title: [Clinical features of patients with pulmonary artery hypertension associated with hereditary hemorrhagic telangiectasia].
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 - 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.
  
  
  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.
authors.affiliation: Department of Cardiology, Wuhan Asia Heart Hospital, Wuhan 430000, China.
authors.forename: Jin-guo
authors.initials: JG
authors.lastname: Lu
authors.forename: Ming-li
authors.initials: ML
authors.lastname: Sun
authors.forename: Bin
authors.initials: B
authors.lastname: Lü
authors.forename: Xiong-biao
authors.initials: XB
authors.lastname: Chen
authors.forename: Zhi-hui
authors.initials: ZH
authors.lastname: Hou
authors.forename: Shi-liang
authors.initials: SL
authors.lastname: Jiang
authors.forename: Ru-ping
authors.initials: RP
authors.lastname: Dai
authors.forename: Xi
authors.initials: X
authors.lastname: Su
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*; D055815:Young Adult