Key result
Adding PVOD aetiology to the REVEAL-ECHO score improves mortality and transplant discrimination to a ~0.64 C-index.
Why the study?
Echocardiography-based risk scores such as the REVEAL-ECHO model were not developed in cohorts with a high prevalence of pulmonary veno-occlusive disease (PVOD) and had not been validated in this severe pulmonary arterial hypertension subtype.
Does incorporating pulmonary veno-occlusive disease (PVOD) aetiology into the REVEAL-ECHO risk score improve the prediction of mortality and lung transplantation in a PAH cohort with high PVOD prevalence?
Cohort (n=282)
Does incorporating pulmonary veno-occlusive disease (PVOD) aetiology into the REVEAL-ECHO risk score improve the prediction of mortality and lung transplantation in a PAH cohort with high PVOD prevalence?
Adding pulmonary veno-occlusive disease (PVOD) etiology to the REVEAL-ECHO score significantly improves risk stratification for mortality and lung transplantation in PAH cohorts with a high prevalence of PVOD.
Background Pulmonary veno-occlusive disease (PVOD) is a severe subtype of pulmonary arterial hypertension (PAH) associated with poor prognosis. Echocardiography-based risk scores such as REVEAL-ECHO model1 were not developed in cohorts with a high prevalence of PVOD and have not been validated in this setting. Purpose To characterize echocardiographic remodelling in PVOD, evaluate prognostic performance of REVEAL-ECHO score for mortality and lung transplantation (LT) in a population with high PVOD prevalence, and develop a PVOD-adapted version of the score. Methods A retrospective cohort of 282 PAH patients was analysed, 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 and the original REVEAL-ECHO model. A modified REVEAL-ECHO score was derived by incorporating PVOD aetiology as an additional weighted variable according to its hazard ratio for the primary outcome. Predictive performance was evaluated using discrimination, calibration, and reclassification metrics. Results Among 282 patients (50 PVOD, 17.7%), baseline hemodynamic were comparable between groups, including mean pulmonary arterial pressure (47 vs. 49 mmHg), pulmonary vascular resistance (8.9 vs. 8.5 Wood units) and cardiac index (2.3 vs. 2.5 L/min; all p>0.20). However, PVOD patients had worse functional status and with shorter 6MWD. However, PVOD patients had worse functional status and shorter 6-minute walk distance. Despite similar invasive hemodynamic, echocardiography showed more advanced right ventricular remodelling in PVOD, including a higher right-to-left ventricular basal diameter ratio (1.3 vs. 1.2, p=0.038), lower tricuspid annular plane systolic excursion (17 vs. 19 mm, p=0.006) and reduced tricuspid annular plane systolic excursion to pulmonary artery systolic pressure ratio (0.22 vs. 0.25 mm/mmHg, p=0.034), consistent with impaired right ventricle–pulmonary arterial coupling. The original REVEAL-ECHO model showed limited prognostic performance for mortality and transplantation in this population compared with REVEAL Lite 2.0 (C-index 0.588 vs. 0.704, p<0.001). Incorporating PVOD aetiology improved the echocardiographic model: the modified score increased discrimination compared with the original REVEAL-ECHO (C-index 0.644 vs. 0.588, p=0.002; AUC 0.627 vs. 0.559, p=0.001), narrowing the performance gap with REVEAL Lite 2.0. The modified model also improved event reclassification compared with both the original REVEAL-ECHO (+24.1%) and REVEAL Lite 2.0 (+5.1%). In transplant-focused analyses, the PVOD-adapted score correctly reclassified 20.0% of LT events and 22.2% of urgent transplantations into higher-risk categories. Conclusions Incorporating PVOD aetiology into REVEAL-ECHO score improves its predictive accuracy and refines mortality and LT risk stratification in cohorts with high PVOD prevalence.
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Ortiz et al. (2026) conducted a cohort in Pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease (PVOD) (n=282). Modified PVOD-adapted REVEAL-ECHO score vs. Original REVEAL-ECHO model and REVEAL Lite 2.0 was evaluated on Mortality and lung transplantation (LT) (C-index 0.644 vs 0.588, p=0.002). Incorporating PVOD aetiology into the REVEAL-ECHO score improved discrimination for mortality and lung transplantation compared to the original score (C-index 0.644 vs 0.588, p=0.002).
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