{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T01:54:14Z","timestamp":1791942854141,"version":"4.3.4"},"reference-count":33,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"9","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2022,5,3]]},"abstract":"<jats:sec xml:lang=\"en\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p xml:lang=\"en\">Current mortality data for pulmonary arterial hypertension (PAH) in the United States are based on registries that enrolled patients prior to 2010. We sought to determine mortality in PAH in the modern era using the PHAR (Pulmonary Hypertension Association Registry).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Methods and Results<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      We identified all adult patients with PAH enrolled in the PHAR between September 2015 and September 2020 (N=935). We used Kaplan\u2010Meier survival analysis and Cox proportional hazards models to assess mortality at 1, 2, and 3 years. Patients were stratified based on disease severity by 3 validated risk scores. In treatment\u2010na\u00efve patients, we compared survival based on initial treatment strategy. The median age was 56 years (44\u201368 years), and 76% were women. Of the 935 patients, 483 (52%) were \u22646\u00a0months from PAH diagnosis. There were 121 deaths (12.9%) during a median follow\u2010up time of 489 days (281\u2013812 days). The 1\u2010, 2\u2010, and 3\u2010year mortality was 8% (95% CI, 6%\u201310%), 16% (95% CI, 13%\u201319%), and 21% (95% CI, 17%\u201325%), respectively. When stratified into low\u2010, intermediate\u2010, and high\u2010risk PAH, the mortality at 1, 2, and 3 years was 1%, 4% to 6%, and 7% to 11% for low risk; 7% to 8%, 11% to 16%, and 18% to 20% for intermediate risk; and 12% to 19%, 22% to 38%, and 28% to 55% for high risk, respectively. In treatment\u2010na\u00efve patients, initial combination therapy was associated with better 1\u2010year survival (adjusted hazard ratio, 0.43 [95% CI, 0.19\u20130.95];\n                      <jats:italic>P<\/jats:italic>\n                      =0.037).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p xml:lang=\"en\">Mortality in the intermediate\u2010 and high\u2010risk patients with PAH remains unacceptably high in the PHAR, suggesting the importance for early diagnosis, aggressive use of available therapies, and the need for better therapeutics.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/jaha.121.024969","type":"journal-article","created":{"date-parts":[[2022,4,27]],"date-time":"2022-04-27T05:43:27Z","timestamp":1651038207000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":202,"title":["Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry"],"prefix":"10.1161","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7477-5763","authenticated-orcid":false,"given":"Kevin Y.","family":"Chang","sequence":"first","affiliation":[{"name":"Department of Medicine University of Minnesota Minneapolis MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1025-8665","authenticated-orcid":false,"given":"Sue","family":"Duval","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Minnesota Minneapolis MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David B.","family":"Badesch","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Colorado Denver CO"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Todd M.","family":"Bull","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Colorado Denver CO"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Murali M.","family":"Chakinala","sequence":"additional","affiliation":[{"name":"Department of Medicine Washington University St. Louis MO"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0183-4704","authenticated-orcid":false,"given":"Teresa","family":"De Marco","sequence":"additional","affiliation":[{"name":"Department of Medicine University of California San Francisco San Francisco CA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4128-3978","authenticated-orcid":false,"given":"Robert P.","family":"Frantz","sequence":"additional","affiliation":[{"name":"Department of Medicine Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2755-5845","authenticated-orcid":false,"given":"Anna","family":"Hemnes","sequence":"additional","affiliation":[{"name":"Department of Medicine Vanderbilt University Nashville TN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3188-7209","authenticated-orcid":false,"given":"Stephen C.","family":"Mathai","sequence":"additional","affiliation":[{"name":"Department of Medicine Johns Hopkins University Baltimore MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erika Berman","family":"Rosenzweig","sequence":"additional","affiliation":[{"name":"Department of Medicine Columbia University New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9203-5512","authenticated-orcid":false,"given":"John J.","family":"Ryan","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Utah Salt Lake City UT"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6210-0467","authenticated-orcid":false,"given":"Thenappan","family":"Thenappan","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Minnesota Minneapolis MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roblee","family":"Allen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonja","family":"Bartolome","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymond","family":"Benza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Cadaret","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Eggert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean","family":"Elwing","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey","family":"Fineman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymond","family":"Foley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. James","family":"Ford","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russel","family":"Hirsch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Grinnan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. Dunbar","family":"Ivy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven","family":"Kawut","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamie","family":"Kennedy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Klinger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Leary","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sula","family":"Mazimba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gautam","family":"Ramani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amresh","family":"Raina","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Runo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Swisher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nidhy","family":"Varghese","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. James","family":"White","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy","family":"Williamson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delphine","family":"Yung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roham","family":"Zamanian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dianne","family":"Zwicke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.j5492"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.11-1460"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.15-0300"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.09-1140"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00072709"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-1293"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.112.124388"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1213917"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1503184"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1209655"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1413687"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehv317"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.018435"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.113.004526"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00740-2017"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehx257"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00889-2017"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.21693\/1933-088X-16.4.185"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202006-612OC"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2020.08.2069"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2019.02.004"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00196611"},{"key":"e_1_3_2_24_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00057010"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00092306"},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-115-5-343"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.898122"},{"key":"e_1_3_2_28_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00094112"},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199602013340504"},{"key":"e_1_3_2_30_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202009-3698OC"},{"key":"e_1_3_2_31_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2017.08.009"},{"key":"e_1_3_2_32_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01897-2018"},{"key":"e_1_3_2_33_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01552-2021"},{"key":"e_1_3_2_34_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201705-0943OC"}],"container-title":["Journal of the American Heart Association"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/JAHA.121.024969","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,6]],"date-time":"2025-02-06T03:08:59Z","timestamp":1738811339000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/JAHA.121.024969"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,5,3]]},"references-count":33,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2022,5,3]]}},"alternative-id":["10.1161\/JAHA.121.024969"],"URL":"https:\/\/doi.org\/10.1161\/jaha.121.024969","relation":{},"ISSN":["2047-9980"],"issn-type":[{"value":"2047-9980","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,5,3]]},"assertion":[{"value":"2021-12-17","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-03-24","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-04-27","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e024969"}}