Pulmonary hypertension in hereditary hemorrhagic telangiectasia: A clinical review.
chronic thromboembolic pulmonary hypertension
hereditary hemorrhagic telangiectasia
high‐output heart failure
pulmonary arterial hypertension
pulmonary hypertension
Journal
Pulmonary circulation
ISSN: 2045-8932
Titre abrégé: Pulm Circ
Pays: United States
ID NLM: 101557243
Informations de publication
Date de publication:
Oct 2023
Oct 2023
Historique:
received:
13
09
2023
revised:
06
10
2023
accepted:
09
10
2023
medline:
23
10
2023
pubmed:
23
10
2023
entrez:
23
10
2023
Statut:
epublish
Résumé
Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant hereditary disorder characterized by recurrent spontaneous epistaxis, mucocutaneous telangiectasias, and solid organ arteriovenous malformations (AVMs). Pulmonary hypertension (PH) is an increasingly recognized complication in patients with HHT, most often precipitated by high-output heart failure in the presence of hepatic AVMs as well as pulmonary arterial hypertension in the form of a proliferative vasculopathy. The presence of PH in patients with HHT is associated with significant elevations in rates of morbidity and mortality. Additionally, there is growing recognition of a thromboembolic propensity in this population that increases the risk of chronic thromboembolic PH, posing unique clinical considerations regarding the use of anticoagulation. Patients with HHT are also at risk of PH due to disorders commonly seen in the general population, including left-sided heart and lung disease. The etiology of PH in HHT is multifaceted and complex; the diagnostic approach and treatment strategies must consider the underlying pathophysiology of HHT. This comprehensive review summarizes current knowledge of PH in HHT, detailing the pathogenesis of known etiologies, diagnostic evaluation, and suggested treatment modalities as well as emerging therapies that may be of future interest.
Identifiants
pubmed: 37868718
doi: 10.1002/pul2.12301
pii: PUL212301
pmc: PMC10585978
doi:
Types de publication
Journal Article
Review
Langues
eng
Pagination
e12301Informations de copyright
© 2023 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.
Déclaration de conflit d'intérêts
The authors declare no conflict of interest.
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