title: Long-term data from the Swiss pulmonary hypertension registry.
abstract: BACKGROUND
  Registries are important for real-life epidemiology on different pulmonary hypertension (PH) groups.
  
  
  OBJECTIVE
  To provide long-term data of the Swiss PH registry of 1998-2012.
  
  
  METHODS
  PH patients have been classified into 5 groups and registered upon written informed consent at 5 university and 8 associated hospitals since 1998. New York Heart Association (NYHA) class, 6-min walk distance, hemodynamics and therapy were registered at baseline. Patients were regularly followed, and therapy and events (death, transplantation, endarterectomy or loss to follow-up) registered. The data were stratified according to the time of diagnosis into prevalent before 2000 and incident during 2000-2004, 2005-2008 and 2009-2012.
  
  
  RESULTS
  From 996 (53% female) PH patients, 549 had pulmonary arterial hypertension (PAH), 36 PH due to left heart disease, 127 due to lung disease, 249 to chronic thromboembolic PH (CTEPH) and 35 to miscellaneous PH. Age and BMI significantly increased over time, whereas hemodynamic severity decreased. Overall, event-free survival was 84, 72, 64 and 58% for the years 1-4 and similar for time periods since 2000, but better during the more recent periods for PAH and CTEPH. Of all PAH cases, 89% had target medical therapy and 43% combination therapy. Of CTEPH patients, 14 and 2% underwent pulmonary endarterectomy or transplantation, respectively; 87% were treated with PAH target therapy.
  
  
  CONCLUSIONS
  Since 2000, the incident Swiss PH patients registered were older, hemodynamically better and mostly treated with PAH target therapies. Survival has been better for PAH and CTEPH diagnosed since 2008 compared with earlier diagnosis or other classifications.
authors: Pulmonary Clinic, University Hospital Zurich, Zurich, Switzerland.
authors: Séverine
authors: S
authors: Mueller-Mottet
authors: Hans
authors: H
authors: Stricker
authors: Guido
authors: G
authors: Domenighetti
authors: Guido
authors: G
authors: Domeninghetti
authors: Andrea
authors: A
authors: Azzola
authors: Thomas
authors: T
authors: Geiser
authors: Markus
authors: M
authors: Schwerzmann
authors: Daniel
authors: D
authors: Weilenmann
authors: Otto
authors: O
authors: Schoch
authors: Jean-Marc
authors: JM
authors: Fellrath
authors: Thierry
authors: T
authors: Rochat
authors: Frederic
authors: F
authors: Lador
authors: Maurice
authors: M
authors: Beghetti
authors: Laurent
authors: L
authors: Nicod
authors: John-David
authors: JD
authors: Aubert
authors: Vladimir
authors: V
authors: Popov
authors: Rudolf
authors: R
authors: Speich
authors: Stephan
authors: S
authors: Keusch
authors: Elisabeth
authors: E
authors: Hasler
authors: Lars C
authors: LC
authors: Huber
authors: Peter
authors: P
authors: Grendelmeier
authors: Michael
authors: M
authors: Tamm
authors: Silvia
authors: S
authors: Ulrich
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D012042:Registries; D013557:Switzerland / Q000453:epidemiology; D055815:Young Adult