The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups

J Heart Lung Transplant. 2017 Sep;36(9):957-967. doi: 10.1016/j.healun.2017.02.016. Epub 2017 Feb 17.

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.

Keywords: biobank; biomarker; etiology subtypes; hypertension; pulmonary; registries; survival.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Age Factors
  • Aged
  • Cause of Death*
  • Cohort Studies
  • Female
  • Germany
  • Hospital Mortality / trends*
  • Hospitals, University
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / mortality*
  • Hypertension, Pulmonary / therapy
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Proportional Hazards Models
  • Registries*
  • Risk Assessment
  • Severity of Illness Index
  • Sex Factors
  • Survival Analysis