Successful bosentan therapy for pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia

被引：15

作者：

Chang, Sung-A ^{[1
]}

Jang, Shin Yi ^{[1
]}

Ki, Chang-Seok ^{[2
]}

Kang, I-Seok ^{[3
]}

Kim, Duk-Kyung ^{[1
]}

机构：

[1] Sungkyunkwan Univ, Samsung Med Ctr, Cardiac & Vasc Ctr,Dept Med, Cardiovasc Imaging Ctr,Sch Med,Div Cardiol, Seoul 135710, South Korea

[2] Sungkyunkwan Univ, Samsung Med Ctr, Sch Med, Dept Lab Med & Genet, Seoul 135710, South Korea

[3] Sungkyunkwan Univ, Samsung Med Ctr, Sch Med, Dept Pediat, Seoul 135710, South Korea

来源：

HEART AND VESSELS | 2011年 / 26卷 / 02期

关键词：

Pulmonary arterial hypertension; Hereditary hemorrhagic telangiectasia; Endothelin receptor antagonist;

D O I：

10.1007/s00380-010-0079-z

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Hereditary hemorrhagic telangiectasia (HHT) is a hereditary vascular disease with multiple arterial venous malformations. Pulmonary arterial hypertension is occasionally associated with HHT; however, there is no evidence of targeted therapy for this subgroup of patients. This report describes a 37-year-old woman who presented with dyspnea on exertion. She was finally diagnosed with pulmonary arterial hypertension associated with HHT (HHT2, MIM 600376), and had multiple arterial venous malformations in the lung, liver and pancreas. An oral dual endothelin receptor (ETA/ETB) antagonist, bosentan, was prescribed, and her symptoms, exercise capacity and laboratory findings imporved greatly after treatment. Pulmonary arterial pressure also decreased. Endothelin receptor antagonist is a possible treatment option for pulmonary arterial hypertension associated with HHT.

引用

页码：231 / 234

页数：4

共 9 条

[1] Primary pulmonary hypertension in families with hereditary haemorrhagic telangiectasia [J].