Risk assessment is the cornerstone of pulmonary arterial hypertension (PAH) management. Risk stratification scores predict prognosis and help individualize treatment.
The aims of the study include the following: (1) to compare the prediction for transplant-free survival (TFs) of 3 risk assessment tools at 3 and 5 years after diagnosis and (2) to analyze whether the initial risk stratification was altered after 1 year of treatment.
We collected retrospectively data of 50 patients diagnosed with PAH Group 1. We categorized them as low, intermediate, and high mortality risk at baseline and at 1 year with the (1) Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk score version 2.0, (2) Swedish/Comparative Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (PH) (COMPERA) score, and (3) French PH Network Registry (FPHR) score.
TFs at 3 years is predicted by the 3 scores computed at baseline with an area under the curve (AUC) of 0.73, 0.73, and 0.77, respectively. The predictive value increased when the scores were calculated after 1 year of treatment (AUC = 0.91, 0.89, and 0.78). The prediction of TFs at 5 years was better evaluated by the COMPERA and FPHR (AUC = 0.85) than by REVEAL 2.0 (AUC = 0.69) computed at baseline. A low risk status was associated with excellent TFs whatever the scoring used.
In accordance with the original publications, the 3 scores are able to predict survival up to 5 years after diagnosis. The better performance of the scores after 1 year is a further evidence for their clinical use and an indirect proof for treatment efficacy.