ArticlePloS one2017
Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability.
Article in PloS one, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 27 citations in OpenAlex.
- Case Report: Shared manifestation, distinct etiologies: severe pulmonary hypertension in both mother and neonate.Frontiers in pediatrics · 2025Article
- Pulmonary vascular manifestations of hereditary haemorrhagic telangiectasia.Pulmonary circulation · 2024Review
- Hereditary hemorrhagic telangiectasia with hepatic arteriovenous shunt diagnosed due to liver damage.Clinical journal of gastroenterology · 2024Article
- Specifications of the ACMG/AMP Variant Curation Guidelines for Hereditary Hemorrhagic Telangiectasia Genes-Human mutation · 2024Article
- Long Term Survival of Heritable Pulmonary Arterial Hypertension Associated with Hereditary Hemorrhagic Telangiectasia: A Case Series.Journal of clinical medicine · 2023Article
- Pulmonary hypertension in hereditary hemorrhagic telangiectasia: A clinical review.Pulmonary circulation · 2023Review
- Cardiac and Hemodynamic Manifestations of Hereditary Hemorrhagic Telangiectasia.The International journal of angiology : official publication of the International College of Angiology, Inc · 2022Review
- Pulmonary hypertension in hereditary haemorrhagic telangiectasia is associated with multiple clinical conditions.ERJ open research · 2021Article
- Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update.Journal of clinical medicine · 2020Review
- Pulmonary Hypertension and Hereditary Hemorrhagic Telangiectasia Related to an ACVRL1 Mutation.Internal medicine (Tokyo, Japan) · 2020Article
- Pulmonary gas exchange in hereditary hemorrhagic telangiectasia patients with liver arteriovenous malformations.Respiratory research · 2019Article
- Deregulation of Drosha in the pathogenesis of hereditary hemorrhagic telangiectasia.Current opinion in hematology · 2019Review
- Diagnosis of pulmonary hypertension.The European respiratory journal · 2019Review
- Pulmonary Arterial Hypertension and Hereditary Haemorrhagic Telangiectasia.International journal of molecular sciences · 2018Review
- Urologic Surgery with Multisystem Comorbidities: A Case Report.The American journal of case reports · 2018Article
- Severe pulmonary arterial hypertension in a patient with hereditary hemorrhagic telangiectasia and multiple pulmonary and hepatic arteriovenous malformations.Annals of pediatric cardiologyArticle
- Different forms of pulmonary hypertension in a family with clinical and genetic evidence for hereditary hemorrhagic teleangectasia type 2.Pulmonary circulationArticle
Corrections and comments
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Authors and funding
18 authors at 10 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundDifferent pulmonary hypertension (PH) mechanisms are associated with hereditary haemorrhagic telangiectasia (HHT). METHODS AND
resultsWe conducted a retrospective study of all suspected cases of PH (echocardiographically estimated systolic pulmonary artery pressure [sPAP] ≥ 40 mmHg) in patients with definite HHT recorded in the French National Reference Centre for HHT database. When right heart catheterization (RHC) was performed, PH cases were confirmed and classified among the PH groups according to the European guidelines. Among 2,598 patients in the database, 110 (4.2%) had suspected PH. Forty-seven of these 110 patients had RHC: 38/47 (81%) had a confirmed diagnosis of PH. The majority of these had isolated post-capillary PH (n = 20). We identified for the first time other haemodynamic profiles: pre-capillary pulmonary arterial hypertension (PAH) cases (n = 3) with slightly raised pulmonary vascular resistances (PVR), and combined post- and pre-capillary PH cases (n = 4). Compared to controls, survival probability was lower in patients with PAH.
conclusionThis study revealed the diversity of PH mechanisms in HHT. The description of combined post- and pre-capillary PH with/or without high cardiac output (CO) suggests either a continuum between the pre- and post-capillary haemodynamic profiles or a different course in response to high CO.
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