BibTex format
@article{Constantine:2025:ehjqcco/qcaf150,
author = {Constantine, A and Dimopoulos, K and Condliffe, R and Clift, P and Jansen, K and Wort, SJ and Chaplin, G and Krishnathasan, K and Dhillon, R and Mookerjee, J and Knight, WB and Jones, CB and Forte, MNV and Wilson, D and Michael, H and Soto, AM and Labrandero, C and Ogando, AR and Galdó, AM and Rodríguez, IG and Cuenca, LMC and Perin, F and Bonora, AM and Ramón, ML and Albert, SV and Riezu, MÁI and López, JME and Del, Cerro Marín MJ and Moledina, S},
doi = {ehjqcco/qcaf150},
journal = {Eur Heart J Qual Care Clin Outcomes},
title = {Paediatric pulmonary arterial hypertension following congenital heart defect repair: Enhanced risk stratification and outcomes in a national cohort.},
url = {http://dx.doi.org/10.1093/ehjqcco/qcaf150},
year = {2025}
}
RIS format (EndNote, RefMan)
TY - JOUR
AB - 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.
AU - Constantine,A
AU - Dimopoulos,K
AU - Condliffe,R
AU - Clift,P
AU - Jansen,K
AU - Wort,SJ
AU - Chaplin,G
AU - Krishnathasan,K
AU - Dhillon,R
AU - Mookerjee,J
AU - Knight,WB
AU - Jones,CB
AU - Forte,MNV
AU - Wilson,D
AU - Michael,H
AU - Soto,AM
AU - Labrandero,C
AU - Ogando,AR
AU - Galdó,AM
AU - Rodríguez,IG
AU - Cuenca,LMC
AU - Perin,F
AU - Bonora,AM
AU - Ramón,ML
AU - Albert,SV
AU - Riezu,MÁI
AU - López,JME
AU - Del,Cerro Marín MJ
AU - Moledina,S
DO - ehjqcco/qcaf150
PY - 2025///
TI - Paediatric pulmonary arterial hypertension following congenital heart defect repair: Enhanced risk stratification and outcomes in a national cohort.
T2 - Eur Heart J Qual Care Clin Outcomes
UR - http://dx.doi.org/10.1093/ehjqcco/qcaf150
UR - https://www.ncbi.nlm.nih.gov/pubmed/41360749
ER -