Andrew Constantine, Konstantinos Dimopoulos, Robin Condliffe, Paul Clift, Katrijn Jansen, S. John Wort, Grace Chaplin, Kaushiga Krishnathasan, Rami Dhillon, Joydeep Mookerjee, W. Brodie Knight, Caroline B. Jones, Maria N. Velasco Forte, Dirk Wilson, Helen Michael, Alberto Mendoza Soto, Carlos Labrandero, Alejandro Rodríguez Ogando, Antonio Moreno Galdó, Inmaculada Guillén Rodríguez, Lina M. Caicedo Cuenca, Francesca Perin, Amparo Moya Bonora, Marta López Ramón, Sandra Villagrá Albert, M. Ángeles Izquierdo Riezu, Juana M. Espín López, María J. Del Cerro Marín, Shahin Moledina
NHS Foundation Trust. Great Ormond Street Hospital for Children NHS Foundation Trust. Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham and University of Birmingham. Royal Brompton Hospital. Imperial College London. Royal Hallamshire Hospital. Freeman Hospital Newcastle Upon Tyne Hospitals and Newcastle University. University College London. Birmingham Children’s Hospital. Royal Manchester Children’s Hospital. Royal Hospital for Children. Alder Hey Children’s Hospital. Bristol Royal Hospital for Children and University Hospitals Bristol and Weston. University Hospital of Wales. Leeds General Infirmary and Leeds Teaching Hospitals. University Hospital 12 de Octubre and Instituto de Investigación Sanitaria Hospital 12 de Octubre. Hospital Universitario La Paz. Hospital Universitario Gregorio Marañón. Hospital Universitario Vall d´Hebron. Hospital Universitario Virgen del Rocío. Clínica Shaio. Hospital Universitario Virgen de las Nieves. Hospital Universitario y Politécnico La Fe. Hospital Universitario Miguel Servet. Unidad de Cardiopatías Congénitas. Hospital Universitario Donostia. Hospital Clínico Universitario Virgen de la Arrixaca. Hospital Universitario Ramón y Cajal.
United Kingdom, Spain and Columbia
European Heart Journal Quality of Care and Clinical Outcomes
Eur Heart J Qual Care Clin Outcomes 2025;
DOI: 10.1093/ehjqcco/qcaf150
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.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Heart Dysfunction Associated with Pulmonary Vascular Disease (Right)
Genetic Factors Associated with Pulmonary Vascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Risk Stratification
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
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