Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Eur Respir J. 2019 Jun 5;53(6):1802004. doi: 10.1183/13993003.02004-2018. Print 2019 Jun.


Background: Current pulmonary hypertension treatment guidelines recommend use of a risk stratification model encompassing a range of parameters, allowing patients to be categorised as low, intermediate or high risk. Three abbreviated versions of this risk stratification model were previously evaluated in patients with pulmonary arterial hypertension (PAH) in the French, Swedish and COMPERA registries. Our objective was to investigate the three abbreviated risk stratification methods for patients with mostly prevalent PAH and chronic thromboembolic pulmonary hypertension (CTEPH), in patients from the PATENT-1/2 and CHEST-1/2 studies of riociguat.

Methods: Risk was assessed at baseline and at follow-up in PATENT-1 and CHEST-1. Survival and clinical worsening-free survival were assessed in patients in each risk group/strata.

Results: With all three methods, riociguat improved risk group/strata in patients with PAH after 12 weeks. The French non-invasive and Swedish/COMPERA methods discriminated prognosis for survival and clinical worsening-free survival at both baseline and follow-up. Furthermore, patients achieving one or more low-risk criteria or a low-risk stratum at follow-up had a significantly reduced risk of death and clinical worsening compared with patients achieving no low-risk criteria or an intermediate-risk stratum. Similar results were obtained in patients with inoperable or persistent/recurrent CTEPH.

Conclusions: This analysis confirms and extends the results of the registry analyses, supporting the value of goal-oriented treatment in PAH. Further assessment of these methods in patients with CTEPH is warranted.

Publication types

  • Clinical Trial, Phase III
  • Multicenter Study
  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Chronic Disease
  • Europe
  • Female
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / drug therapy
  • Hypertension, Pulmonary / mortality*
  • Male
  • Middle Aged
  • Pulmonary Arterial Hypertension / drug therapy
  • Pulmonary Arterial Hypertension / mortality*
  • Pyrazoles / therapeutic use*
  • Pyrimidines / therapeutic use*
  • Registries
  • Risk Assessment*
  • Risk Factors
  • Survival Analysis
  • Thromboembolism / complications
  • Thromboembolism / drug therapy
  • Thromboembolism / mortality*


  • Pyrazoles
  • Pyrimidines
  • riociguat