title: Improved survival of Korean patients with idiopathic pulmonary arterial hypertension after the introduction of targeted therapies.
abstract: OBJECTIVE
  We compared the survival of patients with idiopathic pulmonary arterial hypertension (IPAH), receiving conventional and targeted therapies.
  
  
  BACKGROUND
  IPAH is an incurable disease with high mortality. To manage IPAH, several targeted therapies have been used in Korea.
  
  
  METHODS
  We performed a retrospective study of 71 patients diagnosed with IPAH in a tertiary hospital between January 1994 and February 2013. Patients were classified into "conventional therapy group" (treated with conventional therapies and/or beraprost) and "targeted therapy group" (treated with targeted therapies other than beraprost).
  
  
  RESULTS
  The median age of the patients was 33 years and 50 patients were female. The survival rate at 1, 3, 5, and 10 years was 80.1% 62.0%, 51.5%, and 26.8%, respectively. The survival rate in the targeted therapy group was greater than in the conventional therapy group (p-value = 0.026).
  
  
  CONCLUSIONS
  We believe targeted therapies would improve survival benefits in IPAH patients.
authors.affiliation: Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
authors.forename: Byung Ju
authors.initials: BJ
authors.lastname: Kang
authors.affiliation: Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea; Center for Pulmonary Hypertension and Venous Thromboembolism, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
authors.forename: Sang-Do
authors.initials: SD
authors.lastname: Lee
authors.affiliation: Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
authors.forename: Yeon-Mok
authors.initials: YM
authors.lastname: Oh
authors.affiliation: Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea; Center for Pulmonary Hypertension and Venous Thromboembolism, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea. Electronic address: jsdoc1186@daum.net.
authors.forename: Jae Seung
authors.initials: JS
authors.lastname: Lee
mesh_terms: D000328:Adult; D011464:Epoprostenol / Q000031:analogs & derivatives* / Q000627:therapeutic use; D065627:Familial Primary Pulmonary Hypertension / Q000401:mortality / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D056910:Republic of Korea; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult
chemical_list: C048081:beraprost; D011464:Epoprostenol
keywords: Beraprost; Korea; Molecular targeted therapy; Pulmonary arterial hypertension; Survival