{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T13:43:42Z","timestamp":1792590222340,"version":"4.5.0"},"reference-count":23,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation"],"published-print":{"date-parts":[[2010,7,13]]},"abstract":"<jats:p>\n                    <jats:bold>\n                      <jats:italic>Background\u2014<\/jats:italic>\n                    <\/jats:bold>\n                    Factors that determine survival in pulmonary arterial hypertension (PAH) drive clinical management. A quantitative survival prediction tool has not been established for research or clinical use.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>\n                      <jats:italic>Methods and Results\u2014<\/jats:italic>\n                    <\/jats:bold>\n                    Data from 2716 patients with PAH enrolled consecutively in the US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) were analyzed to assess predictors of 1-year survival. We identified independent prognosticators of survival and derived a multivariable, weighted risk formula for clinical use. One-year survival from the date of enrollment was 91.0% (95% confidence interval [CI], 89.9 to 92.1). In a multivariable analysis with Cox proportional hazards, variables independently associated with increased mortality included pulmonary vascular resistance &gt;32 Wood units (hazard ratio [HR], 4.1; 95% CI, 2.0 to 8.3), PAH associated with portal hypertension (HR, 3.6; 95% CI, 2.4 to 5.4), modified New York Heart Association\/World Health Organization functional class IV (HR, 3.1; 95% CI, 2.2 to 4.4), men &gt;60 years of age (HR, 2.2; 95% CI, 1.6 to 3.0), and family history of PAH (HR, 2.2; 95% CI, 1.2 to 4.0). Renal insufficiency, PAH associated with connective tissue disease, functional class III, mean right atrial pressure, resting systolic blood pressure and heart rate, 6-minute walk distance, brain natriuretic peptide, percent predicted carbon monoxide diffusing capacity, and pericardial effusion on echocardiogram all predicted mortality. Based on these multivariable analyses, a prognostic equation was derived and validated by bootstrapping technique.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>\n                      <jats:italic>Conclusions\u2014<\/jats:italic>\n                    <\/jats:bold>\n                    We identified key predictors of survival based on the patient\u2019s most recent evaluation and formulated a contemporary prognostic equation. Use of this tool may allow the individualization and optimization of therapeutic strategies. Serial follow-up and reassessment are warranted.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>\n                      <jats:italic>Clinical Trial Registration\u2014<\/jats:italic>\n                    <\/jats:bold>\n                    URL: http:\/\/www.clinicaltrials.gov. Unique identifier: NCT00370214.\n                  <\/jats:p>","DOI":"10.1161\/circulationaha.109.898122","type":"journal-article","created":{"date-parts":[[2010,6,29]],"date-time":"2010-06-29T00:39:04Z","timestamp":1277771944000},"page":"164-172","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1397,"title":["Predicting Survival in Pulmonary Arterial Hypertension"],"prefix":"10.1161","volume":"122","author":[{"given":"Raymond L.","family":"Benza","sequence":"first","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dave P.","family":"Miller","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mardi","family":"Gomberg-Maitland","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert P.","family":"Frantz","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aimee J.","family":"Foreman","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher S.","family":"Coffey","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adaani","family":"Frost","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robyn J.","family":"Barst","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David B.","family":"Badesch","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. Gregory","family":"Elliott","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Theodore G.","family":"Liou","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael D.","family":"McGoon","sequence":"additional","affiliation":[{"name":"From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G..."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_2_1_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-115-5-343"},{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.06-2674"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehp022"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2004.02.037"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.130.4.1198"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(11)60769-6"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.2105\/AJPH.82.5.703"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1161\/circulationaha.104.488486"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1093\/biomet\/80.3.557"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/sim.1802"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1080\/01621459.1983.10477973"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0258(19960229)15:4<361::AID-SIM168>3.0.CO;2-4"},{"key":"e_1_3_2_13_2","first-page":"1","volume":"56","year":"2008","unstructured":"Kung HC, Hoyert DL, Xu J, Murphy SL. 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