title: Pulmonary hypertension in Switzerland: treatment and clinical course.
abstract: BACKGROUND
  The prognosis of pulmonary hypertension (PH), especially idiopathic pulmonary arterial hypertension (IPAH), has improved during the recent years. The Swiss Registry for PH represents the collaboration of the various centres in Switzerland dealing with PH and serves as an important tool in quality control. The objective of the study was to describe the treatment and clinical course of this orphan disease in Switzerland.
  
  
  METHODS
  We analyzed data from 222 of 252 adult patients, who were included in the registry between January 1999 and December 2004 and suffered from either PAH, PH associated with lung diseases or chronic thromboembolic PH (CTEPH) with respect to the following data: NYHA class, six-minute walking distance (6-MWD), haemodynamics, treatments and survival.
  
  
  RESULTS
  If compared with the calculated expected figures the one, two and three year mean survivals in IPAH increased from 67% to 89%, from 55% to 78% and from 46% to 73%, respectively. Most patients (90%) were on oral or inhaled therapy and only 10 patients necessitated lung transplantation. Even though pulmonary endarterectomy (PEA) was performed in only 7 patients during this time, the survival in our CTEPH cohort improved compared with literature data and seems to approach outcomes usually seen after PEA. The 6-MWD increased maximally by 52 m and 59 m in IPAH and CTEPH, respectively, but in the long term returned to or below baseline values, despite the increasing use of multiple specific drugs (overall in 51% of IPAH and 29% of CTEPH).
  
  
  CONCLUSIONS
  Our national registry data indicate that the overall survival of IPAH and presumably CTEPH seems to have improved in Switzerland. Although the 6-MWD improved transiently, it decreased in the long term despite specific and increasingly combined drug treatment. Our findings herewith underscore the progressive nature of the diseases and the need for further intense research in the field.
authors.affiliation: Department of Internal Medicine, University Hospital, Zürich, Switzerland. manuel.fischler@usz.ch
authors.forename: Manuel
authors.initials: M
authors.lastname: Fischler
authors.forename: Rudolf
authors.initials: R
authors.lastname: Speich
authors.forename: Lorenz
authors.initials: L
authors.lastname: Dorschner
authors.forename: Laurent
authors.initials: L
authors.lastname: Nicod
authors.forename: Guido
authors.initials: G
authors.lastname: Domenighetti
authors.forename: Michael
authors.initials: M
authors.lastname: Tamm
authors.forename: Thierry
authors.initials: T
authors.lastname: Rochat
authors.forename: John-David
authors.initials: JD
authors.lastname: Aubert
authors.forename: Silvia
authors.initials: S
authors.lastname: Ulrich
mesh_terms: D000328:Adult; D017677:Age Distribution; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology* / Q000503:physiopathology / Q000628:therapy; D015994:Incidence; D016040:Lung Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D011669:Pulmonary Wedge Pressure / Q000502:physiology*; D012307:Risk Factors; D017678:Sex Distribution; D015996:Survival Rate / Q000639:trends; D013557:Switzerland / Q000453:epidemiology; D013997:Time Factors
chemical_list: D000959:Antihypertensive Agents