Aims The right ventricle (RV) is a major determinant of outcome in pulmonary arterial hypertension (PAH), but its assessment has not been completely incorporated in the risk of mortality scores. We therefore evaluated the added value of echocardiography of the RV to risk scores in a large multicentre European prospective cohort of patients with prevalent PAH enrolled in the ULTRAsound RIGHt venTricular eVALUation in PAH (ULTRA RIGHT VALUE) study. Methods and results A total of 401 patients with prevalent PAH enrolled between January 2022 and December 2023 underwent an echocardiography and a risk assessment by Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 and European Society of Cardiology (ESC)/European Respiratory Society (ERS) scoring systems. For both the ESC/ERS and REVEAL scores a one-unit decrease in any morphological parameter increased up to 69% and 92%, respectively, the odds for achieving/maintaining a low-risk status. Similarly, a one-unit increase in any RV functional parameter increased up to 1.28 and 1.78, respectively, the odds for achieving/maintaining a low-risk status. For this purpose, echocardiographic assessments of RV-PA coupling were the most performant. The inclusion of echocardiographic variables into the ESC/ERS and REVEAL 2.0 scores improved the c-index for morbi-mortality prediction from 0.73 [95% confidence interval (CI) 0.69-0.76] to 0.78 (95% CI 0.75-0.81; P < 0.01) and from 0.74 (95% CI 0.67-0.83) to 0.79 (95% CI 0.76-0.82; P < 0.01), respectively, with machine learning techniques showing indexes of RV-PA coupling having the highest influence on the c-index. Conclusion Incorporation of echocardiographic parameters improves risk scores in PAH.
