title: Long-term outcome in a patient with pulmonary hypertension and hereditary hemorrhagic telangiectasia.
abstract: Hereditary hemorrhagic telangiectasia (HHT) may be associated with pulmonary hypertension (PH). In the context that little attention has been given to long-term follow-up of such individuals, we report a patient with PH associated with HHT with special attention to clinical features and long-term response to therapy. To our knowledge, this case represents only the second with a 10-year follow-up reported and demonstrates that aggressive therapy can lead to long-term improvement in clinical parameters and survival.
authors.affiliation: Department of Pulmonary, Allergy, and Critical Care Medicine, A90, Cleveland Clinic, 9500 Euclid Ave, Cleveland, OH 44195, USA. minaio@ccf.org
authors.forename: Omar A
authors.initials: OA
authors.lastname: Minai
authors.forename: Christina
authors.initials: C
authors.lastname: Rigelsky
authors.forename: Charis
authors.initials: C
authors.lastname: Eng
authors.forename: Alejandro C
authors.initials: AC
authors.lastname: Arroliga
authors.forename: James K
authors.initials: JK
authors.lastname: Stoller
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D006328:Cardiac Catheterization; D018450:Disease Progression; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D011669:Pulmonary Wedge Pressure; D012129:Respiratory Function Tests; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*; D013997:Time Factors
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol; C427248:treprostinil