In one paragraphArticle in American journal of respiratory and critical care medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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What it found
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2 · The registryThe trial behind it
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3 · Its place in the literatureWho cites it
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4 · The recordCorrections and comments
5 · Who and what moneyAuthors and funding
16 authors.
Adam M AndruskaDepartment of Medicine, Pulmonary, Allergy, and Critical Care, Stanford University, Stanford, CA, United States.ORCID 0000-0001-5238-2344 Alejandro Cruz-UtrillaPulmonary Hypertension Unit, Department of Cardiology, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain.ORCID 0000-0002-3851-4037 Esther J NossentDepartment of Pulmonary Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Cardiovascular Sciences Research Institute, Amsterdam, the Netherlands.ORCID 0000-0003-3854-4137 Lucas R CelantDepartment of Pulmonary Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Cardiovascular Sciences Research Institute, Amsterdam, the Netherlands.ORCID 0000-0001-6727-7539 Issac Yiqun YangDepartment of Medical Imaging, University of Toronto, Toronto, Canada.
Raquel LynDepartment of Medicine, Pulmonary, Allergy, and Critical Care, Stanford University, Stanford, CA, United States.
Yu Kuang Julie LaiDepartment of Medicine, Pulmonary, Allergy, and Critical Care, Stanford University, Stanford, CA, United States.
Kristina KudelkoDepartment of Medicine, Pulmonary, Allergy, and Critical Care, Stanford University, Stanford, CA, United States.ORCID 0000-0003-1557-4755 Yon SungDepartment of Medicine, Pulmonary, Allergy, and Critical Care, Stanford University, Stanford, CA, United States.ORCID 0000-0001-7114-3867 Edda SpiekerkoetterDepartment of Medicine, Pulmonary, Allergy, and Critical Care, Stanford University, Stanford, CA, United States.ORCID 0000-0002-3868-5186 Vinicio De Jesus PerezDepartment of Medicine, Pulmonary, Allergy, and Critical Care, Stanford University, Stanford, CA, United States.ORCID 0000-0001-5532-8247 Harm Jan BogaardDepartment of Pulmonary Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Cardiovascular Sciences Research Institute, Amsterdam, the Netherlands.ORCID 0000-0001-5371-0346 Pilar Escribano-SubiasPulmonary Hypertension Unit, Department of Cardiology, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain.ORCID 0000-0002-6640-4839 Roham T ZamanianDepartment of Medicine, Pulmonary, Allergy, and Critical Care, Stanford University, Stanford, CA, United States.ORCID 0000-0002-0734-0994 Andrew J SweattDepartment of Medicine, Pulmonary, Allergy, and Critical Care, Stanford University, Stanford, CA, United States.ORCID 0000-0002-0952-5319 Funding
The Wnt7a/ROR2 axis in the pathogenesis of pulmonary arterial hypertensionR01HL134776 · NHLBI · STANFORD UNIVERSITY · PI DE JESUS PEREZ, VINICIO A · 2017 to 2025
$5.0MEndothelial-pericyte interactions in the pathogenesis of pulmonary arterial hypertensionR01HL139664 · NHLBI · STANFORD UNIVERSITY · PI DE JESUS PEREZ, VINICIO A · 2018 to 2025
$4.7MUnderstanding and targeting molecular and cellular events responsible for pulmonary arteriovenous malformation development, growth and regressionR01HL169787 · NHLBI · STANFORD UNIVERSITY · PI Edda Frauke Spiekerkoetter · 2023 to 2026
$2.8MDefining the cellular and molecular mechanisms driving neointimal lesion growth in pulmonary hypertensionR01HL163013 · NHLBI · STANFORD UNIVERSITY · PI KUMAR, MAYA ELISE · 2022 to 2025
$2.7MLipid Peroxidation-Induced Mitochondrial Injury Inhibits Vascular Function in Single Ventricle Congenital Heart DiseaseR01HL165245 · NHLBI · STANFORD UNIVERSITY · PI Sushma Reddy · 2023 to 2026
$2.6MPericytes and postnatal alveolarization: Role of hypoxia inducible factorsR01HL160018 · NHLBI · STANFORD UNIVERSITY · PI ALVIRA, CRISTINA MARIA, CORNFIELD, DAVID N. · 2022 to 2025
$2.6MWhat triggers RV Fiber Re-Orientation in response to RV pressure overload, and what is its Consequence on Inter-Ventricular Decoupling?R01HL152250 · NHLBI · UNIVERSITY OF COLORADO DENVER · PI Vitaly Kheyfets · 2023 to 2026
$1.9MUnderstanding and targeting molecular as well as structural events governing right ventricular adaptation, failure and recovery in pulmonary hypertension using repurposed drugsR01HL158868 · NHLBI · STANFORD UNIVERSITY · PI SPIEKERKOETTER, EDDA FRAUKE · 2021 to 2024
$1.7MThe Role of the CES1 in the Pathogenesis of Pulmonary Arterial HypertensionR01HL172449 · NHLBI · STANFORD UNIVERSITY · PI VINICIO A DE JESUS PEREZ · 2024 to 2026
$1.6MReclassifying Pulmonary Arterial Hypertension Into Immune Phenotypes Using Machine LearningK23HL151892 · NHLBI · STANFORD UNIVERSITY · PI SWEATT, ANDREW JOHN · 2020 to 2024
$942kInstituto de Salud Carlos III PI21/01690Ministry of Science, Innovation and UniversitiesNHLBI NIH HHS 1R01HL172449-01NHLBI NIH HHS 5K23-HL151892NHLBI NIH HHS K23 HL151892NHLBI NIH HHS R01 HL134776NHLBI NIH HHS R01 HL139664NHLBI NIH HHS R01HL139664NHLBI NIH HHS R01 HL152250NHLBI NIH HHS R01HL152250NHLBI NIH HHS R01 HL158868NHLBI NIH HHS R01HL158868NHLBI NIH HHS R01 HL160018NHLBI NIH HHS R01HL160018NHLBI NIH HHS R01 HL163013NHLBI NIH HHS R01HL163013NHLBI NIH HHS R01 HL165245NHLBI NIH HHS R01HL165245NHLBI NIH HHS R01 HL169787NHLBI NIH HHS R01HL169787NHLBI NIH HHS R01 HL172449NHLBI NIH HHS R01HL59886NIH
6 · The paper itselfAbstract
rationaleWhile therapeutic advances have improved survival for patients with Group 1 pulmonary arterial hypertension (PAH), patients with Group 1.5 "PAH with features of venous/capillary involvement" (formerly pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis, now collectively termed PVOD/PCH) remain underrecognized, develop serious complications from usual PAH therapy titrations, and suffer high mortality awaiting necessary lung transplant. Identifying PVOD/PCH early before therapy initiation could aid management and expedite transplant referral.
objectivesWe aimed to develop a likelihood score distinguishing PVOD/PCH from other forms of PAH using clinical variables.
methodsDue to low PVOD/PCH prevalence and no dedicated registries, we leveraged published case-control/case-series studies to assess the ability of several clinical variables to discriminate PVOD/PCH from PAH. From pooled literature-derived data, we performed sensitivity/specificity and simulation-based receiver operating characteristic (ROC) analyses to estimate variable performance. Top-performing variables formed a PVOD/PCH likelihood score, which had its accuracy tested for distinguishing histopathology-confirmed PVOD/PCH cases (n = 37) from PAH controls (n = 60) in 3 cohorts of transplant-eligible patients from the United States, Spain, and the Netherlands. MEASUREMENTS AND MAIN
resultsDLCO, 6-minute walk desaturation, PaO2, sex, smoking history, computed tomography (CT) septal line thickening, and CT lymphadenopathy had the highest sensitivity and specificity performance and were incorporated into the PVOD score. Across test cohorts, the score achieved a ROC area under the curve of 0.97 (95% CI, 0.93-1.00) for discriminating PVOD/PCH and it retained accuracy when data were missing.
conclusionsThis score could facilitate early PVOD/PCH identification in incident PAH, potentially helping expedite transplant referral and informing therapy initiation/titration decisions.
Indexed as
Hemangioma, CapillaryHypertension, PulmonaryPulmonary Arterial HypertensionPulmonary Veno-Occlusive DiseaseAdultCase-Control StudiesDiagnosis, DifferentialFemaleHumansLung TransplantationMaleMiddle AgedROC CurveSensitivity and Specificitypulmonary arterial hypertensionpulmonary capillary hemangiomatosispulmonary veno-occlusive disease
Identifiers
PMID42275164
PMCPMC13519332
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