{"id":"https://openalex.org/W7224112201","doi":"https://doi.org/10.21037/jtd-2026-2748","title":"Clinical outcomes with initial oral triple and dual therapy in echocardiography-defined connective tissue disease-associated pulmonary hypertension: a retrospective single-center cohort study","display_name":"Clinical outcomes with initial oral triple and dual therapy in echocardiography-defined connective tissue disease-associated pulmonary hypertension: a retrospective single-center cohort study","publication_year":2026,"publication_date":"2026-10-01","ids":{"openalex":"https://openalex.org/W7224112201","doi":"https://doi.org/10.21037/jtd-2026-2748","pmid":"https://pubmed.ncbi.nlm.nih.gov/42996963","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/13740786"},"language":"en","primary_location":{"id":"doi:10.21037/jtd-2026-2748","doi":"https://doi.org/10.21037/jtd-2026-2748","is_oa":true,"landing_page_url":"https://doi.org/10.21037/jtd-2026-2748","pdf_url":null,"source":{"id":"https://openalex.org/S2754193772","display_name":"Journal of Thoracic Disease","issn_l":"2072-1439","issn":["2072-1439","2077-6624"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","dongbi-d","doyens","jufo-1","ki-jl-1","norway-1","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310321814","host_organization_name":"AME Publishing Company","host_organization_lineage":["https://openalex.org/P4310321814"],"host_organization_lineage_names":["AME Publishing Company"],"type":"journal"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Thoracic Disease","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"diamond","oa_url":"https://doi.org/10.21037/jtd-2026-2748","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5160058836","display_name":"Xinyi Shen","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":false,"raw_author_name":"Xinyi Shen","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5125938063","display_name":"Qingyi Chen","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":false,"raw_author_name":"Qingyi Chen","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5160028766","display_name":"Menghuan Ren","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":false,"raw_author_name":"Menghuan Ren","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5160043443","display_name":"Lei Wang","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":false,"raw_author_name":"Lei Wang","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5160043889","display_name":"Jian Wu","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":false,"raw_author_name":"Jian Wu","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5147151784","display_name":"Jing Cao","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":false,"raw_author_name":"Jing Cao","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5159989228","display_name":"Yufeng Yin","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":false,"raw_author_name":"Yufeng Yin","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5160033554","display_name":"Tian Ren","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":false,"raw_author_name":"Tian Ren","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5159998713","display_name":"Xin Zhou","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":true,"raw_author_name":"Xin Zhou","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5159994198","display_name":"Tao Cheng","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":true,"raw_author_name":"Tao Cheng","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5035909215","display_name":"Mingjun Wang","orcid":"https://orcid.org/0009-0003-8595-3782","observed_orcids":["https://orcid.org/0009-0003-8595-3782"]},"institutions":[{"id":"https://openalex.org/I4210153519","display_name":"First Affiliated Hospital of Soochow University","ror":"https://ror.org/051jg5p78","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210153519"]}],"countries":["CN"],"is_corresponding":true,"raw_author_name":"Mingjun Wang","raw_affiliation_strings":["Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China","institution_ids":["https://openalex.org/I4210153519"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":["https://openalex.org/A5035909215","https://openalex.org/A5159994198","https://openalex.org/A5159998713"],"corresponding_institution_ids":["https://openalex.org/I4210153519"],"apc_list":null,"apc_paid":null,"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.84348396,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"18","issue":"10","first_page":"1144","last_page":"1144"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10728","display_name":"Pulmonary Hypertension Research and Treatments","score":0.9990000128746033,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10728","display_name":"Pulmonary Hypertension Research and Treatments","score":0.9990000128746033,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11330","display_name":"Systemic Sclerosis and Related Diseases","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2745","display_name":"Rheumatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T12199","display_name":"Vascular Anomalies and Treatments","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/selexipag","display_name":"selexipag","score":0.9409999847412109},{"id":"https://openalex.org/keywords/macitentan","display_name":"macitentan","score":0.7940000295639038},{"id":"https://openalex.org/keywords/tadalafil","display_name":"tadalafil","score":0.9919999837875366},{"id":"https://openalex.org/keywords/dual-therapy","display_name":"dual therapy","score":0.6610000133514404},{"id":"https://openalex.org/keywords/who-functional-class","display_name":"WHO functional class","score":0.6230000257492065},{"id":"https://openalex.org/keywords/6-minute-walk-distance","display_name":"6-minute walk distance","score":0.972000002861023},{"id":"https://openalex.org/keywords/nt-probnp","display_name":"NT-proBNP","score":0.8849999904632568},{"id":"https://openalex.org/keywords/disease-progression","display_name":"disease progression","score":0.5730000138282776},{"id":"https://openalex.org/keywords/treatment-intensification","display_name":"treatment intensification","score":0.6740000247955322},{"id":"https://openalex.org/keywords/adverse-events","display_name":"adverse events","score":0.3880000114440918},{"id":"https://openalex.org/keywords/clinical-outcomes","display_name":"clinical outcomes","score":0.36800000071525574},{"id":"https://openalex.org/keywords/antiproliferative-therapy","display_name":"antiproliferative therapy","score":0.4740000069141388},{"id":"https://openalex.org/keywords/phosphodiesterase-2-inhibitors","display_name":"phosphodiesterase-2 inhibitor","score":0.7310000061988831}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9736999869346619},{"id":"https://openalex.org/C167135981","wikidata":"https://www.wikidata.org/wiki/Q2146302","display_name":"Retrospective cohort study","level":2,"score":0.5958999991416931},{"id":"https://openalex.org/C203092338","wikidata":"https://www.wikidata.org/wiki/Q1340863","display_name":"Clinical endpoint","level":3,"score":0.5579000115394592},{"id":"https://openalex.org/C72563966","wikidata":"https://www.wikidata.org/wiki/Q1303415","display_name":"Cohort","level":2,"score":0.4959000051021576},{"id":"https://openalex.org/C201903717","wikidata":"https://www.wikidata.org/wiki/Q1778788","display_name":"Cohort study","level":2,"score":0.47699999809265137},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.4767000079154968},{"id":"https://openalex.org/C197934379","wikidata":"https://www.wikidata.org/wiki/Q2047938","display_name":"Adverse effect","level":2,"score":0.44200000166893005},{"id":"https://openalex.org/C2780930700","wikidata":"https://www.wikidata.org/wiki/Q1128595","display_name":"Pulmonary hypertension","level":2,"score":0.42010000348091125},{"id":"https://openalex.org/C2776670291","wikidata":"https://www.wikidata.org/wiki/Q1779300","display_name":"Connective tissue disease","level":4,"score":0.41929998993873596},{"id":"https://openalex.org/C518705261","wikidata":"https://www.wikidata.org/wiki/Q25615","display_name":"Connective tissue","level":2,"score":0.4092999994754791},{"id":"https://openalex.org/C2776999253","wikidata":"https://www.wikidata.org/wiki/Q1304270","display_name":"Combination therapy","level":2,"score":0.33239999413490295},{"id":"https://openalex.org/C2775915353","wikidata":"https://www.wikidata.org/wiki/Q1571529","display_name":"Natriuretic peptide","level":3,"score":0.32839998602867126},{"id":"https://openalex.org/C141071460","wikidata":"https://www.wikidata.org/wiki/Q40821","display_name":"Surgery","level":1,"score":0.31769999861717224},{"id":"https://openalex.org/C500440147","wikidata":"https://www.wikidata.org/wiki/Q701216","display_name":"Pharmacotherapy","level":2,"score":0.29350000619888306},{"id":"https://openalex.org/C534529494","wikidata":"https://www.wikidata.org/wiki/Q7891","display_name":"Respiratory system","level":2,"score":0.2784999907016754},{"id":"https://openalex.org/C2775846464","wikidata":"https://www.wikidata.org/wiki/Q66360952","display_name":"Brain natriuretic peptide","level":3,"score":0.27129998803138733},{"id":"https://openalex.org/C535046627","wikidata":"https://www.wikidata.org/wiki/Q30612","display_name":"Clinical trial","level":2,"score":0.27079999446868896},{"id":"https://openalex.org/C188816634","wikidata":"https://www.wikidata.org/wiki/Q2113324","display_name":"Prospective cohort study","level":2,"score":0.25949999690055847},{"id":"https://openalex.org/C205545832","wikidata":"https://www.wikidata.org/wiki/Q17156455","display_name":"Young adult","level":2,"score":0.25839999318122864},{"id":"https://openalex.org/C2781197716","wikidata":"https://www.wikidata.org/wiki/Q864574","display_name":"Biomarker","level":2,"score":0.25130000710487366}],"mesh":[],"locations_count":3,"locations":[{"id":"doi:10.21037/jtd-2026-2748","doi":"https://doi.org/10.21037/jtd-2026-2748","is_oa":true,"landing_page_url":"https://doi.org/10.21037/jtd-2026-2748","pdf_url":null,"source":{"id":"https://openalex.org/S2754193772","display_name":"Journal of Thoracic Disease","issn_l":"2072-1439","issn":["2072-1439","2077-6624"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","dongbi-d","doyens","jufo-1","ki-jl-1","norway-1","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310321814","host_organization_name":"AME Publishing Company","host_organization_lineage":["https://openalex.org/P4310321814"],"host_organization_lineage_names":["AME Publishing Company"],"type":"journal"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Thoracic Disease","raw_type":"journal-article"},{"id":"pmid:42996963","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/42996963","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of thoracic disease","raw_type":"Journal Article"},{"id":"pmh:oai:pubmedcentral.nih.gov:13740786","doi":null,"is_oa":true,"landing_page_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC13740786/","pdf_url":null,"source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"J Thorac Dis","raw_type":"Text"}],"best_oa_location":{"id":"doi:10.21037/jtd-2026-2748","doi":"https://doi.org/10.21037/jtd-2026-2748","is_oa":true,"landing_page_url":"https://doi.org/10.21037/jtd-2026-2748","pdf_url":null,"source":{"id":"https://openalex.org/S2754193772","display_name":"Journal of Thoracic Disease","issn_l":"2072-1439","issn":["2072-1439","2077-6624"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","dongbi-d","doyens","jufo-1","ki-jl-1","norway-1","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310321814","host_organization_name":"AME Publishing Company","host_organization_lineage":["https://openalex.org/P4310321814"],"host_organization_lineage_names":["AME Publishing Company"],"type":"journal"},"license":"cc-by-nc-nd","license_id":"https://openalex.org/licenses/cc-by-nc-nd","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Thoracic Disease","raw_type":"journal-article"},"sustainable_development_goals":[{"display_name":"Good health and well-being","score":1.0,"id":"https://metadata.un.org/sdg/3"}],"study_designs":[{"display_name":"Observational Study","id":"https://openalex.org/study-designs/observational-study"}],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W2258561468","https://openalex.org/W4410276872","https://openalex.org/W4401101136","https://openalex.org/W4231839334","https://openalex.org/W3108692242","https://openalex.org/W4297906477","https://openalex.org/W4411866381","https://openalex.org/W4411421009","https://openalex.org/W4388594112","https://openalex.org/W2906500307"],"abstract_inverted_index":{"Background:":[0],"Connective":[1],"tissue":[2],"disease-associated":[3],"pulmonary":[4,385],"hypertension":[5],"(CTD-PH)":[6],"is":[7,24],"a":[8],"serious":[9],"complication,":[10],"but":[11],"evidence":[12],"on":[13],"early":[14],"oral":[15,33,352,380],"treatment":[16,381],"intensification":[17,382],"in":[18,39,66,108,220,239,304,313,320,329,360,383],"patients":[19,346],"followed":[20],"primarily":[21],"by":[22,63,258],"echocardiography":[23],"limited.":[25],"We":[26],"evaluated":[27],"outcomes":[28,367],"associated":[29,356],"with":[30,154,246,335,347,357,409],"initial":[31,350],"selexipag-containing":[32,351],"triple":[34,80,187,222,353],"therapy":[35,38,53,74,81,188,225,354,370],"vs.":[36,205,210,217,230,248,260,307,316,323,332],"dual":[37,73,192,224,369],"echocardiography-defined":[40,348],"CTD-PH.":[41],"Methods:":[42],"This":[43],"single-center":[44],"retrospective":[45,394],"cohort":[46,344],"included":[47,83],"consecutive":[48],"adults":[49],"who":[50],"initiated":[51],"PH-targeted":[52],"between":[54,243],"April":[55],"2020":[56],"and":[57,69,88,97,137,174,189,200,223,228,273,326,365,404],"June":[58],"2025.":[59],"Treatment":[60],"was":[61,70,105,215,355],"selected":[62],"treating":[64],"physicians":[65],"routine":[67],"care":[68],"not":[71],"randomized:":[72],"comprised":[75],"macitentan":[76],"plus":[77],"tadalafil,":[78],"whereas":[79],"additionally":[82],"selexipag.":[84],"Baseline":[85],"clinical,":[86],"laboratory,":[87],"echocardiographic":[89],"variables":[90],"were":[91,163,169,177,232],"obtained":[92],"from":[93],"standardized":[94],"institutional":[95],"assessments":[96],"reassessed":[98],"at":[99,159,277],"week":[100,235,278],"26.":[101],"The":[102,194],"primary":[103],"endpoint":[104],"the":[106,109,221,237,252,294,390],"change":[107,238],"number":[110],"(0\u20133)":[111],"of":[112,115,288,345,377],"European":[113],"Society":[114,118],"Cardiology/European":[116],"Respiratory":[117],"(ESC/ERS)":[119],"noninvasive":[120,361,378],"low-risk":[121,240],"criteria":[122,241],"met:":[123],"World":[124],"Health":[125],"Organization":[126],"functional":[127,199,366],"class":[128],"(WHO-FC)":[129],"I\u2013II,":[130],"6-minute":[131],"walk":[132],"distance":[133],"(6MWD)":[134],">440":[135],"m,":[136],"B-type":[138],"natriuretic":[139,147],"peptide":[140,148],"(BNP)":[141],"<50":[142],"ng/L":[143],"or":[144,402],"N-terminal":[145],"pro-B-type":[146],"(NT-proBNP)":[149],"<300":[150],"ng/L.":[151],"Complete":[152],"cases":[153],"all":[155,251],"three":[156,253],"components":[157],"available":[158],"both":[160],"time":[161],"points":[162],"analyzed":[164],"without":[165],"imputation.":[166],"Disease-progression":[167],"events":[168,176],"recorded":[170],"through":[171],"30":[172],"weeks,":[173,373],"adverse":[175,301],"assessed":[178],"during":[179],"follow-up.":[180],"Results:":[181],"Of":[182],"119":[183],"patients,":[184],"59":[185],"received":[186,191],"60":[190],"therapy.":[193],"groups":[195,244],"had":[196],"similar":[197],"baseline":[198],"biomarker":[201],"profiles":[202],"(6MWD,":[203],"290.0":[204],"286.9":[206],"m;":[207,266],"NT-proBNP,":[208],"1,455":[209],"1,473":[211],"pg/mL);":[212],"mean":[213],"age":[214],"45.5":[216],"47.2":[218],"years":[219],"group":[226],"respectively,":[227],"93%":[229],"83%":[231],"women.":[233],"At":[234,298],"26,":[236],"differed":[242,276],"(P=0.003),":[245],"57.6%":[247],"33.3%":[249],"meeting":[250],"criteria.":[254],"Mean":[255],"6MWD":[256],"increased":[257],"156":[259],"104":[261],"m":[262],"(adjusted":[263],"difference,":[264],"52":[265],"95%":[267],"confidence":[268],"interval":[269],"(CI):":[270],"29\u201378;":[271],"P<0.001),":[272],"WHO-FC":[274],"distributions":[275],"26":[279,372],"(P=0.007).":[280],"Exploratory":[281],"analysis":[282],"yielded":[283],"an":[284],"hazard":[285],"ratio":[286],"(HR)":[287],"0.75":[289],"(95%":[290],"CI:":[291],"0.39\u20131.43)":[292],"for":[293],"first":[295],"disease-progression":[296],"event.":[297],"least":[299],"one":[300],"event":[302],"occurred":[303,312],"53/59":[305],"(89.8%)":[306],"47/60":[308],"(78.3%)":[309],"patients;":[310],"diarrhea":[311],"24/59":[314],"(40.7%)":[315],"19/60":[317],"(31.7%),":[318],"nausea":[319],"21/59":[321],"(35.6%)":[322],"16/60":[324],"(26.7%),":[325],"jaw":[327],"pain":[328],"16/59":[330],"(27.1%)":[331],"11/60":[333],"(18.3%),":[334],"no":[336],"significant":[337],"between-group":[338],"differences.":[339],"Conclusions:":[340],"In":[341],"this":[342],"real-world":[343],"CTD-PH,":[349],"greater":[358],"improvement":[359],"ESC/ERS":[362],"risk":[363],"status":[364],"than":[368],"over":[371],"supporting":[374],"further":[375],"evaluation":[376],"risk-guided":[379],"CTD-associated":[384],"vascular":[386],"disease.":[387],"However,":[388],"given":[389],"small,":[391],"single-center,":[392],"nonrandomized":[393],"design,":[395],"these":[396],"findings":[397],"cannot":[398],"establish":[399],"superior":[400],"efficacy":[401],"safety":[403],"require":[405],"prospective":[406],"multicenter":[407],"confirmation":[408],"systematic":[410],"hemodynamic":[411],"assessment.":[412]},"counts_by_year":[],"updated_date":"2026-11-09T13:22:03.289127","created_date":"2026-10-29T00:00:00"}
