Paediatric pulmonary arterial hypertension following congenital heart defect repair: enhanced risk stratification and outcomes in a national cohort
Constantine, Andrew Dimopoulos, Konstantinos Condliffe, Robin Clift, Paul Jansen, Katrijn Wort, S John Chaplin, Grace Krishnathasan, Kaushiga Dhillon, Rami Mookerjee, Joydeep
Constantine, Andrew
Dimopoulos, Konstantinos
Condliffe, Robin
Clift, Paul
Jansen, Katrijn
Wort, S John
Chaplin, Grace
Krishnathasan, Kaushiga
Dhillon, Rami
Mookerjee, Joydeep
Abstract
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.
MIDER Authors
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Date
2026-04-29
Type
Article
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Citation
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, Rodríguez Ogando A, Moreno-Galdó A, Guillén Rodríguez I, Cuenca LMC, Perin F, Bonora AM, López Ramón M, Albert SV, Riezu MÁI, Espín López JM, Del Cerro Marín MJ, Moledina S. Paediatric pulmonary arterial hypertension following congenital heart defect repair: enhanced risk stratification and outcomes in a national cohort. Eur Heart J Qual Care Clin Outcomes. 2026 Apr 29;12(3):325-335. doi: 10.1093/ehjqcco/qcaf150.