{"doi":"10.1002/ejhf.3396","title":"Non-Invasive Prediction of Pulmonary Vascular Disease-Related Exercise Intolerance and Survival in Non-Group 1 Pulmonary Hypertension","abstract":"AIMS: The clinical utility of pulmonary hypertension (PH) risk scores in non-group 1 PH with pulmonary vascular disease (PVD) remains unresolved. METHODS AND RESULTS: We utilized the prospective multicenter PVDOMICS cohort with group 2, 3, 4 or 5 PH-related PVD and calculated group 1 PH risk scores (REVEAL 2.0, REVEAL Lite 2, French registry score and COMPERA 2). The c-statistic to predict death was compared separately in (i) pre-capillary PH groups 3/4/5, and (ii) combined post- and pre-capillary PH group 2. Exercise right heart catheterization reserve, ventricular interdependence and right ventricular-pulmonary artery (RV-PA) coupling were compared across risk categories. Among 449 individuals with group 3/4/5 PH, the REVEAL 2.0 risk score had the highest c-statistic for predicting death (0.699, 95% confidence interval [CI] 0.660-0.737, p < 0.0001) with comparable performance using the simpler REVEAL Lite 2 score (0.695, 95% CI 0.656-0.734, p < 0.0001). The French and COMPERA 2 risk scores were also predictive of mortality, but performance of both was statistically inferior to REVEAL 2.0 (c-statistic difference -0.072, 95% CI -0.123 to -0.020, p = 0.006, and -0.043, 95% CI -0.067 to -0.018, p = 0.0007, respectively). RV function and RV-PA coupling measures were prognostic in isolation, but did not add incremental value to REVEAL (p > 0.50 for all). Findings were similar in patients with group 2 PH (n = 239). Stratification by the REVEAL Lite 2 score non-invasively identified non-group 1 PH with more advanced PVD with worse exercise capacity, RV-PA uncoupling, ventricular interdependence and impaired cardiac output reserve (p < 0.05 for all). CONCLUSIONS: Non-invasive REVEAL risk predicts mortality in non-group 1 PH without incremental prognostic value from detailed RV function or RV-PA coupling assessment. Baseline REVEAL Lite 2 risk stratification non-invasively identifies greater pulmonary vascular dysfunction and right heart-related exercise limitation, which may help guide patient selection for targeted pulmonary vascular therapies in non-group 1 PH.","journal":"European Journal of Heart Failure","year":2024,"id":471503,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":2,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.8628,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":481147,"name":"Hilary M. DuBrock","orcid":"0000-0001-8410-4429","position":1,"is_corresponding":false},{"id":270321,"name":"Paul M. Hassoun","orcid":"0000-0003-0601-4975","position":2,"is_corresponding":false},{"id":292361,"name":"Anna R. Hemnes","orcid":"0000-0002-2755-5845","position":3,"is_corresponding":false},{"id":275096,"name":"Evelyn M. Horn","orcid":"0000-0002-5751-9415","position":4,"is_corresponding":false},{"id":236777,"name":"Jane A. Leopold","orcid":"0000-0003-0598-8882","position":5,"is_corresponding":false},{"id":231638,"name":"Franz Rischard","orcid":"0000-0002-6861-8304","position":6,"is_corresponding":false},{"id":366755,"name":"Erika B. Rosenzweig","orcid":"0000-0003-4849-214X","position":7,"is_corresponding":false},{"id":530080,"name":"Nicholas S. Hill","orcid":"0000-0002-8242-8339","position":8,"is_corresponding":false},{"id":228050,"name":"Serpil C. Erzurum","orcid":"0000-0001-5202-9065","position":9,"is_corresponding":false},{"id":235851,"name":"Gerald J. Beck","orcid":"0000-0002-9514-591X","position":10,"is_corresponding":false},{"id":333953,"name":"Stephen C. Mathai","orcid":"0000-0003-3188-7209","position":11,"is_corresponding":false},{"id":536160,"name":"Monica Mukherjee","orcid":"0000-0001-6088-0773","position":12,"is_corresponding":false},{"id":30816,"name":"W. H. Wilson Tang","orcid":"0000-0002-8335-735X","position":13,"is_corresponding":false},{"id":230314,"name":"Barry A. Borlaug","orcid":"0000-0001-9375-0596","position":14,"is_corresponding":false},{"id":333952,"name":"Robert P. Frantz","orcid":"0000-0003-4128-3978","position":15,"is_corresponding":false},{"id":1306707,"name":"and the PVDOMICS Study Group","orcid":null,"position":16,"is_corresponding":false},{"id":230310,"name":"Yogesh N.V. Reddy","orcid":"0000-0001-5322-0902","position":0,"is_corresponding":true}],"reference_count":38,"raw_metadata":null,"created_at":"2026-07-19T02:05:48.788780Z","pmid":"39058211","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}