{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T18:16:29Z","timestamp":1789236989389,"version":"build-2803163510"},"reference-count":25,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2025,12,8]],"date-time":"2025-12-08T00:00:00Z","timestamp":1765152000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,4,29]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD). PAH following CHD repair (repaired PAH-CHD) is an emerging population with increased morbidity and mortality. Existing PAH risk scores are unvalidated in young children with this condition.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>A 20-year cohort from the UK National Paediatric PH Service was studied to describe peri-operative characteristics and outcomes in children with repaired PAH-CHD and to create a tailored risk stratification tool. This tool was externally validated using the national Spanish PH (REHIPED) registry. The study included 178 patients (median age 3.2 years, 58.4% female), with 73.0% referred post-CHD repair. Complex CHD was present in 61.2%, and 48.9% had both pre- and post-tricuspid shunts. Down syndrome was noted in 33.1%. At initial post-operative assessment, 53.1% exhibited symptoms like breathlessness, 30.9% had moderate\u2013severe right ventricular dilatation, and 23.7% showed right ventricular systolic impairment. During a median six-year follow-up of 156 patients, 19.2% died and 3.2% required lung transplantation, with survival rates at one, five, and ten years being 94.7%, 85.9%, and 80.1%, respectively. The developed risk score, based on clinical variables including absence of pre-operative PH, breathlessness, right ventricular dysfunction, and pulmonary vascular resistance index, showed good performance and calibration in predicting outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In this national cohort of children with repaired PAH-CHD, mortality is significant. This novel, simple risk score has been developed and validated specifically for children with repaired PAH-CHD, useful at the time of post-operative assessment to predict outcome and direct management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ehjqcco\/qcaf150","type":"journal-article","created":{"date-parts":[[2025,12,5]],"date-time":"2025-12-05T13:09:19Z","timestamp":1764940159000},"page":"325-335","source":"Crossref","is-referenced-by-count":4,"title":["Paediatric pulmonary arterial hypertension following congenital heart defect repair: enhanced risk stratification and outcomes in a national cohort"],"prefix":"10.1093","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7899-463X","authenticated-orcid":false,"given":"Andrew","family":"Constantine","sequence":"first","affiliation":[{"name":"National Paediatric Pulmonary Hypertension Service UK, Great Ormond Street Hospital for Children NHS Foundation Trust , Great Ormond Street, London WC1N 3JH ,","place":["UK"]},{"name":"Adult Congenital Heart Disease Unit, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust , Birmingham B15 2GW ,","place":["UK"]},{"name":"Department of Cardiovascular Sciences, University of Birmingham , Birmingham B15 2TT ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1300-0504","authenticated-orcid":false,"given":"Konstantinos","family":"Dimopoulos","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield NHS Foundation Trust , London SW3 6NP ,","place":["UK"]},{"name":"National Heart and Lung Institute, Imperial College London , London SW3 6LY ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robin","family":"Condliffe","sequence":"additional","affiliation":[{"name":"Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital , Sheffield S10 2JF ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8906-7647","authenticated-orcid":false,"given":"Paul","family":"Clift","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Unit, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust , Birmingham B15 2GW ,","place":["UK"]},{"name":"Department of Cardiovascular Sciences, University of Birmingham , Birmingham B15 2TT ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katrijn","family":"Jansen","sequence":"additional","affiliation":[{"name":"Adult Congenital and Paediatric Heart Unit, Freeman Hospital Newcastle Upon Tyne Hospitals NHS Foundation Trust , Newcastle Upon Tyne NE7 7DN ,","place":["UK"]},{"name":"Population Health Sciences Institute, Newcastle University , Newcastle upon Tyne NE2 4AX ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S John","family":"Wort","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, Royal Brompton & Harefield NHS Foundation Trust , London SW3 6NP ,","place":["UK"]},{"name":"National Heart and Lung Institute, Imperial College London , London SW3 6LY ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grace","family":"Chaplin","sequence":"additional","affiliation":[{"name":"UCL Institute of Cardiovascular Science, University College London , Gower Street, London WC1E 6BT ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7097-6012","authenticated-orcid":false,"given":"Kaushiga","family":"Krishnathasan","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Unit, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust , Birmingham B15 2GW ,","place":["UK"]},{"name":"Department of Cardiovascular Sciences, University of Birmingham , Birmingham B15 2TT ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rami","family":"Dhillon","sequence":"additional","affiliation":[{"name":"Birmingham Children\u2019s Hospital, Birmingham Women\u2019s and Children\u2019s NHS Foundation Trust , Birmingham B4 6NH ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joydeep","family":"Mookerjee","sequence":"additional","affiliation":[{"name":"Royal Manchester Children\u2019s Hospital, Manchester University NHS Foundation Trust , Manchester M13 9WL ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W Brodie","family":"Knight","sequence":"additional","affiliation":[{"name":"Royal Hospital for Children, NHS Greater Glasgow and Clyde , Glasgow G51 4TF, Scotland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline B","family":"Jones","sequence":"additional","affiliation":[{"name":"Alder Hey Children\u2019s Hospital, Alder Hey Children\u2019s NHS Foundation Trust , Liverpool L12 2AP ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria N Velasco","family":"Forte","sequence":"additional","affiliation":[{"name":"Bristol Royal Hospital for Children, University Hospitals Bristol and Weston NHS Foundation Trust , Bristol BS2 8BJ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dirk","family":"Wilson","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, University Hospital of Wales , Cardiff CF14 4XW ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen","family":"Michael","sequence":"additional","affiliation":[{"name":"Leeds General Infirmary, The Leeds Teaching Hospitals NHS Trust , Leeds LS1 3EX ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Mendoza Soto","sequence":"additional","affiliation":[{"name":"Pediatric Heart Institute, University Hospital 12 de Octubre , Madrid 28041 ,","place":["Spain"]},{"name":"Instituto de Investigaci\u00f3n Sanitaria Hospital 12 de Octubre , Madrid 28041 ,","place":["Spain"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Labrandero","sequence":"additional","affiliation":[{"name":"Servicio de Cardiolog\u00eda Infantil, Hospital Universitario La Paz , Madrid 28046 ,","place":["Spain"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alejandro","family":"Rodr\u00edguez Ogando","sequence":"additional","affiliation":[{"name":"Unidad de Cardiopat\u00edas Cong\u00e9nitas (UCC), Servicio de Cardiolog\u00eda Infantil, Hospital Universitario Gregorio Mara\u00f1\u00f3n , Madrid 28007 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