Functional outcomes in children with anatomically repaired transposition of the great arteries with regard to congenital ventricular septal defect and coronary pattern

摘要

Despite decreasing mortality, functional impairments in children with transposition of the great arteries (TGA) are still a concern. This study analyses health-related physical fitness (HRPF), arterial stiffness and health-related quality of life (HRQoL) in children with anatomically repaired TGA regarding congenital ventricular septal defect (VSD) and coronary patterns.68 children with anatomically repaired TGA with or without VSD (12.9±3.7 years, 19.1% female) were investigated between August 2014 and October 2017. HRPF was assessed by five tests of the FITNESSGRAM, arterial stiffness was measured by oscillometric measurement using Mobil-O-Graph and HRQoL was analysed with a self-report questionnaire (KINDL-R). All test results were compared with a healthy reference cohort (n=2116, 49.1% female) adjusted for sex and age.Children with anatomically repaired TGA had significantly worse HRPF (z-score: ?0.58±0.81, p<0.001), increased pulse wave velocity (TGA: 4.9±0.3 m/s vs healthy: 4.8±0.3 m/s, p=0.028) and central systolic blood pressure (TGA: 105.9±5.8?mm Hg vs healthy: 103.3±5.7?mm Hg, p=0.001). No difference was found for HRQoL between the two groups (total HRQoL score: TGA: 76.5±10.2?vs healthy: 75.2±10.1, p=0.315). Neither the coronary pattern nor a congenital VSD resulted in significant differences in all functional outcomes.Children with anatomically repaired TGA exhibit impaired HRPF and increased arterial stiffness whereas their HRQoL is normal. The underlying coronary pattern seems to have no influence on the functional outcome, nor does an accompanied congenital VSD.