PAH Long-Term Survival Predicted by Reliable Risk Calculator

PAH Dilated Trunk Medical Images
PAH Dilated Trunk Medical Images
Researchers examined the effect of riociguat compared with placebo on the REVEAL risk score and long-term outcomes for pulmonary arterial hypertension.

The Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) risk score (RRS) provides significant predictive value for long-term survival and changes in mortality risk in patients with pulmonary arterial hypertension (PAH) receiving riociguat, according to study findings reported in the Journal of Heart and Lung Transplantation.

Patients with PAH who were enrolled in the 12-week PATENT-1 ( Identifier: NCT00810693) and open-label PATENT-2 ( Identifier: NCT00863681) extension studies of riociguat were included in this analysis (n=396). Investigators evaluated riociguat vs placebo in relation to RRS in PATENT-1. In addition, the researchers applied the RRS to determine the predictive value of the risk score in the change of mortality risk in PATENT-1 on long-term survival outcomes in the PATENT-2 study.

Compared with placebo, the use of riociguat improved both RRS (P =.031) and risk stratum (P =.018) from baseline to 12 weeks in the PATENT-1 study. Baseline RRS, PATENT-1 RRS at 12 weeks, and RRS change during PATENT-1 correlated with both survival (hazard ratios [HRs] for a 1-point reduction in RRS: 0.675, 0.705, and 0.804, respectively) and clinical worsening-free survival (HRs: 0.736, 0.716, and 0.753, respectively) during the 2 years of the PATENT-2 study.

Patients who maintained stability in their risk stratum experienced the greatest survival and clinical worsening-free survival rates, in part due to the large proportion of participants in the low-risk stratum at baseline.

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The investigators of this analysis were unable to use the RRS to determine the parameters affected by riociguat that conferred the greatest effect on long-term outcomes in patients with PAH.

These findings that the RRS holds prognostic utility in patients with PAH are important “considering that contemporary treatment practices are increasingly focused on preventing morbidity due to the improved short-term survival observed with modern therapeutics.”


Benza RL, Farber HW, Frost A, et al. REVEAL risk scores applied to riociguat-treated patients in PATENT-2: impact of changes in risk score on survival [published online November 11, 2017]. J Heart Lung Transplant. doi:10.1016/j.healun.2017.11.006