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. 2025 Dec 8:qcaf150.
doi: 10.1093/ehjqcco/qcaf150. Online ahead of print.

Paediatric pulmonary arterial hypertension following congenital heart defect repair: Enhanced risk stratification and outcomes in a national cohort

Andrew Constantine  1   2   3 , Konstantinos Dimopoulos  4   5 , Robin Condliffe  6 , Paul Clift  2   3 , Katrijn Jansen  7   8 , S John Wort  4   5 , Grace Chaplin  9 , Kaushiga Krishnathasan  2   3 , Rami Dhillon  10 , Joydeep Mookerjee  11 , W Brodie Knight  12 , Caroline B Jones  13 , Maria N Velasco Forte  14 , Dirk Wilson  15 , Helen Michael  16 , Alberto Mendoza Soto  17 , Carlos Labrandero  18 , Alejandro Rodríguez Ogando  19 , Antonio Moreno Galdó  20 , Inmaculada Guillén Rodríguez  21 , Lina M Caicedo Cuenca  22 , Francesca Perin  23 , Amparo Moya Bonora  24 , Marta López Ramón  25 , Sandra Villagrá Albert  26 , M Ángeles Izquierdo Riezu  27 , Juana M Espín López  28 , María J Del Cerro Marín  29 , Shahin Moledina  1   9
Affiliations

Paediatric pulmonary arterial hypertension following congenital heart defect repair: Enhanced risk stratification and outcomes in a national cohort

Andrew Constantine et al. Eur Heart J Qual Care Clin Outcomes. .

Abstract

Background and 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: 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.

Results: 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.

Conclusions: 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.

Keywords: Paediatric cardiology; external validation; observational study; pulmonary hypertension; risk score development.

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