Application of the REVEAL risk score calculator 2.0 in the CHEST study

Raymond L. Benza, Harrison W. Farber, Adaani E. Frost, Hossein Ardeschir Ghofrani, Paul A. Corris, Marc Lambelet, Sylvia Nikkho, Christian Meier, Marius M. Hoeper

Research output: Contribution to journalArticlepeer-review


Background: Currently there are no risk assessment recommendations for chronic thromboembolic pulmonary hypertension (CTEPH). The Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk score (RRS), developed for risk assessment in patients with pulmonary arterial hypertension, has previously predicted outcomes in CTEPH. RRS 2.0 was developed to refine the RRS. Methods: This post hoc analysis of the CHEST study (n = 237), which assessed riociguat in patients with inoperable and persistent/recurrent CTEPH, evaluated RRS 2.0 and its relationship with survival and clinical worsening-free survival (CWFS). Results: At CHEST-1 Week 16, RRS 2.0 significantly improved and more patients moved into the low-risk stratum with riociguat versus placebo; these improvements were maintained at CHEST-2 Week 12. RRS 2.0 at CHEST-1 baseline and Week 16, and change in RRS 2.0 from CHEST-1 baseline to Week 16 were significant predictors of survival and CWFS in CHEST-2. Conclusions: Our data suggest that RRS 2.0 may have utility in predicting outcomes and monitoring treatment response in patients with inoperable or persistent/recurrent CTEPH.

Original languageEnglish (US)
Article number106783
JournalRespiratory Medicine
StatePublished - Apr 1 2022


  • Chronic thromboembolic pulmonary hypertension
  • Pulmonary hypertension
  • Riociguat
  • Risk assessment

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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