Hereditary hemorrhagic telangiectasia: rare cause of pulmonary hypertension?
A 73-year-old woman was admitted to the emergency room with predominantly right-sided heart failure and anemia. Following clinical and imagiological evaluation, a diagnosis of pulmonary hypertension (PH) associated with Hereditary Hemorrhagic Telangiectasia (HHT) was confirmed. The initial response to bosentan plus sildenafil was good, including improvement in functional class and reduction of edema, allowing her to be discharged. Unfortunately, the patient died, due to her underlying condition, before the effects of the combination treatment could be fully assessed. PH should be considered in patients with HTT and screening for pulmonary hypertension should be performed in these patients and their relatives.
Providência
Rui
R
Cardiology Department, Coimbra's Hospital Center, Coimbra, Portugal. rui_providencia@yahoo.com
Cachulo
Maria do Carmo
Mdo C
Costa
Gisela Veríssimo
GV
Silva
Joana
J
Lemos
Carlos Graça
CG
Leitão-Marques
A M
AM
D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000628:therapy; D013449:Sulfonamides / Q000627:therapeutic use; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000209:etiology* / Q000628:therapy
D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan