Background
Studies of outcome following operative correction of truncus arteriosus communis (TA) have focused on mortality and rates of re-intervention. We sought to investigate the clinical status of children and adolescents with surgically corrected TA.
Methods and Results
A cross-sectional study of subjects with TA was performed. Subjects underwent concurrent genetic testing, electrocardiogram, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and completed questionnaires assessing health status and health-related quality of life. Review of their medical history provided retrospective information on cardiac re-intervention and utilization of medical care. Twenty-five subjects with a median age of 11.8 (8.1-18.99) years were enrolled. The prevalence of 22q11.2 deletion was 32%. Incidence of hospitalization, cardiac re-intervention, and non-cardiac operations was highest in the first year of life. Combined catheter-based and operative re-intervention rates were 52% on the conduit and 56% on the pulmonary arteries. Right ventricular ejection fraction and end diastolic volume were normal. Moderate or greater truncal valve insufficiency was seen in 11% of subject, and truncal valve replacement occurred in 8% of subjects. Maximal oxygen consumption (p=0002), maximal work (p<0.0001), and forced vital capacity (p<0.0001) were all lower than normal for age and sex. Physical health status and health-related quality of life were both severely diminished.
Conclusion
Patients with TA demonstrate significant co-morbid disease throughout childhood, significant burden of operative and catheter-based re-intervention, and deficits in exercise performance, functional status, and health-related quality of life.