Hereditary haemorrhagic telangiectasia and pulmonary artery hypertension
WANG Lan
LIU Jin-ming
Abstract：Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder,characterised by the presence of vascular malformations.Pulmonary hypertension (PH) is a rare complication of HHT,including PH associated with high-output heart failure and liver vascular malformations and pulmonary arterial hypertension secondary to HHT.In the present review,the authors describe the clinical presentation,diagnosis and management of both PH presentations of HHT,as well as the underlying genetics and pathophysiology.
Keywords：hereditary haemorrhagic telangiectasiapulmonary hypertensionpulmonary artery hypertension
Publication Date：2017-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 391-394 )