PAH risk assessment tools successfully predict survival for 5 years after diagnosis

A review of 3 common pulmonary arterial hypertension (PAH) risk assessment tools found that they successfully predict survival for up to 5 years after initial diagnosis.

Three Common Risk Assessment Tools Used for Pulmonary Arterial Hypertension (PAH) Successfully Predict Transplant-Free Survival Up to 5 Years After Diagnosis, New Article Says Posted in Breathing.

The 3 assessments examined were the most commonly used scores: the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL), the score developed by the Swedish/Comparative Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), and the score developed by the French PH Network Registry (FPHR).

“Risk stratification scores provide a prediction of mortality risk and allow clinicians to determine [a] patient prognosis and therapeutic approach, and optimizing the timing of lung transplantation,” the authors explained.

REVEAL was originally derived from a cohort of 2716 patients at 54 centers in the United States. It was updated to include all-cause hospitalization in the last 6 months after diagnosis as a new variable and the renal function variable was revised. The REVEAL score classifies patients as low risk with a score of 6 or less with a 12-month mortality risk of 2.6% or less; as intermediate with scores of 7 or 8 with a 12-month mortality risk of 6.2% and 7.0%, respectively; and as high risk with a score of 9 or greater with a 12-month mortality risk of 10.7% or greater.

The FPHR includes 4 low-risk criteria: World Health Organization (WHO)/New York Heart Association (NYHA) Functional Class (FC) I or II; 6 minutes on foot > 440 m; right atrial pressure

COMPERA adds 2 additional variables to the FPHR: brain natriuretic peptide (BNP) 65%. Variables are mapped to low, medium, or high risk level, then the average of the risk levels is summed and divided by the number of available variables. The final number is rounded to the next whole number. Patients with a score of 1 are at low risk, 2 at intermediate risk and 3 at high risk.

The researchers collected retrospective data on 50 patients with PAH and ranked their risk at baseline and at 1 year with the assessments. The mean age of patients was 54.8 years at diagnosis and 68% were female. At baseline, 72% were classified as WHO/NYHA FC III/IV, although this improved at 1 year follow-up to only 38%.

At baseline, 68% were classified as high risk using REVEAL. However, most patients were classified as low or intermediate risk using COMPERA (94%) and FPHR (80%). “Compared to baseline, the number of low-risk patients increased and the number of high-risk patients decreased across all 3 scores calculated at 1 year,” the authors found.

For REVEAL and COMPERA, graft-free survival at 3 years was better predicted by scores from 1 year after diagnosis than scores calculated at inclusion. After 5 years of follow-up, this was only true for REVEAL.

“The 5-year prediction was lower for REVEAL 2.0 compared to COMPERA and FPHR calculated at baseline…despite the fact that REVEAL includes more variables and should therefore be more precise in assessing risk,” the authors explained. They hypothesized that this could be due to the additional variables in REVEAL, which could skew the predictive accuracy after 3 years.

The authors suggested that the scores be recalculated after 6 and/or 12 months because the response to treatment could have a major impact. “Further investigation is needed for the evaluation of potential new variables that could improve the prediction of the 3 risk assessment tools,” they concluded.


Vraka A, Yerly P and Aubert JD. Comparison of risk stratification scores in pulmonary arterial hypertension: a single-center retrospective study at the CHUV. Breathing. Published online January 19, 2022. doi:10.1159/000520886