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High prevalence of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia

✍ Scribed by Sopeña, Bernardo; Pérez-Rodríguez, Ma. Teresa; Portela, Daniel; Rivera, Alberto; Freire, Mayka; Martínez-Vázquez, César


Book ID
123366882
Publisher
Elsevier Science
Year
2013
Tongue
English
Weight
200 KB
Volume
24
Category
Article
ISSN
0953-6205

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## Abstract Pulmonary arterial hypertension (PAH) and hereditary hemorrhagic telangiectasia (HHT) are distinct clinical entities caused by germline mutations in genes encoding members of the TGFβ/BMP superfamily: __BMPR2__ in PAH and __ACVRL1__, __ENG__, or __SMAD4__ in HHT. When PAH and HHT occasi