Pulmonary Circulation最新文献

{"title":"Pulmonary Arterial Hypertension in Latin America: Characteristics, Treatment, and Survival From the LATAM-PAH Meta-Analysis and Systematic Review.","authors":"Marcos Vinicius Fernandes Garcia, Katherine Julian, Evans R Fernández Pérez, Rogerio Souza, Tim Lahm","doi":"10.1002/pul2.70403","DOIUrl":"10.1002/pul2.70403","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) in Latin America and the Caribbean (LAC) remains poorly characterized, with limited regional data on clinical presentation, hemodynamics, treatment patterns, and outcomes. We aimed to characterize demographic features, clinical presentation, hemodynamic severity, treatment patterns, and survival outcomes of right heart catheterization-confirmed PAH cohorts across LAC. We performed a systematic review and meta-analysis of retrospective and prospective cohort studies of adults with right heart catheterization-confirmed PAH in LAC, searching six databases in English, Spanish, and Portuguese without date restrictions. Study-level aggregate data were pooled using random-effects models. Sixteen studies from eight countries (1999-2025) including 2,553 patients were analyzed. Mean age was 43.6 years and 81.5% were female. Etiologies included idiopathic PAH (40.3%), congenital heart disease-associated PAH (25.6%), connective tissue disease-associated PAH (20.6%), and schistosomiasis-associated PAH (15.3% in Brazilian cohorts). At diagnosis, 51% were in functional class III/IV, mean 6-min walk distance was 406 m, mean pulmonary arterial pressure was 55.8 mmHg, and cardiac index was 2.63 L/min/m². Treatment consisted primarily of monotherapy (56%) or dual therapy (33%). Pooled survival was 93.9% at 1 year, 87.5% at 2 years, and 83.0% at 3 years. Predominantly reflecting South American PAH care, our findings establish the first regional benchmark for PAH in LAC. Patients are younger and predominantly female, with a high burden of CHD-PAH and schistosomiasis-associated PAH. Despite delayed diagnosis and limited combination therapy access, survival is comparable to high-income registries, highlighting priorities such as earlier referral, risk assessment, expanded therapy access, and prospective registry development.</p><p><strong>Trail registration: </strong>PROSPERO CRD420251131636.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70403"},"PeriodicalIF":2.4,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13543302/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148897798","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":"Optimized Contrast-Enhanced Micro-Computed Tomography for Simultaneous Imaging of the Ductus Arteriosus and Pulmonary Arteries During the Perinatal Transition.","authors":"Elahe Alizadeh, Patricia D A Lima, Rachel E T Bentley, Ashley Y Martin, Stephen L Archer","doi":"10.1002/pul2.70396","DOIUrl":"10.1002/pul2.70396","url":null,"abstract":"<p><p>Contrast-enhanced micro-computed tomography (micro-CT) provides high-resolution, three-dimensional visualization of small vascular structures in situ, enabling detailed analysis of morphological changes in the perinatal vasculature. The ductus arteriosus (DA) connects the aorta and pulmonary artery (PA), diverting placentally oxygenated blood away from the lungs in utero. Both DA and PA undergo rapid changes in tone with the first breath during the transition from fetal to neonatal oxygen conditions. This study aimed to optimize contrast-enhanced micro-CT protocols to enable simultaneous visualization of the DA and pulmonary vasculature during this transition in fetal rabbits. Two contrast agents, an iodine-based agent (Omnipaque, 300 mg/mL) and two forms of a barium sulphate suspension, were compared to determine which optimally opacified the DA and pulmonary circulation in fetal rabbit kits (<i>n</i> = 22) from new-term New Zealand white rabbits (<i>n</i> = 4). Micro-CT imaging was performed to assess DA patency and changes in PA caliber across a wide range of vessel diameters from ~50 µm to > 1 mm under hypoxia versus normoxia in anesthetized kits. Omnipaque provided adequate opacification of the DA but limited visualization of the pulmonary vasculature. However, unfiltered 25% barium sulfate in 3% gelatin achieved robust, simultaneous visualization of the DA and pulmonary circulation. This agent optimally enhanced contrast in both large vessels and small PAs, enabling simultaneous assessment of DA constriction and pulmonary arterial vasodilation within 20 min of breathing oxygen. This study established an optimal contrast agent and micro-CT imaging strategy for post-mortem assessment of DA and pulmonary vascular morphology during the perinatal transition.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70396"},"PeriodicalIF":2.4,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13543299/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148897788","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":"Observed Weight Gain in Patients With Pulmonary Arterial Hypertension Treated With Sotatercept.","authors":"Rebecca A Greene, Darlene Tat, Allyson Sherman-Roe, Tiffaney Cayton, Jacqueline Lopes, Alexander Jorrin, James Klinger, Christopher J Mullin, Kathryn Needle, William M Oldham, Crossandra Osgood, Navneet Singh, Corey E Ventetuolo","doi":"10.1002/pul2.70391","DOIUrl":"10.1002/pul2.70391","url":null,"abstract":"<p><p>Sotatercept, an activin signaling inhibitor for pulmonary arterial hypertension (PAH), has demonstrated significant improvements in clinical outcomes though its off-target effects are still an area for discovery. Anecdotal increases in total body weight not attributable to fluid retention have been observed. Understanding the potential for weight gain and body composition changes is important as excessive weight gain in the PAH population is deleterious whereas increases in lean body mass may favorably affect exercise capacity. Therefore, we conducted a single-center retrospective observational cohort study of adult Group 1 PAH patients seen at a Pulmonary Hypertension Association-accredited Comprehensive Care Center treated with sotatercept for a minimum of 12 weeks. The primary endpoint was the proportion of patients who experienced clinically significant weight gain of ≥ 5% with sotatercept treatment. Secondary endpoints were the frequency of sotatercept dose adjustments required due to changes in body weight. Exploratory analyses were conducted to identify potential relationships between weight change and covariates of interest. Of 23 patients included, clinically significant weight gain was observed in 43.5% (<i>n</i> = 10). The median absolute change from baseline weight was 3.18 kg (Q1-Q3, -0.80-7.43 kg), and the median percent change was 4.43 (Q1-Q3, -0.78-9.01). Dosage adjustments based on dosing weight changes (increase or decrease) occurred at an incidence of 1.56 per patient-year. These results suggest the importance of ongoing monitoring of body weight throughout sotatercept treatment and that weight gain is commonly associated with treatment, although not previously observed or evaluated in clinical trials to date.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70391"},"PeriodicalIF":2.4,"publicationDate":"2026-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13540266/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148887269","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":"Pressure-Derived Right Ventricular Ejection Fraction During Initial Dual Combination Therapy in Pulmonary Arterial Hypertension.","authors":"Tharsiga Kanagasingam, Jonas Lund, Christoph R Sinning, Gunnar K Lund, Hans Klose, Lars Harbaum","doi":"10.1002/pul2.70394","DOIUrl":"10.1002/pul2.70394","url":null,"abstract":"<p><p>In patients with pulmonary arterial hypertension, pressure-based right ventricular ejection fraction (RVEF) estimated from routine right heart catheterisation correlated with RVEF from magnetic resonance imaging. A framework combining Weibull distribution-derived theoretical isovolumic pressure and time-varying elastance-derived end-systolic pressure showed the closest correlation and was responsive to initial oral combination therapy.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70394"},"PeriodicalIF":2.4,"publicationDate":"2026-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13540265/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148887217","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":"Pulmonary Function and Exercise-Based Assessments for the Detection of Pulmonary Hypertension in Fibrotic Interstitial Lung Disease.","authors":"Vaida Averjanovaitė, Lina Gumbienė, Diana Zakarkaitė, Monika Laukytė-Slėnienė, Eglė Grigonienė, Virginija Rudienė, Taida Ivanauskienė, Virginija Šileikienė","doi":"10.1002/pul2.70402","DOIUrl":"10.1002/pul2.70402","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a frequent complication of fibrotic interstitial lung diseases (ILDs), but its detection remains challenging due to overlapping clinical features. We aimed to evaluate the discriminatory power of physiological and exercise-based markers that differentiate right heart catheterization (RHC)-confirmed PH from low-probability ILD cases. In this prospective observational study of 59 patients with ILD, we evaluated pulmonary function tests (PFTs), cardiopulmonary exercise testing (CPET), and the 6-min walk test (6MWT). Patients deemed at high probability for clinically relevant PH following a multidisciplinary assessment were referred for RHC. Receiver operating characteristic (ROC) analysis was used to compare RHC-confirmed PH cases (<i>n</i> = 18) with a clinically low-probability ILD cohort (<i>n</i> = 19). Patients with RHC-confirmed PH had significantly lower diffusing capacity for carbon monoxide (DLCO) (43.44 ± 13.73% vs. 59.11 ± 13.50%, <i>p</i> = 0.002) and higher ventilatory inefficiency, as measured by the VE/VCO₂ slope (47.00 ± 7.90 vs. 40.71 ± 8.48, <i>p</i> = 0.035). DLCO showed moderate discriminatory performance (AUC 0.799; optimal cut-off 50.5% predicted; sensitivity 72%; specificity 72%), whereas VE/VCO₂ slope showed fair discrimination (AUC 0.711; cut-off ≈ 41.5; sensitivity 75%; specificity 59%). Although 6MWT distance did not differentiate the groups, post-exercise oxygen desaturation and heart rate were significant indicators. In fibrotic ILD, gas exchange and ventilatory inefficiency parameters-specifically DLCO and VE/VCO₂ slope-effectively discriminate RHC-confirmed PH from clinically low-probability cases. These markers offer a practical screening approach to identify patients who should be prioritized for further PH testing.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70402"},"PeriodicalIF":2.4,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13536034/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148881447","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":"Diffusing Capacity of the Lung for Carbon Monoxide and the Risk of All-Cause Mortality in Patients With Pulmonary Hypertension Caused by Fibrosing Mediastinitis: A Multicenter Cohort Study.","authors":"Mengyu Wu, Hongling Su, Xuechun Sun, Sugang Gong, Wei Ding, Yuhong Li, Yuting Li, Wenjie Dong, Bowen Jin, Liming Zhao, Qian Jiang, Xincao Tao, Ou Xu, Wande Yu, Jie Cheng, Xintian Cai, Yunshan Cao","doi":"10.1002/pul2.70401","DOIUrl":"10.1002/pul2.70401","url":null,"abstract":"<p><p>Pulmonary hypertension caused by fibrosing mediastinitis (PH-FM) is a high-risk condition with limited validated tools for prognostic assessment. Although diffusing capacity of the lung for carbon monoxide (DLCO) predicts outcomes in several cardiopulmonary diseases, its prognostic value in PH-FM remains unclear. In this multicenter retrospective cohort study, we included 106 patients with confirmed PH-FM from 14 centers across China. The primary endpoint was all-cause mortality. Cox proportional hazards models were used to assess the association between baseline percent-predicted DLCO and mortality, with sequential adjustment for clinical, hemodynamic, and spirometric variables. The final cohort included 106 patients, of whom 60 had DLCO ≥ 60% predicted and 46 had DLCO < 60% predicted. Over a median follow-up of 36 months (interquartile range [IQR], 14.0-50.8), 27 deaths occurred. Across Cox models, each 10-percentage-point increase in percent-predicted DLCO was associated with a 30% lower risk of all-cause mortality. DLCO < 60% predicted was associated with higher all-cause mortality compared with DLCO ≥ 60% predicted, with hazard ratios ranging from 6.90 to 7.80 across models. Lower baseline percent-predicted DLCO was independently associated with a higher risk of all-cause mortality in patients with PH-FM. DLCO may provide a simple, noninvasive measure to complement current risk assessment in this population.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70401"},"PeriodicalIF":2.4,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13535974/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148881410","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":"Pulmonary Hypertension Molecular Switch: HIF Signaling Pathway.","authors":"Yinhui Sun, Rongzhen Ding, Xianli Su, Li Qin, Lihuai Wang, Jian Yi, Ruicheng Hu, Shuying You, Zhenfei Tan, Aiguo Dai","doi":"10.1002/pul2.70385","DOIUrl":"10.1002/pul2.70385","url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is a multifaceted disorder characterized by the crucial involvement of activated hypoxia-inducible factors (HIFs). Recent investigations have unveiled HIFs' ability to orchestrate a wide array of signaling cascades, encompassing voltage-gated potassium channels, non-selective cation channels (NSCCs), Ca²⁺/PKC/MAPKs, PI3K/Akt, RhoA/ROCK, Notch, VEGF, and miRNA pathways. The interplay of these signaling cascades drives critical events, namely hypoxic pulmonary vasoconstriction (HPV) and hypoxic pulmonary vascular remodeling (HPVR), which collectively contribute to the pathogenesis of PH. This review summarizes the role of HIF signaling in hypoxic pulmonary vasoconstriction, pulmonary vascular remodeling, metabolic reprogramming, inflammation, non-coding RNA regulation, and emerging HIF-targeted therapies.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70385"},"PeriodicalIF":2.4,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13536003/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148881443","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":"Lung Injury After Balloon Pulmonary Angioplasty for CTEPH: Histopathologic Findings and Management Implications.","authors":"Zachary L Steinberg, Tara Krishnan, Lia M Barros, Jonathan Buber, Kelly D Smith, Michael S Mulligan, Peter J Leary, Samuel G Rayner","doi":"10.1002/pul2.70390","DOIUrl":"10.1002/pul2.70390","url":null,"abstract":"<p><p>Balloon pulmonary angioplasty (BPA) has emerged as a guideline-recommended therapy for surgically inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Lung injury occurs in approximately 6% of BPA sessions and is the leading cause of procedure-related mortality. While lung injury post-BPA has recently been associated with vascular injury and hemorrhage, pathologic evidence is lacking regarding whether injury is uniformly hemorrhagic or whether a permeability-mediated pattern, such as seen in reperfusion edema, predominates in some cases. We present a case of severe BPA-associated lung injury resulting in mortality, report the first histopathologic characterization of the parenchymal injury pattern in this setting, and describe how these findings informed successful rescue veno-arterial extracorporeal membrane oxygenation (VA-ECMO) in a second case.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70390"},"PeriodicalIF":2.4,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13528235/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148865698","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":"Pulmonary Arterial Hypertension With Moyamoya Disease Harboring the <i>RNF213 p.Arg4810Lys</i> Variant Presenting a Favorable Response to Monotherapy: A Case Report.","authors":"Atsushi Kuraishi, Takatoyo Kiko, Shinya Fujisaki, Ryo Takano, Hiroya Hayashi, Ryotaro Asano, Eika Hamano, Hiroharu Kataoka, Takeshi Ogo","doi":"10.1002/pul2.70389","DOIUrl":"10.1002/pul2.70389","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) harboring the heterozygous <i>ring finger protein 213 (RNF213) p.Arg4810Lys</i> variant responds poorly to PAH treatment and has a poor prognosis. Here, we present a case of severe PAH and moyamoya disease (MMD) harboring a heterozygous <i>RNF213 p.Arg4810Lys</i> variant that responded well to PAH monotherapy. A 23-year-old woman with cerebral infarction due to MMD was referred to our center for MMD bypass surgery and treatment of severe PAH. Genetic analysis revealed a heterozygous <i>RNF213 p.Arg4810Lys</i> variant. We initiated PAH-targeted therapy before MMD bypass surgery, as bypass surgery was considered high-risk in the presence of severe PAH. Despite a positive response to acute vasoreactivity testing with nitric oxide (NO) inhalation, calcium channel blockers were avoided due to the risk of hypotension-induced cerebral ischemia. We initiated endothelin receptor antagonist (macitentan, 10 mg/day) monotherapy because upfront combination therapy may also cause hypotension. Endothelin receptor antagonist monotherapy improved symptoms and pulmonary hemodynamics, reducing vascular resistance from 11.4 to 7.1 Wood Units after 1 month. Bilateral cerebral bypass surgery was performed after hemodynamic improvement. The clinical spectrum of RNF213-associated PAH may be broader than previously reported. This case highlights the importance of personalized medicine that considers patient conditions and comorbidities when selecting treatment options for patients with PAH harboring the <i>RNF213 p.Arg4810Lys</i> variant.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70389"},"PeriodicalIF":2.4,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13508145/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148832060","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":"The Epidemiology of Pulmonary Hypertension in Sub-Saharan Africa-A Systematic Report.","authors":"Katarina Zeder, Lanjing Wang, Veranyuy Ngah, Armella Santi, Eric W Robbins, Ana O Mocumbi, Judith Namuyonga, Peter Nyasulu, Gabor Kovacs, Marc Humbert, Geoff Strange, Simon Stewart, Guoqing Diao, Bradley A Maron","doi":"10.1002/pul2.70388","DOIUrl":"10.1002/pul2.70388","url":null,"abstract":"<p><p>Noncommunicable diseases (NCDs) are on the rise in sub-Saharan Africa (SSA), with cardiovascular NCD accounting for most deaths. Pulmonary hypertension (PH) is a highly morbid NCD; however, contemporary prevalence data are lacking. We performed a systematic literature search of all echocardiographic or right heart catheterization studies performed in SSA. We grouped studies based on disease populations and performed random-effects meta-analyses, meta-regression, and extrapolation analyses. We included <i>n</i> = 105 reports, comprising 23,384 individuals. Most investigated cohorts included sickle cell disease (SCD; <i>n</i> = 24), human immunodeficiency virus (HIV; <i>n</i> = 19), rheumatic heart disease (RHD; <i>n</i> = 18), hypertensive heart failure (HHF; <i>n</i> = 7), and other heart failure (HF) syndromes (<i>n</i> = 18), while others, including chronic obstructive pulmonary disease (COPD), pulmonary tuberculosis (TB), chronic kidney disease (CKD) and schistosomiasis were underreported and prevalence of PH in patients with specific PH-predisposing diseases from other global regions was used. Estimated PH prevalence was strongly dependent on tricuspid regurgitation velocity (TRV) threshold, referral reason for echocardiography (HIV), and disease severity (RHD, HHF, HF). When taking these factors into account, extrapolation suggests that up to 1.2% may had \"likely\" PH TRV > 3.4 m/s) and 1.9% \"at risk\" PH (TRV > 2.8 m/s) in SSA in 2021, with the largest contributor being RHD (31%), followed by other HF (25%), COPD (18%), CKD (14%), HIV (5%), SCD (4%), schistosomiasis (2%), and TB (1%). PH has a unique epidemiological profile in SSA and based on our extrapolation methods PH prevalence is substantial but varies by disease population, disease severity, and specificity of diagnostic criteria. Population health initiatives focusing on PH prevention, detection, and management in SSA are warranted. <b>Trial Registration:</b> PROSPERO: CRD42023478199.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"16 3","pages":"e70388"},"PeriodicalIF":2.4,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13508142/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148832103","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}