title: Functional class and targeted therapy are related to the survival in patients with pulmonary arterial hypertension.
abstract: OBJECTIVE
  Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH.
  
  
  METHODS
  Patients who were diagnosed with PAH at a single center were reviewed retrospectively. Forty patients (34.9±14.5 years, 80% of female) were enrolled.
  
  
  RESULTS
  Causes were congenital heart disease in 24 (60%), connective tissue disease in 8 (20%) and idiopathic PAH in 6 (15%). Sixteen patients (40%) were WHO functional class III or IV at the time of diagnosis. Twenty seven patients (67.5%) received molecular targeted therapy. During follow-up (53.6±45.5 months), 10 patients (25%) died and 1-, 2-, and 8 year survival rates were 91.3%, 78.7%, and 66.8%, respectively. As expected, median survival of patients with functional class I or II were significantly longer than patients with III or IV (p=0.041). Interestingly, patients with molecular targeted therapy showed longer survival than conventional therapy (p=0.021).
  
  
  CONCLUSIONS
  WHO functional class at the time of diagnosis was the strong predictor of survival, and molecular targeted therapy could significantly improve the survival. Therefore, early screening and intensive management would be crucial to improve the prognosis in the patient with PAH.
authors.affiliation: Division of Cardiology, Heart Center, Gachon University Gil Hospital, Incheon, Korea.; Gachon Cardiovascular Research Institute, Gachon University Gil Hospital, Incheon, Korea.
authors.forename: Yae Min
authors.initials: YM
authors.lastname: Park
authors.affiliation: Division of Cardiology, Heart Center, Gachon University Gil Hospital, Incheon, Korea.; Gachon Cardiovascular Research Institute, Gachon University Gil Hospital, Incheon, Korea. heart@gilhospital.com.
authors.forename: Wook-Jin
authors.initials: WJ
authors.lastname: Chung
authors.affiliation: Gachon Cardiovascular Research Institute, Gachon University Gil Hospital, Incheon, Korea.; Division of Pediatric Cardiology, Gachon University Gil Hospital, Incheon, Korea.
authors.forename: Deok Young
authors.initials: DY
authors.lastname: Choi
authors.affiliation: Division of Rheumatology, Gachon University Gil Hospital, Incheon, Korea.
authors.forename: Han Joo
authors.initials: HJ
authors.lastname: Baek
authors.affiliation: Division of Pulmonology, Gachon University Gil Hospital, Incheon, Korea.
authors.forename: Sung Hwan
authors.initials: SH
authors.lastname: Jung
authors.affiliation: Division of Cardiology, Heart Center, Gachon University Gil Hospital, Incheon, Korea.; Gachon Cardiovascular Research Institute, Gachon University Gil Hospital, Incheon, Korea.
authors.forename: In Suck
authors.initials: IS
authors.lastname: Choi
authors.affiliation: Division of Cardiology, Heart Center, Gachon University Gil Hospital, Incheon, Korea.; Gachon Cardiovascular Research Institute, Gachon University Gil Hospital, Incheon, Korea.
authors.forename: Eak Kyun
authors.initials: EK
authors.lastname: Shin
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D019468:Disease Management; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications; D006801:Humans; D006973:Hypertension / Q000150:complications; D006976:Hypertension, Pulmonary / Q000145:classification* / Q000188:drug therapy* / Q000401:mortality; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D058990:Molecular Targeted Therapy / Q000379:methods*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate
chemical_list: D000959:Antihypertensive Agents
keywords: Pulmonary arterial hypertension; functional classification; molecular targeted therapy; survival