Revisiting REVEAL-ECHO: A focus on pulmonary venooclusive disease
E Gutierrez Ortiz, C Jimenez Lopez-Guarch, A Cruz-Utrilla, C Denche Sanz, S Alonso Charterina, M Velazquez Martin, B Brown Arreola, I Martin de Miguel, C A Quezada Loaiza, F Arribas Ynsurriaga, P Escribano SubiasAbstract
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.Table 1For image description, please refer to the figure legend and surrounding text.Figure 1For image description, please refer to the figure legend and surrounding text.