title: Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry.
abstract: BACKGROUND
  Originally reported to occur predominantly in younger women, idiopathic pulmonary arterial hypertension (IPAH) is increasingly diagnosed in elderly patients. We aimed to describe the characteristics of such patients and their survival under clinical practice conditions.
  
  
  METHODS
  Prospective registry in 28 centers in 6 European countries. Demographics, clinical characteristics, hemodynamics, treatment patterns and outcomes of younger (18-65 years) and elderly (>65 years) patients with newly diagnosed IPAH (incident cases only) were compared.
  
  
  RESULTS
  A total of 587 patients were eligible for analysis. The median (interquartile, [IQR]) age at diagnosis was 71 (16) years. Younger patients (n=209; median age, 54 [16] years) showed a female-to-male ratio of 2.3:1 whereas the gender ratio in elderly patients (n=378; median age, 75 [8] years) was almost even (1.2:1). Combinations of PAH drugs were widely used in both populations, albeit less frequently in older patients. Elderly patients were less likely to reach current treatment targets (6 min walking distance>400 m, functional class I or II). The survival rates 1, 2, and 3 years after the diagnosis of IPAH were lower in elderly patients, even when adjusted for age- and gender-matched survival tables of the general population (p=0.006 by log-rank analysis).
  
  
  CONCLUSIONS
  In countries with an aging population, IPAH is now frequently diagnosed in elderly patients. Compared to younger patients, elderly patients present with a balanced gender ratio and different clinical features, respond less well to medical therapy and have a higher age-adjusted mortality. Further characterization of these patients is required.
  
  
  BACKGROUND
  NCT01347216.
authors.affiliation: Department of Respiratory Medicine and German Center of Lung Research, Hannover Medical School, Hannover, Germany. Electronic address: hoeper.marius@mh-hannover.de.
authors.forename: Marius M
authors.initials: MM
authors.lastname: Hoeper
authors.forename: Doerte
authors.initials: D
authors.lastname: Huscher
authors.forename: H Ardeschir
authors.initials: HA
authors.lastname: Ghofrani
authors.forename: Marion
authors.initials: M
authors.lastname: Delcroix
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
authors.forename: Christian
authors.initials: C
authors.lastname: Schweiger
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Grunig
authors.forename: Gerd
authors.initials: G
authors.lastname: Staehler
authors.forename: Stephan
authors.initials: S
authors.lastname: Rosenkranz
authors.forename: Michael
authors.initials: M
authors.lastname: Halank
authors.forename: Matthias
authors.initials: M
authors.lastname: Held
authors.forename: Christian
authors.initials: C
authors.lastname: Grohé
authors.forename: Tobias J
authors.initials: TJ
authors.lastname: Lange
authors.forename: Juergen
authors.initials: J
authors.lastname: Behr
authors.forename: Hans
authors.initials: H
authors.lastname: Klose
authors.forename: Heinrike
authors.initials: H
authors.lastname: Wilkens
authors.forename: Arthur
authors.initials: A
authors.lastname: Filusch
authors.forename: Martin
authors.initials: M
authors.lastname: Germann
authors.forename: Ralf
authors.initials: R
authors.lastname: Ewert
authors.forename: Hans-Juergen
authors.initials: HJ
authors.lastname: Seyfarth
authors.forename: Karen M
authors.initials: KM
authors.lastname: Olsson
authors.forename: Christian F
authors.initials: CF
authors.lastname: Opitz
authors.forename: Sean P
authors.initials: SP
authors.lastname: Gaine
authors.forename: C Dario
authors.initials: CD
authors.lastname: Vizza
authors.forename: Anton
authors.initials: A
authors.lastname: Vonk-Noordegraaf
authors.forename: Harald
authors.initials: H
authors.lastname: Kaemmerer
authors.forename: J Simon R
authors.initials: JS
authors.lastname: Gibbs
authors.forename: David
authors.initials: D
authors.lastname: Pittrow
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012042:Registries; D015996:Survival Rate / Q000639:trends