Beschreibung
Background:
The 2015 European pulmonary hypertension guidelines propose a risk stratification scheme for patients with PAH. According to 1-year mortality, patients are categorized into low-risk (estimated 1-year mortality risk of < 5%), intermediate-risk (5-10%) and high-risk (> 10%) groups to facilitate a goal-oriented treatment strategy. The aim of the present study was to evaluate the prognostic value of a truncated version of ESC/ERS risk stratification scheme for patients with PH due to ILD.
Methods:
We retrospectively analyzed 395 patients with PH due to ILD from the Gießen PH registry during 1995-2018. By hemodynamic definition, there were 47% with severe, 38% with non-severe and 15% without PH due to ILD. A truncated version of the ESC/ERS risk stratification scheme (based on nine parameters) was applied. The as-sociation between risk class and survival estimate was used to evaluate with Kaplan-Meier and log-rank test. The influence of each prognostic parameter was determined using univariate and multivariate Cox regression analyses.
Results:
The study included 185 patients with severe PH due to ILD. The 1-, 3- and 5-year transplant-free survival was 71%, 57% and 43%, respectively, in the low-risk group, 60%, 30% and 15%, respectively, in the intermediate-risk group and 48%, 13% and 4%, respectively, in the high-risk group. For patients with non-severe PH due to ILD, there were no significant survival differences between the risk groups (p < 0.123). ESC/ERS risk score, WHO functional class, BNP, RA area and SvO2 were significant predictors of transplant-free survival.
Conclusion:
The truncated version of ESC/ERS risk stratification scheme for PAH predicts trans-plant-free survival in patients with severe PH due to ILD.
