• Athiththan Yogeswaran, Henning Gall, Meike Fünderich, Martin R Wilkins, Luke Howard, David G Kiely, Allan Lawrie, Paul M Hassoun, Yuriy Sirenklo, Olena Torbas, Andrew J Sweatt, Roham T Zamanian, Paul G Williams, Marlize Frauendorf, Alexandra Arvanitaki, George Giannakoulas, Khaled Saleh, Hani Sabbour, Hector R Cajigas, Robert Frantz, Imad Al Ghouleh, Stephen Y Chan, Evan Brittain, Jeffrey S Annis, Antonella Pepe, Stefano Ghio, Stylianos Orfanos, Anastasia Anthi, Raphael W Majeed, Jochen Wilhelm, Hossein Ardeschir Ghofrani, Manuel J Richter, Friedrich Grimminger, Sandeep Sahay, Khodr Tello, Werner Seeger, and PVRI-GoDeep-Consortium.
• Department of Internal Medicine, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Giessen (Germany);; Institute for Lung Health (ILH), Cardio-Pulmonary Institute (CPI), Giessen (Germany).
• Chest. 2024 Mar 18.

BackgroundPulmonary hypertension (PH) is a heterogeneous disease with poor prognosis. Accurate risk stratification is essential for guiding treatment decisions in pulmonary arterial hypertension (PAH). While various risk models were developed for PAH, their comparative prognostic potential requires further exploration. Additionally, the applicability of risk scores in PH groups beyond group 1 remains to be investigated.Research QuestionAre risk scores originally developed for PAH predictive in PH group 1-4?Study Design And MethodsWe conducted a comprehensive analysis of outcomes among incident PH patients enrolled in the multicenter worldwide PVRI-GoDeep meta-registry. Analyses were performed across PH groups 1-4 and further subgroups to evaluate the predictive value of PAH-risk scores, including REVEAL Lite 2, REVEAL 2.0, ESC/ERS 2022, COMPERA 3-strata and COMPERA 4-strata.Results8565 patients were included in the study, of whom 3537 patients were assigned to group 1 PH while 1807, 1635, and 1586 patients were diagnosed with group 2, group 3, and group 4 PH. Pulmonary hemodynamics were impaired with median mPAP of 42 [33,52]mmHg and PVR of 7 [4,11]WU. All risk scores were prognostic in the entire PH population and in each of the PH groups 1-4. The REVEAL scores, when used as continuous prediction models, possessed the highest statistical prognostic power and granularity; the COMPERA 4-strata risk score provided sub-differentiation of the intermediate-risk group. Similar results were obtained when separately analyzing various subgroups (PH subgroups 1.1, 1.4.1, 1.4.4; 3.1, 3.2; group 2 with isolated post-capillary-PH versus combined pre-/post-capillary-PH; patients of all groups with concomitant cardiac comorbidities; severe [> 5 WU] versus non-severe PH).InterpretationThis comprehensive study with real-world data from 15 PH-centers showed that PAH-designed risk scores possess predictive power in a large PH cohort, whether considered as common group or calculated separately for each PH group (1-4) and various subgroups.Copyright © 2024. Published by Elsevier Inc.

5 keywords...

hide…