Hereditary hemorrhagic telangiectasia with pulmonary hypertension: a report of three cases.

ABSTRACT:

Background

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder characterized by multisystem vascular malformations, with a low prevalence worldwide. Pulmonary hypertension (PH), a grave complication of HHT, is associated with a poor prognosis. At present, an agreed approach to the management of HHT in patients with PH is not available. The report herein details the diagnostic and therapeutic processes of three patients with HHT complicated by PH, with the aim of providing clinical insights for practitioners.

Case description

The following report details three cases of HHT with PH. All three patients had a history of recurrent epistaxis, as well as dilated blood vessels in the skin and mucous membranes. Consequently, all patients underwent ri

SUBMITTER: Shi W 

PROVIDER: S-EPMC12268784 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature