Valvular aortic stenosis in children. Outcome, physical activity and health-related quality of life

Abstract

Introduction: Isolated valvular aortic stenosis (VAS) accounts for 3–5% of congenital heart disease and presents with a wide spectrum of severity. Critical VAS in the neonate is the most severe form, fatal in the absence of treatment. Physical activity is essential for normal development and is a prerequisite for long-term cardiovascular health. Health-related quality of life (HRQoL) and life satisfaction are important subjective outcome factors. Aim: To investigate survival and treatment outcome in neonates, children and adoles-cents treated for isolated VAS and in the children and adolescents, to study physical activity, HRQoL and life satisfaction. Methods: All patients were treated for VAS with surgical valvotomy as the preferred primary intervention. Patients were identified in surgical registries at the two Swedish paediatric surgical heart centres. A distinction was made between neonatal critical VAS (severe stenosis, with duct-dependent systemic circulation and/or depressed left ventricular function) and neonatal non-critical VAS. Data were collected from patient files and echocardiograms, with survival data from the Swedish Population Registry for long-term follow-up. Physical activity was measured objectively with accelerome-try. HRQoL was measured with KIDSCREEN-52, and life satisfaction was measured with the Satisfaction With Life Scale. Patients were matched with controls from the Swedish Population Registry. Results and conclusions: Mortality after treatment for VAS in neonates and children was low. Transplant-free survival was 96% in the paediatric cohort (n=113), 91% in neonates with critical VAS (n=65) and 98% in neonates with non-critical VAS (n=42) with median follow-up between 11 and 14 years. Reinterventions were common and performed in 38% of the paediatric patients, 58% of neonates with critical VAS and 33% of neonates with non-critical VAS (p=0.008). Both patients and controls fulfilled the WHO recommendations for physical activity to a high degree. Accelerometry revealed a different physical activity pattern in patients compared to controls. Patients rated their HRQoL and life satisfaction as similar to healthy controls. An unexpected finding was a negative association between high-intensity physical activity and psychological well-being and life satisfaction in adolescent patients.