TY - JOUR T1 - Pulmonary Arterial Hypertension in a Patient With Hereditary Hemorrhagic Telangiectasia JO - Archivos de Bronconeumología T2 - AU - Raimondi,Alejandro AU - Blanco,Isabel AU - Pomares,Xavier AU - Barberà,Joan Albert SN - 15792129 M3 - 10.1016/j.arbr.2013.01.006 DO - 10.1016/j.arbr.2013.01.006 UR - https://www.archbronconeumol.org/en-pulmonary-arterial-hypertension-in-patient-articulo-S1579212913000098 AB - Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by the triad of epistaxis, telangiectasia and vascular malformations. Pulmonary vascular complications associated with this disease include pulmonary arteriovenous malformations (AVM) and, less frequently, pulmonary hypertension (PH).We report the case of a patient who presented multiple pulmonary AVM and PH probably due to HHT. Embolization was carried out on one of the AVM and the patient received specific pulmonary arterial hypertension treatment with an endothelin receptor antagonist. We also described the patient's functional and hemodynamic improvement after almost 3 years of follow-up. ER -