Clinical features of patients with pulmonary artery hypertension associated with hereditary hemorrhagic telangiectasia
10.3760/cma.j.issn.0253-3758.2011.02.017
- VernacularTitle:遗传性出血性毛细血管扩张症相关性肺动脉高压六例的临床特征
- Author:
Jin-Guo LU
1
;
Ming-Li SUN
;
Bin L(U)
;
Xiong-Biao CHEN
;
Zhi-Hui HOU
;
Shi-Liang JIANG
;
Ru-Ping DAI
;
Xi SU
Author Information
1. 武汉亚洲心脏病医院
- Keywords:
Telangiectasia,hereditary hemorrhagic;
Hypertension,pulmonary
- From:
Chinese Journal of Cardiology
2011;39(2):164-167
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical manifestations of patients with pulmonary artery hypertension (PAH) associated with hereditary hemorrhagic telangiectasia (HHT). Methods This retrospective analysis summarized the clinical features of 6 patients with PAH associated with HHT hospitalized at department of cardiology in Cardiovascular Institute and Fuwai Hospital between January 2006 and May 2009. Results The mean age of the 6 patients (3 male) was 34 years (8 -67years). Recurrent epistaxis were present in all patients, there were 4 patients with severe PAH and 2 patients with moderate PAH. All of the six patients with PAH associated with HHT were misdiagnosed at the first hospital visit.Clinical symptoms were significantly improved in 4 patients and remained unchanged in 2 patients combined hepatic venous malformation post medical therapy. Conclusions Misdiagnosis for patients with PAH associated with HHT is a common phenomenon in daily clinical practice. Patients could benefit from the corresponding medical therapy after the establishment of the correct diagnosis.
