{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T02:18:46Z","timestamp":1792030726954,"version":"4.4.0"},"reference-count":23,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,8,21]],"date-time":"2023-08-21T00:00:00Z","timestamp":1692576000000},"content-version":"vor","delay-in-days":51,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100002291","name":"Pulmonary Hypertension Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002291","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["1F32HL159917\u201001A1"],"award-info":[{"award-number":["1F32HL159917\u201001A1"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["U01 HL125175"],"award-info":[{"award-number":["U01 HL125175"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["U01 HL125177"],"award-info":[{"award-number":["U01 HL125177"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["U01 HL125205"],"award-info":[{"award-number":["U01 HL125205"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["U01 HL125208"],"award-info":[{"award-number":["U01 HL125208"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["U01 HL125212"],"award-info":[{"award-number":["U01 HL125212"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["U01 HL125215"],"award-info":[{"award-number":["U01 HL125215"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["U01 HL125218"],"award-info":[{"award-number":["U01 HL125218"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2023,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The prevalence of acute vasodilator response (AVR) to inhaled nitric oxide (iNO) during right heart catheterization (RHC) is 12% in idiopathic pulmonary arterial hypertension (IPAH). AVR, however, is reportedly lower in other disease\u2010associated pulmonary arterial hypertension (PAH), such as connective tissue disease (CTD). The prevalence of AVR in patients on PAH therapy (prevalent cases) is unknown. We sought to determine AVR prevalence in Group 1 PH in the PVDOMICS cohort of incident and prevalent patients undergoing RHC. AVR was measured in response to 100% O<jats:sub>2<\/jats:sub> and O<jats:sub>2<\/jats:sub> plus iNO, with positivity defined as (1) decrease in mean pulmonary artery pressure (mPAP) by \u226510\u2009mmHg to a value \u226440\u2009mmHg, with no change or an increase in cardiac output (definition 1); or (2) decrease in mPAP by \u226512% and pulmonary vascular resistance by \u226530% (definition 2). AVR rates and cumulative survival were compared between incident and prevalent patients. In 338 mainly prevalent (86%) patients, positive AVR to O<jats:sub>2<\/jats:sub>\u2010only was &lt;2%, and 5.1% to 16.9%, based on definition 1 and 2 criteria, respectively; following O<jats:sub>2<\/jats:sub>\u2009+\u2009iNO. IPAH AVR prevalence (4.1%\u201318.7%) was similar to prior reports. AVR positivity was 7.7% to 15.4% in mostly CTD\u2010PAH prevalent cases, and 2.6% to 11.8% in other PAH groups. Survival was 89% in AVR\u00a0responders versus 77% in nonresponders from PAH diagnosis, and 91% versus 86% from PVDOMICS enrollment (log\u2010rank test <jats:italic>p<\/jats:italic>\u2009=\u20090.04 and <jats:italic>p<\/jats:italic>\u2009=\u20090.05, respectively). In conclusion, AVR in IPAH patients is similar to prior studies. AVR in non\u2010IPAH patients was higher than previously reported. The relationship between PAH therapy, AVR response, and survival warrants further investigation.<\/jats:p>","DOI":"10.1002\/pul2.12281","type":"journal-article","created":{"date-parts":[[2023,8,22]],"date-time":"2023-08-22T08:12:12Z","timestamp":1692691932000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Frequency of acute vasodilator response (AVR) in incident and prevalent patients with pulmonary arterial hypertension: Results from the pulmonary vascular disease phenomics study"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7584-2489","authenticated-orcid":false,"given":"Mario","family":"Naranjo","sequence":"first","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Medicine Johns Hopkins University Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erika B.","family":"Rosenzweig","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Medicine Columbia University New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna R.","family":"Hemnes","sequence":"additional","affiliation":[{"name":"Division of Allergy, Pulmonary and Critical Care Medicine Vanderbilt University Medical Center Nashville Tennessee USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam","family":"Jacob","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Cleveland Clinic Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ankit","family":"Desai","sequence":"additional","affiliation":[{"name":"Department of Medicine, College of Medicine The University of Arizona Tucson Arizona USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8242-8339","authenticated-orcid":false,"given":"Nicholas S.","family":"Hill","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, and Sleep Medicine Tufts Medical Center Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. Brett","family":"Larive","sequence":"additional","affiliation":[{"name":"Department of Quantitative Health Sciences Cleveland Clinic Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Emanuel","family":"Finet","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Cleveland Clinic Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane","family":"Leopold","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Brigham and Women's Hospital Harvard University Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evelyn","family":"Horn","sequence":"additional","affiliation":[{"name":"Division of Cardiology Weill Cornell University New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4128-3978","authenticated-orcid":false,"given":"Robert","family":"Frantz","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franz","family":"Rischard","sequence":"additional","affiliation":[{"name":"Department of Medicine, College of Medicine The University of Arizona Tucson Arizona USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serpil","family":"Erzurum","sequence":"additional","affiliation":[{"name":"Department of Inflammation and Immunity Cleveland Clinic Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerald","family":"Beck","sequence":"additional","affiliation":[{"name":"Department of Quantitative Health Sciences Cleveland Clinic Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen C.","family":"Mathai","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Medicine Johns Hopkins University Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul M.","family":"Hassoun","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Medicine Johns Hopkins University Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the PVDOMICS Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,8,21]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01889-2018"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202204-0731OC"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra040291"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00002-2022"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2009.04.018"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0735-1097(91)90556-O"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199207093270203"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00879-2022"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.104.488486"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0b013e31818193bb"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00090608"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.117.311737"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01913-2018"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.4103\/2045-8932.83449"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01032-2015"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2018.11.030"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2015.10.023"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehq170"},{"issue":"2","key":"e_1_2_10_20_1","first-page":"82","article-title":"Pulmonary vasoreactivity and phenotypes in pulmonary arterial hypertension associated to connective tissue diseases","volume":"70","author":"Hernandez\u2010Oropeza JL","year":"2018","journal-title":"Rev Invest Clin"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200510-1668OC"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0029-1233310"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2006.11.022"},{"issue":"11","key":"e_1_2_10_24_1","first-page":"2347","article-title":"Pulmonary veno\u2010occlusive disease and scleroderma associated pulmonary hypertension","volume":"33","author":"Johnson SR","year":"2006","journal-title":"J Rheumatol"}],"container-title":["Pulmonary Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pul2.12281","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,7]],"date-time":"2024-03-07T12:12:45Z","timestamp":1709813565000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pul2.12281"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,7]]},"references-count":23,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,7]]}},"alternative-id":["10.1002\/pul2.12281"],"URL":"https:\/\/doi.org\/10.1002\/pul2.12281","archive":["Portico"],"relation":{},"ISSN":["2045-8940","2045-8940"],"issn-type":[{"value":"2045-8940","type":"print"},{"value":"2045-8940","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,7]]},"assertion":[{"value":"2023-04-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-08-09","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-08-21","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e12281"}}