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

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.; Soto, AM.; Labrandero, C.; Ogando, AR.; Galdo, AM.; Rodriguez, IG.; Cuenca, LMC.; Perin, F.; Bonora, AM.; Ramon, ML.; Albert, SV.; Riezu, MAI.; Lopez, JME.; Del Cerro Marin, MJ.; Moledina, S.

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

All Authors

Constantine, A.

Dimopoulos, K.

Condliffe, R.

Clift, P.

Jansen, K.

Wort, SJ.

Chaplin, G.

Krishnathasan, K.

Dhillon, R.

Mookerjee, J.

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LTHT Author

Michael, Helen

LTHT Department

Leeds Children's Hospital
Paediatric Cardiology

Publication Date

2025

Item Type

Journal Article

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.