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Lookup NU author(s): Dr Katrijn Jansen
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© The Author(s) 2025. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. Aims: 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. Methods and results: 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–severe 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. Conclusion: 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.
Author(s): Constantine A, Dimopoulos K, Condliffe R, Clift P, Jansen K, Wort SJ, Chaplin G, Krishnathasan K, Dhillon R, Mookerjee J, Knight WB, Jones CB, Forte MNV, Wilson D, Michael H, Mendoza Soto A, Labrandero C, Rodriguez Ogando A, Moreno-Galdo A, Guillen Rodriguez I, Cuenca LMC, Perin F, Bonora AM, Lopez Ramon M, Albert SV, Riezu MAI, Espin Lopez JM, Del Cerro Marin MJ, Moledina S
Publication type: Article
Publication status: Published
Journal: European Heart Journal - Quality of Care and Clinical Outcomes
Year: 2026
Volume: 12
Issue: 3
Pages: 325-335
Print publication date: 01/05/2026
Online publication date: 08/12/2025
Acceptance date: 02/12/2025
ISSN (print): 2058-5225
ISSN (electronic): 2058-1742
Publisher: Oxford University Press
URL: https://doi.org/10.1093/ehjqcco/qcaf150
DOI: 10.1093/ehjqcco/qcaf150
PubMed id: 41360749
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