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Hemodynamics of Pulmonary Hypertension in the Setting of Hereditary Hemorrhagic Telangiectasia

✍ Scribed by Lyle, M.A.; Fenstad, E.R.; McGoon, M.D.; Frantz, R.P.; Krowka, M.J.; Kane, G.C.; Swanson, K.L.

Book ID
122175914
Publisher
Elsevier Science
Year
2014
Tongue
English
Weight
89 KB
Volume
33
Category
Article
ISSN
1557-3117

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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 TGFΞ²/BMP superfamily: __BMPR2__ in PAH and __ACVRL1__, __ENG__, or __SMAD4__ in HHT. When PAH and HHT occasi