title: Improved survival in medically treated chronic thromboembolic pulmonary hypertension.
abstract: BACKGROUND
  The surgical indication for chronic thromboembolic pulmonary hypertension (CTEPH) has been modified due to recognition of peripheral type CTEPH and changes in surgical methods and skill. Bosentan and sildenafil are used as modern oral therapy (mod Tx) in patients with inoperable CTEPH, although it remains unknown whether they have positive effects on survival.
  
  
  RESULTS
  A total of 202 patients were diagnosed with CTEPH at Chiba University Hospital between 1986 and 2010, 100 of whom underwent pulmonary endarterectomy. Seven medically treated patients with pulmonary vascular resistance (PVR) ≤ 300 dyn·s·cm(-5) were regarded as having mild disease. Survival rate was stratified by date of diagnosis (group 1, 1986-1998; group 2, 1999-2004; group 3, 2005-2010), and prognostic factors in the remaining 95 medically treated patients were investigated. Group 3 included the most patients treated with mod Tx (group 1, 9.1%; group 2, 24.2%; group 3, 65.0%) and had significantly better survival than either group 1 or 2 (5-year survival: group 1, 54.6%; group 2, 69.7%; group 3, 87.3%). Patients receiving mod Tx had significantly better survival than those not on mod Tx (5-year survival: 88.9% vs. 60.2%). Multivariate analysis showed that mod Tx, lower PVR, and lack of comorbidity were significant predictors of better outcome.
  
  
  CONCLUSIONS
  Medically treated patients with CTEPH had a better survival rate, and the use of mod Tx contributed to improved survival.
authors.affiliation: Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan. rintaro09281980@yahoo.co.jp
authors.forename: Rintaro
authors.initials: R
authors.lastname: Nishimura
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Tanabe
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Sugiura
authors.forename: Ayako
authors.initials: A
authors.lastname: Shigeta
authors.forename: Takayuki
authors.initials: T
authors.lastname: Jujo
authors.forename: Ayumi
authors.initials: A
authors.lastname: Sekine
authors.forename: Seiichiro
authors.initials: S
authors.lastname: Sakao
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Kasahara
authors.forename: Koichiro
authors.initials: K
authors.lastname: Tatsumi
mesh_terms: D000328:Adult; D000368:Aged; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011655:Pulmonary Embolism / Q000378:metabolism / Q000503:physiopathology / Q000628:therapy; D012189:Retrospective Studies; D015996:Survival Rate; D014655:Vascular Resistance