ArticleThe European respiratory journal2023
Adult-onset idiopathic peripheral pulmonary artery stenosis.
Article in The European respiratory journal, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed.
- Conceptual Reconsideration of the Classification of Pulmonary Hypertension: From 5 Groups to 2 Groups.JACC. Asia · 2026Review
- Chronic thromboembolic pulmonary hypertension: towards an etiology-oriented conceptual framework.Frontiers in medicine · 2026Review
- Assessing rare disease understanding: a novel disease readiness level framework.Orphanet journal of rare diseases · 2025Article
- Why Is Pulmonary Endarterectomy Underused in Japan? Epidemiology, Genetics, and a Practical Operability Framework.Pulmonary circulation · 2025Review
- Balloon Pulmonary Angioplasty in HeterozygousJournal of the American Heart Association · 2025Article
- Medial Dissection Following Balloon Angioplasty in Peripheral Pulmonary Artery Stenosis Harbouring HomozygousCJC open · 2025Article
- CXCL12/CXCR4 pathway as a novel therapeutic target for RNF213-associated pulmonary arterial hypertension.Scientific reports · 2024Article
- Chronic thromboembolic pulmonary disease.The European respiratory journal · 2024Review
- A rare presentation of pulmonary hypertension.International journal of cardiology. Congenital heart disease · 2024Article
- Prevalence and Clinical Characteristics of HeterozygousJournal of the American Heart Association · 2024Article
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24 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPeripheral pulmonary artery stenosis (PPS) refers to stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although paediatric PPS is well described, the clinical characteristics of adult-onset idiopathic PPS have not been established. Our objectives in this study were to characterise the disease profile of adult-onset PPS.
methodsWe collected data in Japanese centres. This cohort included patients who underwent pulmonary angiography (PAG) and excluded patients with chronic thromboembolic pulmonary hypertension or Takayasu arteritis. Patient backgrounds, right heart catheterisation (RHC) findings, imaging findings and treatment profiles were collected.
results44 patients (median (interquartile range) age 39 (29-57) years; 29 females (65.9%)) with PPS were enrolled from 20 centres. In PAG, stenosis of segmental and peripheral pulmonary arteries was observed in 41 (93.2%) and 36 patients (81.8%), respectively. 35 patients (79.5%) received medications approved for pulmonary arterial hypertension (PAH) and 22 patients (50.0%) received combination therapy. 25 patients (56.8%) underwent transcatheter pulmonary angioplasty. RHC data showed improvements in both mean pulmonary arterial pressure (44
conclusionsIn this study, patients with adult-onset idiopathic PPS presented with segmental and peripheral pulmonary artery stenosis. Although patients had severe pulmonary hypertension at baseline, they showed a favourable treatment response to PAH drugs combined with transcatheter pulmonary angioplasty.
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