E-REVEAL Lite 2.0 scoring for early prediction of disease progression in pulmonary arterial hypertension

Risk stratification is an essential tool in the management of pulmonary arterial hypertension (PAH). These tools lack detailed echocardiographic assessment which plays a central role in clinical risk assessment in PAH. Thus, we aimed at assessing whether adding echocardiography-driven data to REVEAL Lite 2.0 (Registry to Evaluate Early and Long-Term PAH Disease Management) improves the assessment of risk stratification in PAH. A retrospective analysis of 134 consecutive patients between January 2016 and December 2019 was done. We identified patients who experienced a disease progression “event” defined by the initiation of intravenous (IV) or parenteral prostacyclin, transplant referral, or death due to PAH. All other PAH patients who did not experience an “event” during this period were included in the analysis as controls. Echocardiography and REVEAL Lite 2.0 were collected from 4 to 8 months before the event and compared with the control group to predict the risk of a disease progression event. One hundred and ten patients were included in the final analysis with 22 experiencing a disease progression event and 88 remaining stable during the study period. Different echocardiographic parameters were combined with REVEAL Lite 2.0 scores in both groups. The combination of REVEAL Lite 2.0 and the left ventricular end-diastolic (LVED) eccentricity index (as a continuous variable) had the highest area under the curve (AUC) of 0.87, which approached a significant difference with that of the REVEAL Lite 2.0 alone (p = 0.052). An additional multivariable regression model that included REVEAL Lite 2.0, LVED eccentricity index as a continuous variable, and RAP achieved the best AUC at 0.88 (0.80, 0.96), which was significantly different from that of the REVEAL Lite 2.0 alone (AUC 0.77 [0.66, 0.88]; p = 0.049). These results suggest that combining different echocardiographic parameters to REVEAL Lite 2.0 provides more statistically accurate risk predictions compared to REVEAL Lite 2.0 alone. A combination of LVED eccentricity index with REVEAL Lite 2.0 achieved the best AUC in predicting the event in our cohort.