Ipertensione Arteriosa Polmonare Pediatrica dopo Riparazione di Defetto Cardiaco Congenito: Stratificazione del Rischio Ampliata e Outcomes in una Cohort Nazionale.

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% refer