Revisiting REVEAL-ECHO: A Focus on Pulmonary Veno-Occlusive Disease

Pulmonary veno-occlusive disease (PVOD) is a severe subtype of pulmonary arterial hypertension (PAH) with especially poor prognosis. Existing risk scores, such as REVEAL Lite 2.0 and REVEAL-ECHO, were not developed in cohorts with high PVOD prevalence. This study aimed to characterize echocardiographic remodeling in PVOD; to evaluate predictive performance of REVEAL-ECHO for mortality and lung transplantation (LT); and to develop a PVOD-adapted version of REVEAL-ECHO score.

A retrospective cohort of 282 PAH patients was analyzed, including 50 PVOD patients. PVOD diagnosis was based on histological confirmation, genetic testing, or compatible clinical and radiological features. Risk was stratified using REVEAL Lite 2.0, the original REVEAL-ECHO model, and a PVOD-modified REVEAL-ECHO model. A combined 4-strata score integrating REVEAL Lite 2.0 with the modified model was also evaluated.

PVOD patients exhibited worse right ventricular remodeling despite similar hemodynamics. REVEAL Lite 2.0 outperformed the original REVEAL-ECHO model, whereas incorporation of PVOD etiology significantly improved the echocardiography-based model. The 4-strata PVOD model showed the best overall prognostic performance, with the highest discrimination and improved reclassification of adverse outcomes across all evaluated endpoints. Concordant low-risk classification by both clinical and echocardiographic models identified a subgroup with a high negative predictive value.

Incorporating PVOD etiology into REVEAL-ECHO score improves its predictive accuracy and refines risk stratification in cohorts with high PVOD prevalence. Integrating etiology with clinical and echocardiographic assessment enhances risk discrimination and may support more individualized decision-making regarding transplant listing timing, including identification of a low-risk subgroup in whom listing could be deferred.