ArticleHeart and vessels2011
Successful bosentan therapy for pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia.
Article in Heart and vessels, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it, 29 citations in OpenAlex.
- Pancreatic arteriovenous malformations as a manifestation of hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease): a systematic review.BMJ open gastroenterology · 2026Pooled it
- Article
- Rapid improvement in postpartum pulmonary hypertension associated with hereditary hemorrhagic telangiectasia: A case report and review of literature.World journal of clinical cases · 2025Article
- Imaging manifestations of hereditary hemorrhagic telangiectasia with pulmonary arterial hypertension: a case report.Frontiers in cardiovascular medicine · 2025Article
- Case report: Successful use of sotatercept to treat pulmonary arterial hypertension in a patient with hereditary hemorrhagic telangiectasia.Respiratory medicine case reports · 2025Article
- Case report: Use of sotatercept to treat pulmonary arterial hypertension in a patient with hereditary hemorrhagic telangiectasia.Respiratory medicine case reports · 2025Article
- 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 arterial hypertension associated with pulmonary arteriovenous malformations and pulmonary veno-occlusive disease: A devastating combination.Respiratory medicine case reports · 2021Article
- Pulmonary Hypertension and Hereditary Hemorrhagic Telangiectasia Related to an ACVRL1 Mutation.Internal medicine (Tokyo, Japan) · 2020Article
- Pulmonary Arterial Hypertension and Hereditary Haemorrhagic Telangiectasia.International journal of molecular sciences · 2018Review
- Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability.PloS one · 2017Article
- Pulmonary hypertension in hereditary haemorrhagic telangiectasia.World journal of cardiology · 2015Article
- Pulmonary hypertension in a patient with hereditary haemorrhagic telangiectasia.BMJ case reports · 2013Article
- The short- and medium-term results of transcatheter closure of atrial septal defect with severe pulmonary arterial hypertension.Heart and vessels · 2012Article
- The clinical characteristics and long-term prognosis of pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia.Pulmonary circulationArticle
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hereditary hemorrhagic telangiectasia (HHT) is a hereditary vascular disease with multiple arterial venous malformations. Pulmonary arterial hypertension is occasionally associated with HHT; however, there is no evidence of targeted therapy for this subgroup of patients. This report describes a 37-year-old woman who presented with dyspnea on exertion. She was finally diagnosed with pulmonary arterial hypertension associated with HHT (HHT2, MIM 600376), and had multiple arterial venous malformations in the lung, liver and pancreas. An oral dual endothelin receptor (ET(A)/ET(B)) antagonist, bosentan, was prescribed, and her symptoms, exercise capacity and laboratory findings improved greatly after treatment. Pulmonary arterial pressure also decreased. Endothelin receptor antagonist is a possible treatment option for pulmonary arterial hypertension associated with HHT.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.