Functional outcome in contemporary children with total cavopulmonary connection – Health-related physical fitness, exercise capacity and health-related quality of life

Objective: Children and adolescents with an univentricular heart after total cavopulmonary connection (TCPC) have functional impairments. This study assesses health-related physical fitness (HRPF) and exercise capacity, as well as their relation to health-related quality of life (HRQoL) in patients with an univentricular heart after total-pulmonary connection (TCPC). Patients and methods: Between July 2014 and October 2016 a total of 78 children and adolescents with TCPC (12.0 ± 3.2 years, 21 female) performed a motor test including five tasks for strength and flexibility during their routine follow-up appointment. They also underwent a symptom limited cardio-pulmonary exercise test and filled in a HRQoL questionnaire (KINDL-R). Patients' data were compared to a recent sample of healthy children (n = 1650, 12.6 ± 2.4 years, 49% female). Results: Multivariable regressions corrected for sex, age and BMI showed that TCPC patients achieved 12.4 repetitions of curl-ups (p < 0.001) and 2.6 push-ups (p = 0.010) less than healthy counterparts. They had impairments in trunk (− 8.5 cm; p < 0.001), shoulder (− 7.5 cm; p < 0.001) and lower limb flexibility (− 4.7 cm; p < 0.001). Peak oxygen uptake was reduced to 34.8 ± 7.5 ml/min/kg and 77.7% respectively, compared to peers (p < 0.001). Ventilatory efficiency was also impaired (healthy: 27.5 ± 2.9 vs. TCPC: 31.6 ± 3.3; p < 0.001). HRQoL did not differ significantly (p = 0.233). Conclusions: Children and adolescents with TCPC still present impaired HRPF and exercise capacity whereas HRQoL is similar to healthy peers. Since low HRPF may yield to worse motor competence and exercise capacity, early screening for HRPF and early treatment, if indicated, is recommended.