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Pulmonary Arterial Hypertension in a Patient With Hereditary Hemorrhagic Telangiectasia

✍ Scribed by Raimondi, Alejandro; Blanco, Isabel; Pomares, Xavier; Barberà, Joan Albert

Book ID
123048175
Publisher
Elsevier Science
Year
2013
Tongue
Spanish
Weight
493 KB
Volume
49
Category
Article
ISSN
0300-2896

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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