title: The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups.
abstract: BACKGROUND
  Pulmonary hypertension (PH) is a severe, progressive disease. Although 5 PH subgroups are recognized, reports on survival have focused mainly on pulmonary arterial hypertension (PAH).
  
  
  METHODS
  Long-term transplant-free survival and its determinants were investigated in patients with PH (diagnosed by right heart catheterization) within a prospective registry at a single referral center in Giessen, Germany.
  
  
  RESULTS
  In total, 2,067 patients were enrolled (PAH, 685 patients [33.1%]; pulmonary venous hypertension, 307 patients [14.9%]; PH due to lung diseases (LD-PH), 546 patients [26.4%; mainly interstitial lung disease and chronic obstructive pulmonary disease]; chronic thromboembolic PH, 459 patients [22.2%]; PH owing to miscellaneous/unknown causes, 70 patients [3.4%]). Median follow-up was 37 months. Differences in transplant-free survival between etiologic groups were highly significant (p < 0.001), with 1-, 3- and 5-year survival rates of 88.2%, 72.2% and 59.4%, respectively, for those with PAH compared with 79.5%, 52.7% and 38.1%, respectively, for patients with LD-PH. Patients' age, gender and 6-minute walk distance (6MWD), but not New York Heart Association (NYHA) functional class, associated significantly with survival across all PH subtypes in multivariate Cox regression analyses.
  
  
  CONCLUSIONS
  This is the largest single-center PH cohort described so far. Some parameters used in clinical practice do not independently predict survival. Age, gender and 6MWD outperformed NYHA functional class in predicting survival across all etiologic groups.
authors.affiliation: Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK; Department of Epidemiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors.forename: Henning
authors.initials: H
authors.lastname: Gall
authors.affiliation: Department of Epidemiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors.forename: Janine F
authors.initials: JF
authors.lastname: Felix
authors.affiliation: Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK.
authors.forename: Franziska K
authors.initials: FK
authors.lastname: Schneck
authors.affiliation: Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK; Comprehensive Pneumology Center, Ludwig-Maximilians University, Asklepios Clinic Gauting and Helmholtz Center Munich, Munich, Germany.
authors.forename: Katrin
authors.initials: K
authors.lastname: Milger
authors.affiliation: Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK.
authors.forename: Natascha
authors.initials: N
authors.lastname: Sommer
authors.affiliation: Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK.
authors.forename: Robert
authors.initials: R
authors.lastname: Voswinckel
authors.affiliation: Department of Epidemiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors.forename: Oscar H
authors.initials: OH
authors.lastname: Franco
authors.affiliation: Department of Epidemiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands; Department of Epidemiology, T.H. Chan School of Public Health, Harvard University, Boston, Massachusetts, USA.
authors.forename: Albert
authors.initials: A
authors.lastname: Hofman
authors.affiliation: Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK.
authors.forename: Ralph T
authors.initials: RT
authors.lastname: Schermuly
authors.affiliation: Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK.
authors.forename: Norbert
authors.initials: N
authors.lastname: Weissmann
authors.affiliation: Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK.
authors.forename: Friedrich
authors.initials: F
authors.lastname: Grimminger
authors.affiliation: Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK.
authors.forename: Werner
authors.initials: W
authors.lastname: Seeger
authors.affiliation: Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen, Marburg Lung Center, German Center for Lung Research, Giessen, Germany, and Department of Medicine, Imperial College London, UK. Electronic address: ardeschir.ghofrani@innere.med.uni-giessen.de.
authors.forename: Hossein A
authors.initials: HA
authors.lastname: Ghofrani
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D002423:Cause of Death; D015331:Cohort Studies; D005260:Female; D005858:Germany; D017052:Hospital Mortality / Q000639:trends*; D006785:Hospitals, University; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016016:Proportional Hazards Models; D012042:Registries; D018570:Risk Assessment; D012720:Severity of Illness Index; D012737:Sex Factors; D016019:Survival Analysis
keywords: biobank; biomarker; etiology subtypes; hypertension; pulmonary; registries; survival