• 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 Pulmonary Vascular Research Institute GoDeep Consortium.
• Department of Internal Medicine, Universities of Giessen and Marburg Lung Center, Member of the German Center for Lung Research, Giessen, Germany; Institute for Lung Health, Cardio-Pulmonary Institute (CPI), Giessen, Germany.
• Chest. 2024 Sep 1; 166 (3): 585603585-603.

BackgroundPulmonary hypertension (PH) is a heterogeneous disease with a poor prognosis. Accurate risk stratification is essential for guiding treatment decisions in pulmonary arterial hypertension (PAH). Although various risk models have been 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 groups 1 through 4?Study Design And MethodsWe conducted a comprehensive analysis of outcomes among patients with incident PH enrolled in the multicenter worldwide Pulmonary Vascular Research Institute GoDeep meta-registry. Analyses were performed across PH groups 1 through 4 and further subgroups to evaluate the predictive value of PAH risk scores, including the Registry to Evaluate Early and Long-Term PAH Disease Mangement (REVEAL) Lite 2, REVEAL 2.0, European Society of Cardiology/European Respiratory Society 2022, Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) 3-strata, and COMPERA 4-strata.ResultsEight thousand five hundred sixty-five patients were included in the study, of whom 3,537 patients were assigned to group 1 PH, whereas 1,807 patients, 1,635 patients, and 1,586 patients were assigned to group 2 PH, group 3 PH, and group 4 PH, respectively. Pulmonary hemodynamics were impaired with median mean pulmonary arterial pressure of 42 mm Hg (interquartile range, 33-52 mm Hg) and pulmonary vascular resistance of 7 Wood units (WU) (interquartile range, 4-11 WU). All risk scores were prognostic in the entire PH population and in each of the PH groups 1 through 4. The REVEAL scores, when used as continuous prediction models, demonstrated the highest statistical prognostic power and granularity; the COMPERA 4-strata risk score provided subdifferentiation of the intermediate-risk group. Similar results were obtained when separately analyzing various subgroups (PH subgroups 1.1, 1.4.1, and 1.4.4; PH subgroups 3.1 and 3.2; group 2 with isolated postcapillary PH vs combined precapillary and postcapillary PH; patients of all groups with concomitant cardiac comorbidities; and severe [> 5 WU] vs nonsevere 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 to the group or calculated separately for each PH group (1-4) and various subgroups.Trial RegistryClinicalTrials.gov; No.: NCT05329714; URL: www.Clinicaltrialsgov.Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.

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