The findings of an evaluation of several risk assessment tools for pulmonary arterial hypertension (PAH) were recently published in the journal Respiration.

An evaluation of 3 risk assessment tools for pulmonary arterial hypertension (PAH) found that each of the tools had value in predicting survival up to 5 years from baseline, and that the predictive value of these tools improved with evaluations done after 1 year of treatment for PAH.

In the current study, investigators compared the predictive value with respect to transplant-free survival (TFs) in PAH for 3 risk assessment tools: 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. The researchers retrospectively assessed these tools’ predictive value for predicting survival at 3 and 5 years post PAH diagnosis in a cohort of 50 patients. The investigators also assessed whether patients’ initial stratification changed when patients were evaluated with each tool 1 year after initiation of PAH treatment.

In the analysis, each tool was used to divide the 50 patients studied into low, intermediate, and high mortality risk groups at baseline and 1 year. At baseline, transplant-free survival rates at 3 years were predicted, with an area under the curve (AUC) of 0.73 for REVEAL, 0.73 for COMPERA, and 0.77 for FPHR. After 1 year of treatment, this predictive value increased, with AUCs of 0.91 for REVEAL, 0.89 for COMPERA, and 0.78 for FPHR. Importantly, low-risk status on any test was strongly associated with excellent transplant-free survival rates.

“Further investigation is necessary for the evaluation of potential new variables that could improve the prediction of the 3 risk assessment tools,” concluded the authors. “For example, peak oxygen uptake measured during cardiopulmonary exercise testing would be a potential candidate marker. Finally, as individual response to treatment has a major impact for prognosis, score calculation after 6 and/or 12 months should be strongly recommended.”

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