ArticleWorld journal of cardiology2015
Pulmonary hypertension in hereditary haemorrhagic telangiectasia.
Article in World journal of cardiology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.
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The trial behind it
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Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 49 citations in OpenAlex.
- Gastrointestinal Manifestations of Hereditary Hemorrhagic Telangiectasia (HHT): A Systematic Review of the Literature.Digestive diseases and sciences · 2017Pooled it
- Obstructive airway disorders affecting individuals with hereditary hemorrhagic telangiectasia: A database review.PloS one · 2025Article
- Case Report: Shared manifestation, distinct etiologies: severe pulmonary hypertension in both mother and neonate.Frontiers in pediatrics · 2025Article
- Anaesthetic management for caesarean section in a patient with hereditary haemorrhagic telangiectasia and severe epistaxis during pregnancy.BMJ case reports · 2023Article
- High cardiac output state due to pelvic arterio-venous shunt: a case report and review of the literature.ESC heart failure · 2023Review
- Current guidelines for diagnosis and management of hepatic involvement in hereditary hemorrhagic teleangiectasia.World journal of hepatology · 2023Review
- The Shunt of It.Chest · 2023Article
- Laparoscopic Double Hepatic Artery Banding/Ligation for Patients With Hepatic Hereditary Haemorrhagic Telangiectasia (HHHT).EJVES vascular forum · 2023Article
- An Unusual Cause of Right Heart Dysfunction and High Output Heart Failure in a Young Woman.Journal of cardiovascular development and disease · 2022Article
- Cardiac and Hemodynamic Manifestations of Hereditary Hemorrhagic Telangiectasia.The International journal of angiology : official publication of the International College of Angiology, Inc · 2022Review
- Homozygous GDF2 nonsense mutations result in a loss of circulating BMP9 and BMP10 and are associated with either PAH or an "HHT-like" syndrome in children.Molecular genetics & genomic medicine · 2021Article
- Pulmonary Vascular Complications in Hereditary Hemorrhagic Telangiectasia and the Underlying Pathophysiology.International journal of molecular sciences · 2021Review
- Heritable pulmonary arterial hypertension complicated by multiple pulmonary arteriovenous malformations.Respiratory medicine case reports · 2021Article
- Ammonia Predicts Hepatic Involvement and Pulmonary Hypertension in Patients With Hereditary Hemorrhagic Telangiectasia.Clinical and translational gastroenterology · 2020Article
- Pulmonary arteriovenous malformations: diagnostic and treatment characteristics.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2019Observational
- Pulmonary Hypertension that Developed During Treatment for Hepatopulmonary Syndrome and Pulmonary Arteriovenous Malformation.Internal medicine (Tokyo, Japan) · 2019Article
- Hereditary Hemorrhagic Telangiectasia with SMAD4 Mutations Is Associated with Fatty Degeneration of the Left Ventricle, Coronary Artery Aneurysm, and Abdominal Aortic Aneurysm.Internal medicine (Tokyo, Japan) · 2019Article
- Effect of Transcriptional Regulator ID3 on Pulmonary Arterial Hypertension and Hereditary Hemorrhagic Telangiectasia.International journal of vascular medicine · 2019Review
- Paediatric pulmonary hypertension caused by an ACVRL1 mutation presenting as Ortner syndrome.Cardiology in the young · 2018Article
- Pulmonary Arterial Hypertension and Hereditary Haemorrhagic Telangiectasia.International journal of molecular sciences · 2018Review
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disorder characterised by vascular malformations in predominantly the brain, liver and lungs. Pulmonary hypertension (PH) is increasingly recognised as a severe complication of HHT. PH may be categorised into two distinct types in patients with HHT. Post-capillary PH most often results from a high pulmonary blood flow that accompanies the high cardiac output state associated with liver arteriovenous malformations. Less frequently, the HHT-related gene mutations in ENG or ACVRL1 appear to predispose patients with HHT to develop pre-capillary pulmonary arterial hypertension. Differentiation between both forms of PH by right heart catheterisation is essential, since both entities are associated with severe morbidity and mortality with different treatment options. Therefore all HHT patients should be referred to an HHT centre.
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