{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T16:31:14Z","timestamp":1793809874151,"version":"4.6.1"},"reference-count":22,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"9","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation"],"published-print":{"date-parts":[[2026,9,1]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND:<\/jats:title>\n                    <jats:p>Risk prediction is fundamental to pulmonary hypertension (PH) guideline-based care, yet pediatric-specific risk prediction models remain limited, relying primarily on single predictors, expert opinion, or application of adult models to children. The authors developed and externally validated a data-driven 1-year risk prediction model for pediatric PH.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS:<\/jats:title>\n                    <jats:p>Pediatric patients with PH (n=345; World Symposium on Pulmonary Hypertension groups 1 and 3) enrolled in the Pediatric Pulmonary Hypertension Network Registry (2014\u20132020; 50.4% male; median age, 4.9 years [interquartile range, 1.9\u201310.3]) were split into training (80%) and test cohorts (20%). The Dutch National Registry for Pulmonary Hypertension in Childhood (n=155 [1993\u20132020]) and the Spanish Registry of Pediatric Pulmonary Hypertension (n=327 [2009\u20132023]) were used for external validation. From 176 variables, BorutaSHAP feature selection with random forest identified 16 predictors for a 1-year outcome of time to death, transplant, Potts shunt, or atrial septostomy, modeled using extreme gradient boosting. Performance was assessed with the area under the receiver operating characteristic curve, confusion matrices, calibration, and Kaplan-Meier event-free survival.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS:<\/jats:title>\n                    <jats:p>The final model achieved an area under the receiver operating characteristic curve of 0.90 (0.79\u20130.97) and 99% (96%\u201399%) negative predictive value in testing, dividing participants into 3 groups with strong outcome discrimination. External validation showed an area under the receiver operating characteristic curve of 0.76 (Dutch National Registry for Pulmonary Hypertension in Childhood, 0.70\u20130.81) and 0.77 (Spanish Registry of Pediatric Pulmonary Hypertension, 0.73\u20130.82) with negative predictive values of 93% (93%\u201397%) and 96% (93%\u201397%), respectively. Kaplan-Meier analysis significantly differentiated outcomes by risk group.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS:<\/jats:title>\n                    <jats:p>This multicenter, validated model provides good 1-year risk prediction in pediatric PH across World Symposium on Pulmonary Hypertension groups 1 and 3, providing a robust tool for clinical risk stratification to guide therapy and addressing a gap in pediatric PH care.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circulationaha.125.077391","type":"journal-article","created":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T09:00:16Z","timestamp":1786438816000},"page":"805-818","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["The Pediatric Pulmonary Hypertension International Risk Score: A Prediction Model for Outcomes Using Machine Learning"],"prefix":"10.1161","volume":"154","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8598-9078","authenticated-orcid":true,"given":"Megan","family":"Griffiths","sequence":"first","affiliation":[{"name":"Heart Center, Children\u2019s Health, Division of Cardiology, Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX (M.G.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8889-1084","authenticated-orcid":true,"given":"Bhargava K.","family":"Chinni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-4707-9352","authenticated-orcid":true,"given":"Chantal","family":"Lokhorst","sequence":"additional","affiliation":[{"name":"Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children\u2019s Hospital, University Medical Center Groningen, University of Groningen, The Netherlands (C. Lokhorst, J.M.D., R.M.F.B.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3146-5831","authenticated-orcid":true,"given":"Johannes M.","family":"Douwes","sequence":"additional","affiliation":[{"name":"Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children\u2019s Hospital, University Medical Center Groningen, University of Groningen, The Netherlands (C. Lokhorst, J.M.D., R.M.F.B.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8055-768X","authenticated-orcid":true,"given":"Lynn A.","family":"Sleeper","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Boston Children\u2019s Hospital and Department of Pediatrics, Harvard Medical School, MA (L.A.S., M.P.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-0771-6618","authenticated-orcid":true,"given":"Jennifer","family":"Tingo","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Children\u2019s Hospital of Philadelphia, PA (J.T.)."},{"name":"Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia (J.T.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7292-2085","authenticated-orcid":true,"given":"Steven H.","family":"Abman","sequence":"additional","affiliation":[{"name":"Pediatric Heart and Lung Center, Department of Pediatrics, University of Colorado, Aurora (S.H.A.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4849-214X","authenticated-orcid":true,"given":"Erika B.","family":"Rosenzweig","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Department of Pediatrics, Maria Fareri Children\u2019s Hospital at WMC Health, Valhalla, NY (E.B.R.)."},{"name":"Department of Pediatrics, New York Medical College, Valhalla, NY (E.B.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9112-7618","authenticated-orcid":true,"given":"Jennifer E.","family":"Schramm","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology and Critical Care Medicine, Division of Pediatric Cardiac Intensive Care, Johns Hopkins School of Medicine, Baltimore, MD (J.E.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1709-9022","authenticated-orcid":true,"given":"Eric D.","family":"Austin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0291-9630","authenticated-orcid":true,"given":"Mary P.","family":"Mullen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Boston Children\u2019s Hospital and Department of Pediatrics, Harvard Medical School, MA (L.A.S., M.P.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3575-2427","authenticated-orcid":true,"given":"Alba","family":"Torrent-Vernetta","sequence":"additional","affiliation":[{"name":"Pediatric Allergy and Pulmonology Section, Department of Pediatrics, Vall d\u2019Hebron Hospital Universitari, Vall d\u2019Hebron Barcelona Hospital Campus, Universitat Aut\u00f2noma de Barcelona, Spain (A.T.-V.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-4806-1891","authenticated-orcid":true,"given":"Carlos","family":"Labrandero","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology Department, \u201cLa Paz\u201d University Hospital, Madrid, Spain (C. 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