The added value of echocardiography in pulmonary arterial hypertension risk assessment an artificial intelligence machine learning–derived analysis of the ULTRA RIGHT VALUE registry

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.

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Journal
European Heart Journal - Cardiovascular Imaging
Published
2026-09-17
DOI
https://doi.org/10.1093/ehjci/jeag232
Primary Topic
Pulmonary Hypertension Research and Treatments
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article
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article

The added value of echocardiography in pulmonary arterial hypertension risk assessment an artificial intelligence machine learning–derived analysis of the ULTRA RIGHT VALUE registry

George Giannakoulas, Enrico Maggio, Domenico Filomena, Roberto Badagliacca et al.
European Heart Journal - Cardiovascular Imaging
Pulmonary Hypertension Research and Treatments
article

The added value of echocardiography in pulmonary arterial hypertension risk assessment an artificial intelligence machine learning–derived analysis of the ULTRA RIGHT VALUE registry

George Giannakoulas, Enrico Maggio, Domenico Filomena, Roberto Badagliacca, Nadia Cedrone, Alessio Micheli, Giovanna Manzi, Grzegorz Kopeć, Carmen Jiménez López-Guarch, Laura Scelsi, Marco Podda, Jakub Stępniewski, Giulio Savonitto, Andrea Vergara, Alessandra Greco, Kamil Jonas, Antonio Orlando, Danilo Alunni Fegatelli, Pilar Escribano-subias, Stefano Ghio, Francesco Lo Giudice, Michele D’Alto, Mauro Acquaro, Khodr Tello, Vasiliki Patsiou, Silvia Papa, Wendy Gin‐Sing, Robert Naeije, Carmine Dario Vizza, Polykarpos Psochias, Alejandro Cruz-Utrilla, Alexandra Mihai, Luke Howard, Selin Yildiz, Antonio Nicoliello, Nils Kremer
article en

Abstract

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.

European Heart Journal - Cardiovascular Imaging
University of Pisa (IT), Jagiellonian University (PL), University of Trieste (IT), Imperial College Healthcare NHS Trust (GB), Justus-Liebig-Universität Gießen (DE), Aristotle University of Thessaloniki (GR), Policlinico Umberto I (IT), AHEPA University Hospital (GR), Hammersmith Hospital (GB), John Paul II Hospital (PL), German Center for Lung Research (DE), Universities of Giessen and Marburg Lung Center (DE), Ospedale Sandro Pertini (IT), Ospedale Monaldi (IT), Policlinico San Matteo Fondazione (IT), Cardio-Pulmonary Institute (DE), Department of Public Health (MM), Sapienza University of Rome (IT)
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Openalex Percentile: Top 12%
Pulmonary Hypertension Research and Treatments
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