title: Epidemiology and long-term survival of pulmonary arterial hypertension in the Czech Republic: a retrospective analysis of a nationwide registry.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) is a severe and progressive disease characterized by increased pulmonary vascular resistance, ultimately leading to right heart failure and death. Epidemiological data from national registries are growing worldwide, but are still unavailable in Eastern Europe.
  
  
  METHODS
  A PAH registry was initiated in January 2007 using a nationwide network of echocardiographic centers and four diagnostic centers that specialize in PAH. All patients aged above 18 years, diagnosed with PAH and monitored between January 2000 and December 2007 were included. Patients diagnosed with PAH between January and December 2007 were classified as incident. The survival analyses were performed up to the end of 2010. Prognostic factors at the time of diagnosis were identified using uni- and multivariable Cox proportional hazard models.
  
  
  RESULTS
  Overall, 191 patients were included (100 prevalent cases, 91 incident cases). Patients were predominantly female (n = 125) and had a mean age of 51.9 ± 16.9 years. Incident patients were significantly older at the time of diagnosis than prevalent patients (p < 0.001). Most patients (60.7%) had idiopathic PAH; 20.4% had PAH associated with congenital heart disease and 11.4% had PAH associated with connective tissue disease. Estimates of prevalence and incidence of PAH in adults were 22.4 cases per million and 10.7 cases per million per year, respectively. The 1-, 2- and 3-year survival rates in the incident PAH cohort were 89% (95% confidence intervals [CI] 83-95%), 78% (95% CI 70-87%) and 74% (95% CI 65-83%), respectively. Lower survival rates were significantly associated with higher age (hazard ratio [HR] 6.6 95% CI 1.4-30.9) and lower creatinine clearance (HR 3.3 95% CI 1.1-9.7).
  
  
  CONCLUSIONS
  This is the first study in Eastern Europe to describe the prevalence, incidence and survival of patients with PAH from a national representative registry. This registry from the Czech Republic highlights that diagnosis of PAH is frequently made late in the disease continuum when patients have significant functional impairment.
authors.affiliation: 1st Faculty of Medicine, 2nd Medical Department, Clinical Department of Cardiology and Angiology, Charles University, Prague, Czech Republic. pavel.jansa@vfn.cz.
authors.forename: Pavel
authors.initials: P
authors.lastname: Jansa
authors.forename: Jiri
authors.initials: J
authors.lastname: Jarkovsky
authors.forename: Hikmet
authors.initials: H
authors.lastname: Al-Hiti
authors.forename: Jana
authors.initials: J
authors.lastname: Popelova
authors.forename: David
authors.initials: D
authors.lastname: Ambroz
authors.forename: Tomas
authors.initials: T
authors.lastname: Zatocil
authors.forename: Regina
authors.initials: R
authors.lastname: Votavova
authors.forename: Pavel
authors.initials: P
authors.lastname: Polacek
authors.forename: Jana
authors.initials: J
authors.lastname: Maresova
authors.forename: Michael
authors.initials: M
authors.lastname: Aschermann
authors.forename: Petr
authors.initials: P
authors.lastname: Brabec
authors.forename: Ladislav
authors.initials: L
authors.lastname: Dusek
authors.forename: Ales
authors.initials: A
authors.lastname: Linhart
mesh_terms: D000328:Adult; D000368:Aged; D018153:Czech Republic / Q000453:epidemiology; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology*; D015994:Incidence; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D055815:Young Adult