Background/aim
Estimation of prognosis in pulmonary arterial hypertension (PAH) has been influenced by that various risk stratification models use different numbers of prognostic parameters, as well as the lack of a comprehensive and time-saving risk assessment calculator. We therefore evaluated the various ESC/ERS-based three- and four-strata risk stratification models and established a comprehensive internet-based calculator to facilitate risk assessment.
Methods
Between January 1st, 2000, and July 26th, 2021, 773 clinical assessments on 169 incident PAH patients were evaluated at diagnosis and follow-ups. Risk scores were calculated using the original SPAHR/COMPERA three-strata model, the updated SPAHR three-strata model with divided intermediate risk, and the simplified three-parameter COMPERA 2.0 four-strata model. The original SPAHR/COMPERA- and the updated SPAHR models were tested for both 3-6 and 7-11 available parameters, respectively. Prognostic accuracy (area under the ROC curve, AUC) and time-dependent AUCs (Uno’s cumulative c-statistics, uAUC) were calculated for 1-, 3-, and 5-year mortality.
Results
At baseline, both the original SPAHR/COMPERA and the updated SPAHR models, using up to six parameters, provided the highest accuracy (uAUC=0.73 for both models) in predicting 1-, 3-, and 5-year mortality. At follow-ups, the updated SPAHR model with divided intermediate risk (7-11 parameters), provided the highest accuracy for 1-, 3-, and 5-year mortality (uAUC=0.90), followed by the original SPAHR/COMPERA model (7-11 parameters) (uAUC=0.88), and the COMPERA 2.0 model (uAUC=0.85).
Conclusions
The present study facilitates risk assessment in PAH by introducing a comprehensive internet-based risk score calculator. At baseline, utilizing the original- or the updated SPAHR models using up to six parameters was favourable, the latter additionally offering sub-characterization of the intermediate risk group. Our findings support the 2022 ESC/ERS PH guidelines strategy for risk stratification suggesting the utilization of a three-strata model at baseline and a simplified four-strata model at follow-ups. Our findings furthermore support the utility of the updated SPAHR model with divided intermediate risk, when a more comprehensive assessment is needed at follow-ups, complementing the three-parameter COMPERA 2.0 model. Larger multi-centre studies are encouraged to validate the utility of the updated SPAHR model.