{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T15:15:54Z","timestamp":1787498154779,"version":"build-2736575974"},"reference-count":22,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2026,1,18]],"date-time":"2026-01-18T00:00:00Z","timestamp":1768694400000},"content-version":"vor","delay-in-days":2564,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2019,1,11]],"date-time":"2019-01-11T00:00:00Z","timestamp":1547164800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/3.0\/"}],"funder":[{"name":"Actelion Pharmaceuticals Ltd, Allschwil, Switzerland"},{"name":"Actelion Pharmaceuticals Ltd, Allschwil, Switzerland"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2019,3,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Patients with pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH) after defect correction have a poor prognosis compared with other CHD-PAH patients. Therefore, it is important that these patients are treated as early and effectively as possible. Evidence supporting the use of PAH therapies in patients with corrected CHD-PAH from randomised controlled trials is limited. The purpose of these analyses was to characterise the corrected CHD-PAH patients from the GRIPHON study and examine the response to selexipag.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>Out of the 110 patients diagnosed with corrected CHD-PAH, 55 had atrial septal defects, 38 had ventricular septal defects, 14 had persistent ducti arteriosus, and 3 had defects not further specified. Hazard ratios (HR) and 95% confidence intervals (CI) for the primary composite endpoint were calculated using Cox proportional hazard models. Compared with the non-CHD patients from GRIPHON, patients with corrected CHD-PAH were slightly younger, with a greater proportion being treatment-naive and in World Health Organization functional class I\/II. The rate of the primary composite endpoint of morbidity\/mortality was lower in patients with corrected CHD-PAH who were treated with selexipag compared with those treated with placebo (HR 0.58; 95% CI 0.25, 1.37). The most common adverse events were those known to be related to selexipag.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>These post-hoc analyses of GRIPHON provide valuable information about a large population of patients with corrected CHD-PAH, and suggest that selexipag may delay disease progression and was well-tolerated in patients with corrected CHD-PAH.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ejhf.1375","type":"journal-article","created":{"date-parts":[[2019,1,11]],"date-time":"2019-01-11T05:30:20Z","timestamp":1547184620000},"page":"352-359","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":55,"title":["Selexipag Treatment for Pulmonary Arterial Hypertension Associated with Congenital Heart Disease After Defect Correction: Insights from the Randomised Controlled Griphon Study"],"prefix":"10.1093","volume":"21","author":[{"given":"Maurice","family":"Beghetti","sequence":"first","affiliation":[{"name":"Pediatric Cardiology Unit, Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque P\u00e9diatrique, University of Geneva and Lausanne , Lausanne ,","place":["Switzerland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard N.","family":"Channick","sequence":"additional","affiliation":[{"name":"David Geffen School of Medicine, University of California Los Angeles , Los Angeles, CA ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly M.","family":"Chin","sequence":"additional","affiliation":[{"name":"Division of Internal Medicine, University of Texas Southwestern , Dallas, TX ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lilla","family":"Di Scala","sequence":"additional","affiliation":[{"name":"Actelion Pharmaceuticals Ltd , Allschwil ,","place":["Switzerland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sean","family":"Gaine","sequence":"additional","affiliation":[{"name":"National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital , Dublin ,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hossein-Ardeschir","family":"Ghofrani","sequence":"additional","affiliation":[{"name":"University of Giessen and Marburg Lung Center, Giessen, Germany, member of the German Center of Lung Research, and Department of Medicine, Imperial College London , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marius M.","family":"Hoeper","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Hannover Medical School and German Centre for Lung Research , Hannover ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene M.","family":"Lang","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna , Vienna ,","place":["Austria"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vallerie V.","family":"McLaughlin","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University of Michigan Health System , Ann Arbor, MI ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ralph","family":"Preiss","sequence":"additional","affiliation":[{"name":"Actelion Pharmaceuticals Ltd , Allschwil ,","place":["Switzerland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lewis J.","family":"Rubin","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of California , San Diego, CA ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00e9rald","family":"Simonneau","sequence":"additional","affiliation":[{"name":"APHP, Centre de R\u00e9f\u00e9rence de l'Hypertension Pulmonaire, Service de Pneumologie et Soins Intensifs, CHU Bic\u00eatre, Universit\u00e9 Paris-Sud, Le Kremlin-Bic\u00eatre , Paris ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Sitbon","sequence":"additional","affiliation":[{"name":"APHP, Centre de R\u00e9f\u00e9rence de l'Hypertension Pulmonaire, Service de Pneumologie et Soins Intensifs, CHU Bic\u00eatre, Universit\u00e9 Paris-Sud, Le Kremlin-Bic\u00eatre , Paris ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor F.","family":"Tapson","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Medicine, Cedars Sinai Medical Center , Los Angeles, CA 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