Telangiectasia hereditária hemorrágica: causa rara de hipertensão pulmonar?

Abstract

: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.

The clinical characteristics and long‐term prognosis of pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia

“…A few studies have reported the beneficial treatment effect of Bosentan or the combination of Bosentan and Sidenafil on HHT-PAH patients. 7 , 8 It also seems rational to treat HHT-PAH patients according to the PAH guidelines. However, clinical management experience for HHT-PAH patients is sparse as no long-term studies are available.…”