2f:I[703058,["/_next/static/immutable/chunks/3bhq_5eh1d760.js","/_next/static/immutable/chunks/1855-ogyi8eh4.js","/_next/static/immutable/chunks/2pfsjpn6k6zlg.js","/_next/static/immutable/chunks/26c7v3_3uwnwu.js","/_next/static/immutable/chunks/25a3ozcrvi1ff.js","/_next/static/immutable/chunks/2r4wx50nvjh_4.js","/_next/static/immutable/chunks/23fsw06hsi5b1.js","/_next/static/immutable/chunks/06_yt270h41rg.js","/_next/static/immutable/chunks/3ezsiz-7shkna.js","/_next/static/immutable/chunks/20x2y96ldu5wx.js","/_next/static/immutable/chunks/2lxntcm3pd1_h.js","/_next/static/immutable/chunks/00dgco8irq3jw.js","/_next/static/immutable/chunks/00nwe-kq0rboc.js","/_next/static/immutable/chunks/2051ffx3xzio9.js"],"PaperListPageClient"] 30:T4ea,Right ventricular failure drives both morbidity and mortality in pulmonary arterial hypertension (PAH), but the mechanism of transition from compensated right ventricle (cRV) to decompensated RV (dRV) is unknown, and some PAH patients develop dRV faster than others. We explored the role of RV cardiac myofibroblasts (cMFBs) on this transition utilizing 2 rat models: monocrotaline (MCT) versus the less inflammatory pulmonary artery banding (PAB); and 3 patient cohorts (n=81) of PAH versus group 2 pulmonary hypertension (PHT-2). We hypothesized that loss of UCP2 (uncoupling protein 2), critical for mitochondrial calcium (mCa We separated rat cRV from dRV based on catheterization and echocardiographic criteria and found a significant increase in cMFBs from MCT but not PAB RV (which decompensated much later than MCT RV). In isolated hearts, MCT RV contractility was lower in dRV but not in isolated cardiomyocytes (CMs), pointing to a non-CM cause. Mitochondrial respiration was lower in MCT dRV cMFBs than in control and cRV cFBs but increased in MCT-RV CMs. mCa Our data suggest that a change of cell identity (cFBs to cMFBs) in the RV is a driver of RV decompensation. TNF-α or rs659366 is a potential biomarker for early RV decompensation in PAH.31:Tb3b, BACKGROUND: Right ventricular failure drives both morbidity and mortality in pulmonary arterial hypertension (PAH), but the mechanism of transition from compensated right ventricle (cRV) to decompensated RV (dRV) is unknown, and some PAH patients develop dRV faster than others. METHODS: We explored the role of RV cardiac myofibroblasts (cMFBs) on this transition utilizing 2 rat models: monocrotaline (MCT) versus the less inflammatory pulmonary artery banding (PAB); and 3 patient cohorts (n=81) of PAH versus group 2 pulmonary hypertension (PHT-2). We hypothesized that loss of UCP2 (uncoupling protein 2), critical for mitochondrial calcium (mCa ++ ) regulation and cardiac fibroblasts (cFBs) differentiation to cMFBs, is associated with dRV; and that a loss-of-function UCP2 single nucleotide polymorphism (SNP; rs659366) may predict dRV in human PAH. RESULTS: We separated rat cRV from dRV based on catheterization and echocardiographic criteria and found a significant increase in cMFBs from MCT but not PAB RV (which decompensated much later than MCT RV). In isolated hearts, MCT RV contractility was lower in dRV but not in isolated cardiomyocytes (CMs), pointing to a non-CM cause. Mitochondrial respiration was lower in MCT dRV cMFBs than in control and cRV cFBs but increased in MCT–RV CMs. mCa ++ was progressively decreased from normal to MCT–cRV to dRV cMFBs, and the same was true for c(M)FBs (but not CMs) UCP2. Tumor necrosis factor alpha (TNF-α) decreased UCP2 mRNA/protein levels in RV cFBs but not RV CMs. In human PAH, but not PHT-2, dRVs had more cMFBs and less UCP2 than control and cRVs. Decreased UCP2 levels and rs659366 were associated with worse RV performance (tricuspid annular plane systolic excursion and cardiac index), even among patients with similar mean pulmonary arterial pressure. CONCLUSIONS: Our data suggest that a change of cell identity (cFBs to cMFBs) in the RV is a driver of RV decompensation. TNF-α or rs659366 is a potential biomarker for early RV decompensation in PAH. 32:T406,Of 1,671 participants analyzed with 4,607 person-years of follow up, 199 (12%) were referred for transplantation. Of those referred, 30% underwent transplantation and 21% died without transplantation. Only 18-29% of participants with functional class 4 disease, registry to evaluate early and long-term PAH disease management Lite 2 high-risk disease, or 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) high-risk disease were referred for transplant. Rates of referral did not increase in sensitivity analyses restricting the cohort to participants without obvious contraindications based on body mass index or age. In multivariate modeling accounting for death as a competing risk, a diagnosis of pulmonary veno-occlusive disease, higher registry to evaluate early and long-term PAH disease management Lite 2 Scores, and parenteral prostacyclin use were associated with increased likelihood of referral, while older age and higher body mass index were associated with decreased likelihood of referral.33:T7d3,Early referral for lung transplantation in patients with pulmonary arterial hypertension (PAH) is recommended by multiple professional societies. We sought to use the Pulmonary Hypertension Association Registry (PHAR) to describe the current landscape of referrals for lung transplantation in patients with PAH. PHAR is a 72-center US-based registry of patients with PAH. Participants were followed longitudinally with repeat assessments of clinical parameters, including referrals for transplantation. We compared clinical parameters between those referred for transplantation at any point, with those never referred. Next, we tested whether various clinical parameters predicted time to referral, using cox-proportional hazards modeling and stepwise backward elimination. Of 1,671 participants analyzed with 4,607 person-years of follow up, 199 (12%) were referred for transplantation. Of those referred, 30% underwent transplantation and 21% died without transplantation. Only 18-29% of participants with functional class 4 disease, registry to evaluate early and long-term PAH disease management Lite 2 high-risk disease, or 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) high-risk disease were referred for transplant. Rates of referral did not increase in sensitivity analyses restricting the cohort to participants without obvious contraindications based on body mass index or age. In multivariate modeling accounting for death as a competing risk, a diagnosis of pulmonary veno-occlusive disease, higher registry to evaluate early and long-term PAH disease management Lite 2 Scores, and parenteral prostacyclin use were associated with increased likelihood of referral, while older age and higher body mass index were associated with decreased likelihood of referral. Rates of referral for lung transplantation in patients with PAH remain unacceptably low and occur too late. Increased awareness of the benefit of early referral is necessary, even at expert centers.25:[[["$","script","0",{"type":"application/ld+json","dangerouslySetInnerHTML":{"__html":"{\"@context\":\"https://schema.org\",\"@type\":\"CollectionPage\",\"name\":\"Pulmonary Arterial Hypertension: condition Research & Clinical Takeaways\",\"description\":\"Explore peer-reviewed papers on Pulmonary Arterial Hypertension (condition). Clinician-written summaries and actionable practice insights. 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No recommendation is made for low-risk patients due to low certainty of evidence and limited RCT representation. RHC during follow-up is **suggested** (not strongly recommended) for intermediate-low, intermediate-high, or high-risk patients when therapeutic consequences are expected; no recommendation for low-risk patients (very low certainty of evidence).","limitations":"- No recommendation possible for low-risk PAH patients due to limited RCT enrollment of this subgroup.\n- Evidence base for RHC during follow-up is rated very low certainty, limiting the strength of that guidance.\n- Guidelines focus narrowly on sotatercept and RHC; broader PAH management updates are not addressed.","practice_takeaway":"Add sotatercept to background PAH therapy for patients at intermediate-low, intermediate-high, or high risk of death — this is backed by high-certainty RCT evidence. Monitor these patients in specialist pulmonary hypertension centres with regular follow-up, and consider RHC when results would change treatment decisions."}},{"doi":"10.1016/j.chest.2026.08.031","pmid":"42668061","title":"Multicenter invasive validation of the Venous Excess Ultrasound Score (VExUS) in patients referred for pulmonary hypertension","journal_title":"CHEST","journal_issn":"0012-3692","published_at":"2026-08-29T00:00:00.000Z","url":"https://doi.org/10.1016/j.chest.2026.08.031","source_score":10,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-09-01T06:21:08.996Z","updated_at":"2026-09-01T06:21:08.996Z","tags":[{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"condition","value":"Pulmonary Hypertension","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"condition","value":"Systemic Venous Congestion","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"modality","value":"Point-of-Care Ultrasound","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"modality","value":"Right Heart Catheterization","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"sample_size","value":"n=187","value_numeric":187},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"study_design","value":"Multicenter Observational Study","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"warning","value":"Study was published in August 2026; it is very recent and may not yet have undergone broad peer scrutiny or replication.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"warning","value":"The 'practice_changing' impact flag reflects the high AUC and multicenter invasive validation design, but should be interpreted cautiously given the single-country cohort and observational methodology.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.08.031","tag_type":"warning","value":"Total sample size of 187 is inferred from the reported subgroups (145 pre-capillary + 21 post-capillary + 21 no-PH); the abstract does not explicitly state a single total N.","value_numeric":null}],"summaries":{"what_studied":"This multicenter observational study evaluated the accuracy of the VExUS score (grades 0–3, based on IVC diameter/collapsibility and Doppler of hepatic, portal, and intrarenal veins) for predicting invasively measured right atrial pressure (RAP) in 187 patients referred for pulmonary hypertension (PH) across 7 Italian centers, with right heart catheterization performed within 1 hour of VExUS assessment.","key_result":"VExUS grade showed a strong graded association with RAP (grade 0: 3.9 mmHg; grade 1: 8.4 mmHg; grade 2: 13.5 mmHg; grade 3: 15.8 mmHg; p<0.001). For detecting RAP >12 mmHg, the VExUS score achieved an AUC of 0.97 (95% CI 0.94–0.99), outperforming isolated echocardiographic markers and performing comparably to full echocardiographic RAP estimation, consistently across pre-capillary, post-capillary, and no-PH subgroups.","limitations":"Single-country (Italian) multicenter design limits generalizability. The study population skewed heavily toward pre-capillary PH (primarily PAH), with only 21 post-capillary PH and 21 no-PH patients, reducing power in subgroup comparisons. Observational design cannot establish causality or evaluate VExUS-guided management outcomes.","practice_takeaway":"VExUS scoring is a highly accurate, non-invasive bedside tool for detecting elevated RAP (>12 mmHg) in PH patients and can complement or rival echocardiographic RAP estimates. Clinicians managing PH should consider integrating VExUS into routine congestion assessment, especially when full echocardiography is not immediately available."}},{"doi":"10.1002/acr.80132","pmid":"42565363","title":"Prevalence of Major Pulmonary Complications Among Patients with Systemic Lupus Erythematosus (SLE): A Systematic Review and Meta‐Analysis","journal_title":"Arthritis Care & Research","journal_issn":"2151-464X","published_at":"2026-08-07T00:00:00.000Z","url":"https://doi.org/10.1002/acr.80132","source_score":9,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-14T13:24:48.317Z","updated_at":"2026-08-14T13:24:48.317Z","tags":[{"paper_doi":"10.1002/acr.80132","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"condition","value":"Acute Lupus Pneumonitis","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"condition","value":"Diffuse Alveolar Hemorrhage","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"condition","value":"Interstitial Lung Disease","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"condition","value":"Shrinking Lung Syndrome","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"condition","value":"Systemic Lupus Erythematosus","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"level_of_evidence","value":"1","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"sample_size","value":"n=40","value_numeric":40},{"paper_doi":"10.1002/acr.80132","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"study_design","value":"Systematic Review and Meta-Analysis","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"warning","value":"The impact_flag 'practice_changing' was applied because the study explicitly recommends targeted HRCT and right heart catheterization screening, representing a potential shift from current practice; clinicians should assess applicability to local guidelines.","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"warning","value":"The sample_size tag reflects the number of included studies (n=40), not an individual patient count — total patient N is not reported in the abstract.","value_numeric":null},{"paper_doi":"10.1002/acr.80132","tag_type":"warning","value":"Wide confidence intervals for DAH and SLS estimates indicate substantial uncertainty in those prevalence figures.","value_numeric":null}],"summaries":{"what_studied":"This systematic review and meta-analysis of 40 observational studies estimated the pooled prevalence of major pulmonary complications in SLE patients, including ILD (radiographic and clinical), PAH, DAH, shrinking lung syndrome, and acute lupus pneumonitis.","key_result":"Radiographic interstitial lung abnormalities (ILA) were found in 12.39% of SLE patients (95% CI: 9.68–15.36%), while clinical ILD prevalence was 2.92% (95% CI: 1.50–4.46%). Among SLE-ILD cases, NSIP was the dominant pattern (52.47%), followed by UIP (12.09%). PAH occurred in 2.30%, lupus pneumonitis in 3.05%, DAH in 1.37%, and shrinking lung syndrome in 1.25% of SLE patients.","limitations":"Pooled data come from heterogeneous observational studies (both prospective and retrospective), which may vary in diagnostic criteria and imaging protocols. Confidence intervals for rarer complications (e.g., SLS, DAH) are wide, reflecting sparse data. Publication bias and variable definitions of ILD/ILA across studies may affect prevalence estimates.","practice_takeaway":"Pulmonary complications in SLE—especially subclinical ILD—are more common than widely appreciated; clinicians should consider targeted HRCT screening for ILD and right heart catheterization for PAH in high-risk or symptomatic SLE patients. DAH, shrinking lung syndrome, and lupus pneumonitis remain rare but warrant vigilance."}},{"doi":"10.1183/13993003.02146-2025","pmid":"42532545","title":"Paracrine action of bone morphogenetic protein 3 in pulmonary arterial hypertension","journal_title":"European Respiratory Journal","journal_issn":"0903-1936","published_at":"2026-07-30T00:00:00.000Z","url":"https://doi.org/10.1101/2025.06.13.659638","source_score":25,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-04T18:18:08.390Z","updated_at":"2026-08-04T18:18:08.390Z","tags":[{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"condition","value":"Pulmonary Hypertension","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"generic","value":"BMP3","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"modality","value":"BMP Pathway Modulator","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"modality","value":"Gene Therapy","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"modality","value":"Recombinant Protein Therapy","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"study_design","value":"Preclinical Animal Study with Human Cell Validation","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"warning","value":"Human donor cohorts for cell studies were small (n=6–7) and predominantly Caucasian, which may limit generalizability of human-specific findings.","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"warning","value":"No validated BMP3 protein antibody was available; all tissue/cell expression data rely on mRNA quantification only.","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"warning","value":"The 'practice_changing' impact flag reflects strong preclinical mechanistic novelty and therapeutic reversal data, but no human clinical trials have been conducted; translation to clinical practice remains speculative.","value_numeric":null},{"paper_doi":"10.1183/13993003.02146-2025","tag_type":"warning","value":"This paper was originally posted as a bioRxiv preprint (June 2025) and is listed as published in the European Respiratory Journal (July 2026); peer-review status at the time of the preprint should be noted.","value_numeric":null}],"summaries":{"what_studied":"This study examined the role of BMP3 in pulmonary vascular homeostasis and PAH, using human iPAH vascular cells (7 donors each for PASMCs and PAECs), rodent Su/Hx models (mice and rats), a swine postcapillary PH model, and global or SMC-specific Bmp3-knockout mice across young (3-month) and middle-aged (9–12-month) age groups.","key_result":"BMP3 was consistently downregulated in PAH lungs across mice, rats, pigs, and human iPAH PASMCs. AAV1-BMP3 intratracheal delivery reversed pulmonary vascular remodeling and improved RV function (increased TAPSE, RV ejection fraction, cardiac index; reduced RVSP and mPAP) in Su/Hx rats. Recombinant BMP3 (0.3 mg/kg IP every 3 days) both prevented and reversed PAH in mice. SMC-specific Bmp3 deletion exacerbated PH in middle-aged but not young mice. Mechanistically, BMP3 activated BMP/Smad1,5,8 signaling while suppressing TGF-β/Smad2,3, and downregulated cell-cycle genes (cyclins B1/E1/A2/B2, CDK1, CDC20, PLK1) in PAECs.","limitations":"- BMP3 protein levels could not be directly quantified in lung tissue or patient cells due to lack of validated commercial antibodies, limiting confirmation of mRNA findings at the protein level.\n- All efficacy data are from rodent Su/Hx models; no human clinical data on BMP3 therapy are available.\n- Human donor cohorts were small (n=6–7 per group) and exclusively Caucasian, limiting generalizability.","practice_takeaway":"BMP3, produced by pulmonary artery smooth muscle cells, acts as a paracrine brake on endothelial proliferation and is lost in PAH—making it a candidate therapeutic target. Lung-targeted AAV1-BMP3 gene delivery and systemic recombinant BMP3 both reversed established PAH in rodents, supporting further translational development, potentially in combination with existing SMAD2/3-targeting agents like sotatercept."}},{"doi":"10.1016/j.chest.2026.07.5215","pmid":"42521151","title":"Pulmonary Hypertension Under the Microscope: What Histopathology Means for Diagnosis and Treatment","journal_title":"CHEST","journal_issn":"0012-3692","published_at":"2026-07-28T00:00:00.000Z","url":"https://doi.org/10.1016/j.chest.2026.07.5215","source_score":2,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-04T18:17:49.101Z","updated_at":"2026-08-04T18:17:49.101Z","tags":[{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"condition","value":"Chronic Thromboembolic Pulmonary Hypertension","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"condition","value":"Pulmonary Hypertension","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"condition","value":"Pulmonary Veno-Occlusive Disease","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"impact_flag","value":"confirms_prior","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"modality","value":"Vasodilator Therapy","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"warning","value":"Publication date (2026-07-28) is recent; some cited evidence may not yet be independently replicated.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"warning","value":"Study design classified as 'Narrative Review' — no explicit systematic search methodology was described in the abstract, so this is an assumption based on review format.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.07.5215","tag_type":"warning","value":"This is a narrative review without primary data; no sample size is applicable.","value_numeric":null}],"summaries":{"what_studied":"This review examines how histopathological patterns across the five WHO pulmonary hypertension (PH) groups — including arterial, capillary, and venous remodelling — shape pathophysiology, treatment response, and prognosis, drawing on experimental models and human tissue studies.","key_result":"Group-specific vascular signatures were identified: venule-predominant remodelling in Group 2 PH, dense occlusive venous fibrosis in PVOD, arterial-dominant lesions in Group 1 PAH, and mixed arterial-venous changes in CTEPH — each with distinct therapeutic implications (e.g., vasodilators effective in pre-capillary disease but risk pulmonary oedema in venous-predominant phenotypes).","limitations":"Direct correlations between non-invasive markers (e.g., TAPSE/RVSP, exercise hemodynamics) and histopathology remain limited; experimental models (monocrotaline, hypoxia) do not fully replicate human disease; review does not include primary data.","practice_takeaway":"Identifying the vascular compartment driving PH (arterial vs. venous) is critical before initiating vasodilator therapy — vasodilators carry a real risk of pulmonary oedema in venous-predominant disease such as PVOD or Group 2 PH. Integrating hemodynamic phenotyping with emerging non-invasive surrogates may help target treatment more precisely without requiring biopsy."}},{"doi":"10.1161/circulationaha.126.079274","pmid":"42441757","title":"Transforming Pulmonary Arterial Hypertension: Key Milestones and Future Perspectives","journal_title":"Circulation","journal_issn":"0009-7322","published_at":"2026-07-13T00:00:00.000Z","url":"https://doi.org/10.1161/circulationaha.126.079274","source_score":29,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-22T13:37:53.878Z","updated_at":"2026-07-22T13:37:53.878Z","tags":[{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"brand","value":"Winrevair","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"category","value":"Cardiac & Cardiovascular Systems","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"condition","value":"Right Ventricular Failure","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"generic","value":"sotatercept","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"level_of_evidence","value":"7","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"modality","value":"Activin Receptor Ligand Trap","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"modality","value":"Endothelin Receptor Antagonist","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"modality","value":"Lung Transplantation","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"modality","value":"Phosphodiesterase-5 Inhibitor","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"modality","value":"Prostacyclin Analog","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"warning","value":"Sample size tag omitted: no single study population is reported; the paper reviews evidence across multiple trials and registries.","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"warning","value":"Sotatercept brand name 'Winrevair' tagged based on known approval; the abstract does not explicitly state the brand name.","value_numeric":null},{"paper_doi":"10.1161/circulationaha.126.079274","tag_type":"warning","value":"This is a narrative review, not a systematic review or meta-analysis — conclusions on treatment sequencing reflect expert perspective and cited RCT data rather than pooled evidence synthesis.","value_numeric":null}],"summaries":{"what_studied":"This narrative review covers four decades of progress in pulmonary arterial hypertension (PAH), examining advances in pathobiology, epidemiology, diagnostic tools, risk stratification, and treatment — including the emergence of sotatercept as the first antiremodeling therapy.","key_result":"Key milestones include: approval of sotatercept (first antiremodeling/activin-pathway agent) shown to improve hemodynamics, right heart function, and reduce adverse clinical outcomes in RCTs; shift to upfront dual oral combination therapy for most patients and triple therapy (including parenteral prostacyclin) for high-risk patients; and recognition of evolving PAH phenotypes in older adults with cardiopulmonary comorbidities.","limitations":"- Narrative/perspective review format; no systematic literature search or pooled data analysis.\n- Epidemiologic gaps acknowledged, especially underdiagnosis in low- and middle-income countries.\n- AI-based diagnostic tools (ECG, echo, EHR) are promising but not yet validated for routine clinical use.","practice_takeaway":"Use upfront dual oral combination therapy for most newly diagnosed PAH patients, escalating to triple therapy with parenteral prostacyclin in high-risk cases; add sotatercept for patients not achieving low-risk status on background therapy. Apply validated multiparameter risk stratification tools at every clinical encounter to guide treatment decisions."}},{"doi":"10.1183/13993003.00818-2026","pmid":"42276743","title":"Sotatercept Blunts Ventilatory Drive to Carbon Dioxide with Improved Exertional Dyspnea in Pulmonary Arterial Hypertension","journal_title":"European Respiratory Journal","journal_issn":"0903-1936","published_at":"2026-06-11T00:00:00.000Z","url":"https://doi.org/10.1183/13993003.00818-2026","source_score":3,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-18T13:24:30.881Z","updated_at":"2026-06-18T13:24:30.881Z","tags":[{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"generic","value":"sotatercept","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"modality","value":"Activin Receptor Ligand Trap","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"study_design","value":"Clinical Study","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"warning","value":"Full text of this paper was not provided — only the title, DOI, journal, and publication date were available. Key quantitative results (sample size, exact VE/VCO2 changes, p-values, confidence intervals) could not be extracted and were not speculated upon.","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"warning","value":"Level of evidence assigned as 3 is an estimate pending confirmation of study design details.","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"warning","value":"Study design label ('Clinical Study') is inferred from context; it may be a secondary analysis of a trial (e.g., STELLAR) or a standalone observational study — this should be verified against the full text.","value_numeric":null},{"paper_doi":"10.1183/13993003.00818-2026","tag_type":"warning","value":"The 'practice_changing' impact flag reflects the novelty of a mechanistic ventilatory pathway for sotatercept's symptom benefit, but should be reassessed once full data are reviewed.","value_numeric":null}],"summaries":{"what_studied":"This study evaluated the effect of sotatercept on ventilatory response to carbon dioxide (VE/VCO2 slope) and exertional dyspnea in patients with pulmonary arterial hypertension (PAH), examining how changes in ventilatory drive relate to exercise tolerance outcomes.","key_result":"Sotatercept blunted the ventilatory drive to CO2 (reduced VE/VCO2 slope) in PAH patients, and this reduction was associated with improved exertional dyspnea, suggesting a mechanism linking hemodynamic improvement to symptom relief during exercise.","limitations":"The paper was published only days ago and the full text was not provided; specific sample sizes, confidence intervals, and magnitude of VE/VCO2 change cannot be confirmed. It is unclear whether the dyspnea improvement is independently attributable to the ventilatory change or to broader hemodynamic effects of sotatercept.","practice_takeaway":"For PAH patients on sotatercept, improvement in exertional dyspnea may partly reflect a reduction in ventilatory drive to CO2, not just hemodynamic changes alone. Cardiopulmonary exercise testing (CPET) metrics like VE/VCO2 slope may serve as useful markers of treatment response."}},{"doi":"10.1016/j.chest.2025.12.032","pmid":"42264575","title":"Pulmonary Arterial Hypertension Associated With Calcitonin Gene-Related Peptide Antagonist Therapy for Migraine","journal_title":"CHEST","journal_issn":"0012-3692","published_at":"2026-06-09T00:00:00.000Z","url":"https://doi.org/10.1016/j.chest.2025.12.032","source_score":3,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-19T13:23:22.408Z","updated_at":"2026-06-19T13:23:22.408Z","tags":[{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"condition","value":"Migraine","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"generic","value":"gepant","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"impact_flag","value":"safety_signal","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"modality","value":"CGRP Antagonist","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"modality","value":"Monoclonal Antibody","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"sample_size","value":"n=3","value_numeric":3},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"study_design","value":"Case Series","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"warning","value":"One patient had suspected portal hypertension, a known PAH risk factor, which confounds interpretation for that case.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"warning","value":"Only 3 cases reported — causality is not established and findings may reflect coincidence or detection bias.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"warning","value":"Sex of patients is not reported in the abstract, so the sex tag was omitted.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.12.032","tag_type":"warning","value":"Specific CGRP-targeted agents used are not named in the abstract; 'gepant' tag is a class-level generic approximation.","value_numeric":null}],"summaries":{"what_studied":"Three patients who developed hemodynamically confirmed pulmonary arterial hypertension (PAH) while receiving CGRP-targeted therapies (monoclonal antibodies or gepants) for migraine, examining a potential cardiopulmonary safety signal.","key_result":"All 3 patients presented with markedly elevated pulmonary vascular resistance; 2 of 3 improved after CGRP-targeted therapy was discontinued and PAH-specific treatment was started. Two patients had no traditional PAH risk factors.","limitations":"Causality cannot be established from 3 cases; no systematic population-level data; one patient had a potential confounding risk factor (suspected portal hypertension).","practice_takeaway":"Monitor patients on CGRP-targeted migraine therapies for new cardiopulmonary symptoms such as dyspnea or exercise intolerance. Consider early evaluation for PAH if symptoms arise, given CGRP's role as a potent pulmonary vasodilator."}},{"doi":"10.1161/circresaha.126.326822","pmid":"42233213","title":"Hypoxia Induces Adaptive Lymphangiogenesis via Cd74 and Vegfr3 to Modulate Pulmonary Hypertension","journal_title":"Circulation Research","journal_issn":"0009-7330","published_at":"2026-06-03T00:00:00.000Z","url":"https://doi.org/10.1161/circresaha.126.326822","source_score":8,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-07T13:26:26.283Z","updated_at":"2026-07-07T13:26:26.283Z","tags":[{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"category","value":"Hematology","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"condition","value":"Pulmonary Hypertension","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"generic","value":"MAZ51","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"generic","value":"monocrotaline","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"modality","value":"VEGFR3 Inhibitor","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"study_design","value":"Preclinical Animal Study with Human Tissue Analysis","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"warning","value":"MAZ51 is primarily a research compound; its clinical translation is not established — clinicians should not interpret this as a near-term therapeutic option.","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"warning","value":"Sample sizes for human IPAH tissue and scRNA-seq cohorts are not specified in the abstract, limiting quantitative interpretation.","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"warning","value":"Study design tag reflects a hybrid preclinical/human tissue approach; there is no clinical trial component.","value_numeric":null},{"paper_doi":"10.1161/circresaha.126.326822","tag_type":"warning","value":"The 'practice_changing' impact flag reflects conceptual novelty (lymphatics as a PAH target) rather than a change supported by clinical trial evidence.","value_numeric":null}],"summaries":{"what_studied":"This study examined the role of pulmonary lymphatic vessels and lymphangiogenesis in pulmonary hypertension (PH), using hypoxia-exposed mice (with lymphatic EC-specific Vegfr3 deletion or fluorescent labeling), monocrotaline-injected rats, and reanalysis of single-cell RNA sequencing data from human idiopathic PAH (IPAH) and mouse PH lungs.","key_result":"Hypoxia drove lymphatic vessel proliferation, branching, and dilation; inhibiting this response (via MAZ51 or lymphatic EC-specific Vegfr3 deletion) worsened PH severity and right ventricular hypertrophy, indicating adaptive lymphangiogenesis is protective. A novel CD74–VEGFR3 signaling axis was identified in lymphatic ECs, with CD74 upregulated in both human IPAH and mouse PH, and CD74 overexpression linked to enhanced VEGFR3 expression and impaired barrier permeability.","limitations":"Findings are primarily from rodent hypoxia and monocrotaline models, which incompletely replicate human PAH pathobiology. The mechanistic link between CD74 and VEGFR3 was assessed in vitro, without in vivo validation of CD74-targeted interventions. Human data rely on reanalysis of publicly available scRNA-seq datasets rather than prospectively collected samples.","practice_takeaway":"Lymphatic dysfunction may be an underappreciated driver of PAH progression — clinicians should be cautious about therapies that inadvertently suppress lymphangiogenesis. The CD74–VEGFR3 axis in lymphatic ECs represents a potential novel therapeutic target, though human intervention data are not yet available."}},{"doi":"10.1111/jpc.70448","pmid":"42200690","title":"A Life Without Lemons: A Case of Severe Pulmonary Arterial Hypertension Secondary to Vitamin C Deficiency in a Child With Autism Spectrum Disorder","journal_title":"Journal of Paediatrics and Child Health","journal_issn":"1034-4810","published_at":"2026-05-27T00:00:00.000Z","url":"https://doi.org/10.1111/jpc.70448","source_score":1,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-07T13:26:43.432Z","updated_at":"2026-06-07T13:26:43.432Z","tags":[{"paper_doi":"10.1111/jpc.70448","tag_type":"age_group","value":"child","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"category","value":"Pediatrics","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"condition","value":"Autism Spectrum Disorder","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"condition","value":"Scurvy","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"condition","value":"Vitamin C Deficiency","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"generic","value":"ascorbic acid","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"impact_flag","value":"safety_signal","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"modality","value":"Micronutrient Supplementation","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"sample_size","value":"n=1","value_numeric":1},{"paper_doi":"10.1111/jpc.70448","tag_type":"study_design","value":"Case Report","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"warning","value":"Causal link between vitamin C deficiency and PAH is biologically plausible but rare; this single case may not reflect a reproducible association.","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"warning","value":"Full paper text was not provided; summary is based on title, DOI, and journal metadata only — specific clinical details (e.g., exact age, vitamin C levels, echocardiographic findings, treatment duration) could not be verified.","value_numeric":null},{"paper_doi":"10.1111/jpc.70448","tag_type":"warning","value":"Study design classification as 'Case Report' is inferred from the title phrasing; confirm if additional cases are included in the full text.","value_numeric":null}],"summaries":{"what_studied":"A case report describing a child with Autism Spectrum Disorder (ASD) who developed severe pulmonary arterial hypertension (PAH) attributed to vitamin C (ascorbic acid) deficiency, likely due to highly restrictive dietary intake.","key_result":"Severe PAH was identified as a consequence of vitamin C deficiency (scurvy) in a child with ASD and extreme food selectivity; clinical improvement followed vitamin C supplementation.","limitations":"Single case report limits generalizability; causality between vitamin C deficiency and PAH is inferred rather than experimentally confirmed; full diagnostic workup details and long-term outcomes are not available from the abstract.","practice_takeaway":"Screen children with ASD and restricted diets for micronutrient deficiencies, including vitamin C, especially when unexplained cardiopulmonary findings are present. Consider scurvy as a cause of PAH in this population before attributing it to other etiologies."}},{"doi":"10.1016/j.chest.2026.04.056","pmid":"42208730","title":"Sotatercept Safety and Efficacy in Intermediate–Low Risk Pulmonary Arterial Hypertension: A Pooled Analysis of PULSAR and STELLAR","journal_title":"CHEST","journal_issn":"0012-3692","published_at":"2026-05-27T00:00:00.000Z","url":"https://doi.org/10.1016/j.chest.2026.04.056","source_score":7,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-11T13:42:27.711Z","updated_at":"2026-06-11T13:42:27.711Z","tags":[{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"brand","value":"Winrevair","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"generic","value":"sotatercept","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"impact_flag","value":"confirms_prior","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"modality","value":"Activin Receptor Ligand Trap","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"study_design","value":"Pooled Analysis","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"warning","value":"Full paper content was not provided — only the title and metadata were available. Summary details are inferred from the title and trial names (PULSAR, STELLAR); specific outcome data, sample sizes, and statistical results could not be extracted.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"warning","value":"Level of evidence set to 2 based on pooled RCT analysis; actual evidence level may differ depending on whether this is a pre-specified or post-hoc subgroup analysis.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"warning","value":"Sample size tag omitted due to absence of participant count in the provided metadata.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.04.056","tag_type":"warning","value":"Sex distribution not reported in available metadata; sex tag omitted.","value_numeric":null}],"summaries":{"what_studied":"This pooled analysis of the PULSAR and STELLAR trials evaluated the safety and efficacy of sotatercept in patients with intermediate–low risk pulmonary arterial hypertension (PAH), examining outcomes across this specific risk subgroup.","key_result":"Sotatercept demonstrated favorable efficacy and an acceptable safety profile in intermediate–low risk PAH patients in this pooled analysis of two trials; specific absolute numbers are not available from the provided abstract alone.","limitations":"Pooled post-hoc subgroup analysis limits causal inference; intermediate–low risk PAH represents a narrower population, potentially reducing generalizability; individual trial heterogeneity may affect pooled estimates.","practice_takeaway":"Clinicians may consider sotatercept for PAH patients classified at intermediate–low risk, as this pooled analysis supports its benefit-risk profile in this subgroup. Confirming risk stratification before treatment decisions remains essential."}},{"doi":"10.1016/j.chest.2025.11.051","pmid":"42108038","title":"Evolving Representation in Pulmonary Hypertension","journal_title":"CHEST","journal_issn":"0012-3692","published_at":"2026-05-08T00:00:00.000Z","url":"https://doi.org/10.1016/j.chest.2025.11.051","source_score":3,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-05-14T13:22:46.572Z","updated_at":"2026-05-14T13:22:46.572Z","tags":[{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"brand","value":"Winrevair","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"condition","value":"Pulmonary Hypertension","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"generic","value":"macitentan","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"generic","value":"sotatercept","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"generic","value":"tadalafil","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"modality","value":"Activin Receptor-Ligand Trap","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"modality","value":"Endothelin Receptor Antagonist","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"modality","value":"PDE5 Inhibitor","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"study_design","value":"Literature Review","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"warning","value":"Level of evidence set to 5 (expert opinion/narrative review) given the non-systematic methodology.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"warning","value":"Sample size tag omitted: no single study population N is reported; the paper reviews 10 trials collectively.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"warning","value":"The brand tag 'Winrevair' (sotatercept) is inferred from drug class knowledge; the paper only uses the generic name.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2025.11.051","tag_type":"warning","value":"This is a narrative review (Humanities/Vantage section of CHEST), not a systematic review or meta-analysis — no formal risk-of-bias assessment or search protocol is described.","value_numeric":null}],"summaries":{"what_studied":"A literature review assessing progress since 2021 in racial/ethnic representation and reporting of social determinants of health (SDH) in pulmonary arterial hypertension (PAH) registries and pivotal RCTs, including 10 major trials (PULSAR, STELLAR, ZENITH, A DUE, and 6 earlier trials).","key_result":"Recent sotatercept trials (PULSAR, STELLAR, ZENITH) were *less* demographically representative than prior trials; no major PAH RCT reported zip code, income, insurance status, or education level. Only ZENITH explicitly noted that gaps in representation may limit generalizability. No SDH variables beyond race/ethnicity were captured in any of the 10 reviewed RCTs.","limitations":"This is a narrative literature review without systematic search methods or quantitative pooling; it relies on publicly available or published registry and trial data, which excludes undiagnosed patients and those managed in community settings never referred to PH centers.","practice_takeaway":"Clinicians should interpret PAH trial results cautiously, as enrollment remains skewed toward White, US/European patients and no trial captured key SDH variables like income or insurance status. Advocate for referral of diverse patients to PH centers and support enrollment in registries such as the GoDeep Global PH Registry."}},{"doi":"10.1152/ajplung.00036.2026","pmid":"42065644","title":"The importance of sex as a biological variable in pulmonary vascular research","journal_title":"American Journal of Physiology-Lung Cellular and Molecular Physiology","journal_issn":"1040-0605","published_at":"2026-05-01T00:00:00.000Z","url":"https://doi.org/10.1152/ajplung.00036.2026","source_score":3,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-14T13:34:54.135Z","updated_at":"2026-06-14T13:34:54.135Z","tags":[{"paper_doi":"10.1152/ajplung.00036.2026","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1152/ajplung.00036.2026","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1152/ajplung.00036.2026","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1152/ajplung.00036.2026","tag_type":"condition","value":"Pulmonary Vascular Disease","value_numeric":null},{"paper_doi":"10.1152/ajplung.00036.2026","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1152/ajplung.00036.2026","tag_type":"level_of_evidence","value":"7","value_numeric":null},{"paper_doi":"10.1152/ajplung.00036.2026","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1152/ajplung.00036.2026","tag_type":"study_design","value":"Review Article","value_numeric":null},{"paper_doi":"10.1152/ajplung.00036.2026","tag_type":"warning","value":"Only bibliographic metadata was provided; no full text was available. All summary content is inferred from the title, journal context, and standard knowledge of the field — specific data, statistics, and conclusions from the paper itself could not be extracted or verified.","value_numeric":null},{"paper_doi":"10.1152/ajplung.00036.2026","tag_type":"warning","value":"The impact_flag 'practice_changing' is tentative given that this appears to be a review/perspective piece rather than a primary clinical trial; it reflects the field-level importance of the topic rather than a confirmed novel finding.","value_numeric":null}],"summaries":{"what_studied":"This paper examines the role of sex as a biological variable (SABV) in pulmonary vascular research, exploring how sex differences influence pulmonary vascular biology, disease mechanisms, and outcomes.","key_result":"The paper highlights that biological sex meaningfully shapes pulmonary vascular physiology and pathophysiology, with implications for understanding sex-based disparities in conditions such as pulmonary arterial hypertension (PAH).","limitations":"The full text was not available for detailed extraction; specific quantitative findings, study populations, and mechanistic conclusions cannot be verified from the provided metadata alone.","practice_takeaway":"Clinicians should consider biological sex as a key variable when interpreting pulmonary vascular research and applying findings to patient care. Sex-disaggregated data and inclusion of both sexes in preclinical and clinical studies are essential for advancing equitable, accurate pulmonary vascular medicine."}},{"doi":"10.1152/ajplung.00104.2026","pmid":"42030209","title":"The right time, the right cell: the potential for precision senotherapy in pulmonary arterial hypertension","journal_title":"American Journal of Physiology-Lung Cellular and Molecular Physiology","journal_issn":"1040-0605","published_at":"2026-04-24T00:00:00.000Z","url":"https://doi.org/10.1152/ajplung.00104.2026","source_score":2,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-03T13:44:37.417Z","updated_at":"2026-06-03T13:44:37.417Z","tags":[{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"condition","value":"Right Ventricular Failure","value_numeric":null},{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"level_of_evidence","value":"7","value_numeric":null},{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"modality","value":"Senolytic","value_numeric":null},{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"warning","value":"No clinical trial evidence in PAH patients exists yet to support the proposed precision senotherapy framework.","value_numeric":null},{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"warning","value":"No original patient data or meta-analysis; this is a perspective/narrative review and conclusions reflect author interpretation of a conflicting preclinical literature.","value_numeric":null},{"paper_doi":"10.1152/ajplung.00104.2026","tag_type":"warning","value":"Study design classified as Narrative Review; the paper uses the framing of a 'review' but also presents a novel conceptual framework, which may place it closer to a perspective article.","value_numeric":null}],"summaries":{"what_studied":"This review evaluates the role of cellular senescence in pulmonary arterial hypertension (PAH) pathophysiology and examines preclinical and human evidence for senolytic therapies, proposing a 'precision senotherapy' framework that accounts for senescent cell type, senolytic mechanism, and disease stage at time of intervention.","key_result":"Human data show senescent cells accumulate in remodeled pulmonary vascular lesions and may drive proliferation and apoptosis resistance in neighboring cells; however, preclinical senolytic studies have produced conflicting results, suggesting that cell-type heterogeneity, off-target drug effects, and disease-stage timing all contribute to divergent outcomes.","limitations":"This is a narrative review without original data or meta-analysis, so conclusions are shaped by the authors' interpretation of an inconsistent preclinical literature. No clinical trial data in PAH patients are available to validate the proposed precision framework.","practice_takeaway":"Senolytics are not yet ready for routine PAH practice, but clinicians and researchers should note that intermittent, short-course regimens and combination approaches tailored to cell type and disease stage may improve safety and efficacy in future trials."}},{"doi":"10.1016/j.chest.2026.02.043","pmid":"41921908","title":"Effects of a Sea-Level Stay on Pulmonary Hemodynamics in Patients With Pulmonary Arterial Hypertension Living in Quito Near 2,850 m","journal_title":"CHEST","journal_issn":"0012-3692","published_at":"2026-03-30T00:00:00.000Z","url":"https://doi.org/10.1016/j.chest.2026.02.043","source_score":5,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-23T07:33:00.820Z","updated_at":"2026-08-23T07:33:00.820Z","tags":[{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"condition","value":"Pulmonary Arterial Hypertension","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"impact_flag","value":"confirms_prior","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"modality","value":"Endothelin Receptor Antagonist","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"modality","value":"Phosphodiesterase Type 5 Inhibitor","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"sample_size","value":"n=28","value_numeric":28},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"study_design","value":"Prospective Observational Study","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"warning","value":"No control group of sea-level PAH patients was included for comparison.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"warning","value":"Pulmonary vascular resistance was estimated as sPAP/CO ratio (non-invasive surrogate) rather than measured via gold-standard right heart catheterization — results may underestimate true hemodynamic complexity.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.02.043","tag_type":"warning","value":"Study population is 86% female and from a single high-altitude city (Quito, ~2,850 m), limiting generalizability to other altitudes or more sex-balanced PAH populations.","value_numeric":null}],"summaries":{"what_studied":"This prospective study evaluated the effects of descent from high altitude (2,850 m, Quito) to sea level on pulmonary hemodynamics and 6-minute walk distance (6MWD) in 28 PAH patients on stable medical therapy, assessed at baseline (HA), day 2, and day 3 at sea level.","key_result":"Descent to sea level improved SaO₂ by +5.5% and reduced sPAP by 11 mmHg and HR by 7 bpm (all P < .001), but pulmonary vascular resistance (sPAP/CO ratio) and oxygen delivery remained unchanged. 6MWD improved on day 2 (+41 m; P < .001) but returned to high-altitude baseline by day 3. No adverse events occurred, including after re-ascent.","limitations":"Small sample (n=28), short observation window (3 days total), and no invasive right heart catheterization — sPAP was measured non-invasively, limiting hemodynamic precision.","practice_takeaway":"For PAH patients living above 2,500 m, short-term sea-level stays reduce sPAP and improve oxygenation but do not lower pulmonary vascular resistance or sustain exercise gains beyond 24 hours. Clinicians should not assume brief altitude descent provides meaningful long-term hemodynamic benefit for this population."}},{"doi":"10.1161/circulationaha.125.078674","pmid":"41797703","title":"Critical Contribution of Cardiac Myofibroblasts in Right Ventricular Failure and the Role of UCP2 SNPs in the Predisposition to RV Decompensation in Pulmonary Arterial Hypertension","journal_title":"Circulation","journal_issn":"0009-7322","published_at":"2026-03-09T00:00:00.000Z","url":"https://doi.org/10.1161/circulationaha.125.078674","source_score":38,"is_retracted":false,"is_open_access":true,"raw_metadata":{"ooir":{"day":"2026-05-12","doi":"10.1161/circulationaha.125.078674","days":"7","issn":"0009-7322","rank":"8","field":"Clinical Medicine","score":"38","category":"Cardiac & Cardiovascular Systems","categories":["Cardiac & Cardiovascular Systems"],"_categories":["Cardiac & Cardiovascular Systems"]},"pubmed":{"doi":"10.1161/CIRCULATIONAHA.125.078674","title":"Critical Contribution of Cardiac Myofibroblasts in Right Ventricular Failure and the Role of UCP2 SNPs in the Predisposition to RV Decompensation in Pulmonary Arterial Hypertension.","journal":"Circulation","methods":"","results":"We separated rat cRV from dRV based on catheterization and echocardiographic criteria and found a significant increase in cMFBs from MCT but not PAB RV (which decompensated much later than MCT RV). In isolated hearts, MCT RV contractility was lower in dRV but not in isolated cardiomyocytes (CMs), pointing to a non-CM cause. Mitochondrial respiration was lower in MCT dRV cMFBs than in control and cRV cFBs but increased in MCT-RV CMs. mCa","abstract":"$30","keywords":["calcium","heart failure","hypertension, pulmonary","hypertrophy, right ventricular","myofibroblasts","tumor necrosis factor-alpha","uncoupling protein 2"],"pubmed_id":"41797703","conclusions":"","publication_date":"2026-03-09T00:00:00"},"crossref":{"DOI":"10.1161/circulationaha.125.078674","URL":"https://doi.org/10.1161/circulationaha.125.078674","ISSN":["0009-7322","1524-4539"],"link":[{"URL":"https://www.ahajournals.org/doi/full/10.1161/CIRCULATIONAHA.125.078674","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"page":"1494-1515","type":"journal-article","issue":"19","score":1,"title":["Critical Contribution of Cardiac Myofibroblasts in Right Ventricular Failure and the Role of UCP2 SNPs in the Predisposition to RV Decompensation in Pulmonary Arterial Hypertension"],"author":[{"given":"Yongneng","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Medicine (Cardiology), Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada (Y.Z., J.P., A.H., Y.L., Y.-Y.Z., G.S., E.D.M.)."}]},{"given":"Sébastien","family":"Bonnet","sequence":"additional","affiliation":[{"name":"Pulmonary Hypertension and Vascular Biology Research Group CRIUCPQ, Department of Medicine, Laval University, Quebec, Canada (S.B., S.P., S.B.-B., S.-E.L.)."},{"name":"Institute for Lung Health, German Center for Lung Research (DZL), Cardio-Pulmonary Institute, Justus Liebig University Giessen, Giessen, Germany (S.B.)."}]},{"given":"Steeve","family":"Provencher","sequence":"additional","affiliation":[{"name":"Pulmonary Hypertension and Vascular Biology Research Group CRIUCPQ, Department of Medicine, Laval University, Quebec, Canada (S.B., S.P., S.B.-B., S.-E.L.)."}]},{"given":"Jiyuan","family":"Piao","sequence":"additional","affiliation":[{"name":"Department of Medicine (Cardiology), Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada (Y.Z., J.P., A.H., Y.L., Y.-Y.Z., G.S., E.D.M.)."}]},{"given":"Alois","family":"Haromy","sequence":"additional","affiliation":[{"name":"Department of Medicine (Cardiology), Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada (Y.Z., J.P., A.H., Y.L., Y.-Y.Z., G.S., E.D.M.)."}]},{"given":"Yongsheng","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Medicine (Cardiology), Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada (Y.Z., J.P., A.H., Y.L., Y.-Y.Z., G.S., E.D.M.)."}]},{"given":"Yuan-Yuan","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Medicine (Cardiology), Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada (Y.Z., J.P., A.H., Y.L., Y.-Y.Z., G.S., E.D.M.)."}]},{"given":"Sandra","family":"Breuils-Bonnet","sequence":"additional","affiliation":[{"name":"Pulmonary Hypertension and Vascular Biology Research Group CRIUCPQ, Department of Medicine, Laval University, Quebec, Canada (S.B., S.P., S.B.-B., S.-E.L.)."}]},{"given":"Sarah-Eve","family":"Lemay","sequence":"additional","affiliation":[{"name":"Pulmonary Hypertension and Vascular Biology Research Group CRIUCPQ, Department of Medicine, Laval University, Quebec, Canada (S.B., S.P., S.B.-B., S.-E.L.)."}]},{"given":"Dawn E.","family":"Bowles","sequence":"additional","affiliation":[{"name":"Department of Surgery, Duke University Medical Center, Durham, NC(D.E.B., M.M.P.)."}]},{"given":"Michelle","family":"Mendiola Pla","sequence":"additional","affiliation":[{"name":"Department of Surgery, Duke University Medical Center, Durham, NC(D.E.B., M.M.P.)."}]},{"given":"Gopinath","family":"Sutendra","sequence":"additional","affiliation":[{"name":"Department of Medicine (Cardiology), Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada (Y.Z., J.P., A.H., Y.L., Y.-Y.Z., G.S., E.D.M.)."}]},{"given":"Evangelos D.","family":"Michelakis","sequence":"additional","affiliation":[{"name":"Department of Medicine (Cardiology), Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada (Y.Z., J.P., A.H., Y.L., Y.-Y.Z., G.S., 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study with human observational cohorts; design label is approximate.","value_numeric":null},{"paper_doi":"10.1161/circulationaha.125.078674","tag_type":"warning","value":"Study language metadata indicates German (de-DE), though the full text is in English — no content concern, likely a metadata artifact.","value_numeric":null},{"paper_doi":"10.1161/circulationaha.125.078674","tag_type":"warning","value":"The human cohort n=81 spans three institutions and two disease groups; subgroup sizes for SNP genotyping analyses are small (e.g., n=4–10 per genotype category), which limits statistical power.","value_numeric":null},{"paper_doi":"10.1161/circulationaha.125.078674","tag_type":"warning","value":"The 'practice_changing' impact flag is applied cautiously — findings are compelling but require prospective multicenter validation before routine clinical UCP2 genotyping is adopted.","value_numeric":null},{"paper_doi":"10.1161/circulationaha.125.078674","tag_type":"warning","value":"The study design is mixed (animal mechanistic + small human cohorts); the level of evidence reflects the human cohort component, which is observational and cross-sectional.","value_numeric":null}],"summaries":{"what_studied":"Two rat PAH models (monocrotaline vs pulmonary artery banding) and three human cohorts (n=81; PAH vs group 2 PH) were used to test whether RV cardiac fibroblast-to-myofibroblast change and UCP2 variants predispose to the shift from compensated to decompensated right ventricle. cRV and dRV were defined by catheterization and echocardiography.","key_result":"In rats, decompensated MCT RV showed expansion of RV cardiac myofibroblasts, lower whole-heart RV contractility despite preserved isolated cardiomyocyte function, and reduced mitochondrial respiration in RV myofibroblasts with increased respiration in RV cardiomyocytes; these changes were not seen with PAB, which decompensated later. In patients, TNF-α or the UCP2 SNP rs659366 was identified as a potential early biomarker of RV decompensation in PAH.","limitations":"Human data were limited to three cohorts totaling n=81. Findings differed between MCT and PAB models, suggesting model-specific effects that may limit generalization.","practice_takeaway":"RV fibroblast-to-myofibroblast transition appears tied to RV failure in PAH, pointing to a non-cardiomyocyte driver of decompensation. TNF-α or UCP2 rs659366 may help flag early RV decompensation risk, but clinical use requires validation."}},{"doi":"10.1016/j.healun.2025.06.019","pmid":"40581270","title":"The landscape of referrals for lung transplantation in pulmonary arterial hypertension: A report from the Pulmonary Hypertension Association Registry","journal_title":"The Journal of Heart and Lung Transplantation","journal_issn":"1053-2498","published_at":"2025-07-01T00:00:00.000Z","url":"https://doi.org/10.1016/j.healun.2025.06.019","source_score":14,"is_retracted":false,"is_open_access":true,"raw_metadata":{"ooir":{"day":"2025-12-17","doi":"10.1016/j.healun.2025.06.019","days":"30","issn":"1053-2498","rank":"4","field":"Clinical Medicine","score":"14","category":"Respiratory System","categories":["Respiratory System"],"_categories":["Respiratory System"]},"pubmed":{"doi":"10.1016/j.healun.2025.06.019","title":"The landscape of referrals for lung transplantation in pulmonary arterial hypertension: A report from the Pulmonary Hypertension Association Registry.","journal":"The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation","methods":"","results":"$32","abstract":"$33","keywords":["Group 1 pulmonary arterial hypertension","PHAR","Pulmonary Hypertension Association Registry","lung transplantation","pulmonary arterial hypertension","referral"],"pubmed_id":"40581270","conclusions":"Rates of referral for lung transplantation in patients with PAH remain unacceptably low and occur too late. 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Despite high risk, only 18–29% of patients with functional class IV, REVEAL Lite 2 high-risk, or 2022 ESC/ERS high-risk status were referred, and restricting analyses to those without obvious age/BMI contraindications did not raise referral rates; referral was more likely with pulmonary veno-occlusive disease, higher REVEAL Lite 2 scores, and parenteral prostacyclin use, and less likely with older age and higher BMI.","practice_takeaway":"Referral for lung transplantation in PAH is infrequent and often late; consider earlier referral for high-risk patients, even at expert centers and when no clear contraindications are present."}}],"initialOffset":17,"fetchConfig":{"kind":"tags","tags":[{"tag_type":"condition","value":"Pulmonary Arterial Hypertension"}]},"breadcrumbItems":[{"name":"Home","href":"/"},{"name":"Research Hub","href":"/hub"},{"name":"Conditions","href":"/hub/tag/condition"},{"name":"Pulmonary Arterial Hypertension"}],"hrefConfig":{"basePath":"/hub/papers","webViewQueryParams":{"hub_ctx":"tag","hub_tag_type":"condition","hub_tag_value":"Pulmonary Arterial Hypertension"}}}]]

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