Clinical features of patients with pulmonary artery hypertension associated with hereditary hemorrhagic telangiectasia / 中华心血管病杂志
Chinese Journal of Cardiology
;
(12): 164-167, 2011.
Article
in Chinese
| WPRIM
| ID: wpr-244031
ABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinical manifestations of patients with pulmonary artery hypertension (PAH) associated with hereditary hemorrhagic telangiectasia (HHT).</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>The mean age of the 6 patients (3 male) was 34 years (8 - 67 years). 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.</p><p><b>CONCLUSIONS</b>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.</p>
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Telangiectasia, Hereditary Hemorrhagic
/
Retrospective Studies
/
Hypertension, Pulmonary
Type of study:
Observational study
Limits:
Adolescent
/
Adult
/
Aged
/
Child
/
Female
/
Humans
/
Male
Language:
Chinese
Journal:
Chinese Journal of Cardiology
Year:
2011
Type:
Article
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