Pulmonary Arterial Hypertension in a Patient With Hereditary Hemorrhagic Telangiectasia

被引：6

作者：

Raimondi, Alejandro ^{[1
]}

Blanco, Isabel ^{[1
]}

Pomares, Xavier ^{[2
]}

Albert Barbera, Joan ^{[1
]}

机构：

[1] Univ Barcelona, Serv Pneumol, Hosp Clin Inst Invest Biomed August Pi & Sunyer I, Barcelona, Spain

[2] Corp Parc Tauli, Serv Pneumol, Barcelona, Spain

来源：

ARCHIVOS DE BRONCONEUMOLOGIA | 2013年 / 49卷 / 03期

关键词：

Pulmonary hypertension; Arteriovenous malformations; Bosentan; Activin receptor-like kinase 1; Morphogenetic protein receptor II gene; Genetic alterations; Rendu-Osler-Weber syndrome; ARTERIOVENOUS-MALFORMATIONS; COMPLICATIONS; EMBOLIZATION;

D O I：

10.1016/j.arbres.2012.04.021

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

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. (C) 2012 SEPAR. Published by Elsevier Espana, S.L. All rights reserved.

引用

页码：119 / 121

页数：3