context. RNA-seq suggests that the key mechanisms downstream of Mas1 responsible for the therapeutic effect of ACE2 and AR234960 are the rescue of cytoskeletal and microtubule defects. Together, these findings indicate that direct activation of Mas1 will likely be effective in treating pulmonary arterial hypertension, but raise the question of the identity of the endogenous ligand(s).</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70083"},"PeriodicalIF":2.2,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12005592/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144012425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Prospective Analysis of Vasoreactivity and Mortality in WHO Group 3 Pulmonary Hypertension.","authors":"Daniel J Strick, Carl Tanba, Meredith A Kaplan, Nicholas S Hill, Harrison W Farber, David Condon, Ioana R Preston","doi":"10.1002/pul2.70078","DOIUrl":"https://doi.org/10.1002/pul2.70078","url":null,"abstract":"<p><p>Prognostic markers of Group 3 pulmonary hypertension (PH) remain largely unknown. In this study, we evaluate clinical data to provide a comprehensive profile of patients with Group 3 PH and evaluate the potential use of vasoreactivity testing as a prognostic tool within this population. We hypothesized that patients with a stronger vasoconstrictive component of their pulmonary vascular disease would have a more favorable prognosis. Patients were given inhaled nitric oxide during their right heart catheterization to determine if they met the European Respiratory Society guidelines for having a positive vasoreactivity test as defined for patients with Group 1 pulmonary arterial hypertension (PAH). While vasoreactivity response is proven to predict survival in subgroups of PAH, there was no significant relationship between change in mean pulmonary artery pressure (mPAP) during acute vasodilator challenge and survival within our cohort. On the contrary, patients with larger decreases in pulmonary vascular resistance (PVR) during the acute vasodilator challenge were at a significantly higher risk of mortality. The data suggests that the change in PVR during acute vasodilator challenge may be a better indicator of survival in patients with WHO Group 3 PH than the change in mPAP.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70078"},"PeriodicalIF":2.2,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12006029/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144036396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Physical Activity, Sleep, and Quality of Life in Pulmonary Arterial Hypertension: Novel Insights From Wearable Devices.","authors":"Andrew M Hughes, Alisha Lindsey, Jeffrey Annis, Kelly Burke, Hiral Master, Luke G Silverman-Lloyd, Jonah D Garry, Michael J Blaha, Erika S Berman Rosenzweig, Robert P Frantz, Paul M Hassoun, Evelyn M Horn, Jane A Leopold, Franz P Rischard, Brett Larive, Nicholas S Hill, Serpil C Erzurum, Gerald J Beck, Anna R Hemnes, Evan L Brittain","doi":"10.1002/pul2.70069","DOIUrl":"10.1002/pul2.70069","url":null,"abstract":"<p><p>Reduced functional capacity and poor sleep quality are common in pulmonary arterial hypertension (PAH). Wearable devices are an emerging, user-friendly tool to capture activity and sleep information. We aimed to determine whether Fitbit-derived activity and sleep trends provide clinically meaningful information in patients with PAH. Our prospective observational study recruited patients with PAH from across the United States using remote enrollment strategies and in-person efforts. Participants wore a Fitbit device for 12 weeks at baseline and a subgroup with 1-year follow-up. A matched control cohort was generated from the <i>All of Us</i> Research Program and we evaluated changes in patients with PAH compared to matched controls. Among 110 patients with baseline monitoring, average daily steps correlated with 6MWD (<i>r</i> = 0.61, <i>p</i> < 0.001) and percent rapid eye movement (REM) sleep (<i>r</i> = 0.28, <i>p</i> = 0.008). In 44 PAH participants who completed baseline and 1-year monitoring, there was a group-time interaction for percent light sleep (<i>p</i> = 0.024) and percent REM sleep (<i>p</i> = 0.034), which demonstrated that sleep quality worsened in patients with PAH over 1 year compared to matched controls. Average daily steps decreased in patients with PAH from 5200 [IQR 3212-7458] at baseline to 4651 [IQR 2912-6827] at 1 year (<i>p</i> = 0.008). In conclusion, our study demonstrated the potential clinical value of wearable devices by showing that activity and sleep quality are reduced in PAH compared to matched controls and these measures decline over time. Future studies should investigate if monitoring these health behaviors detects early functional decline and whether targeted interventions may improve outcomes.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70069"},"PeriodicalIF":2.2,"publicationDate":"2025-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12003557/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144042571","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Editorial on \"Developing Benchmarks in the Diagnosis and Treatment of Pulmonary Arterial Hypertension in a Tertiary, Academic Medical Center\".","authors":"Robert P Frantz","doi":"10.1002/pul2.70081","DOIUrl":"10.1002/pul2.70081","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70081"},"PeriodicalIF":2.2,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12000534/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144043522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Importance of age at diagnosis of pulmonary hypertension in children living at high altitude: Longitudinal follow-up of 86 patients.","authors":"Gabriel F Diaz, Carlos E Diaz-Castrillon, Alicia Marquez Garcia, Rachel K Hopper, Vinicio de J Perez","doi":"10.1002/pul2.70017","DOIUrl":"10.1002/pul2.70017","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) at high altitudes presents unique characteristics due to hypobaric hypoxia. We aimed to evaluate the association between early diagnosis and clinical outcomes among children with severe PH living at high altitudes. A retrospective analysis was conducted on 86 children, divided into three age groups at the time of diagnosis: ≤3, 3-10, and ≥10 years. The median age at diagnosis was 4 years, with 48% of patients under 3 years old. Over a median follow-up of 6 years (interquartile ranges 2.5-8), 10% had a follow-up exceeding 15 years. Mortality rates were lowest in those diagnosed before age 3 (22% vs. 48% vs. 25%, <i>p</i> = 0.06). Responders to the prolonged hyperoxia test (PHT) were younger and had a significantly lower mortality hazard ratio (0.23, 95% confidence interval 0.08-0.70; <i>p</i> = 0.01). Early detection of PH in children living at high altitudes is associated with a higher likelihood of having a positive response to the PHT and subsequently, lower mortality rates. These findings underscore the importance of early diagnosis in improving long-term outcomes for this population.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 2","pages":"e70017"},"PeriodicalIF":2.2,"publicationDate":"2025-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11994476/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144049884","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}