Abstract
BACKGROUND: Studies of outcome after operative correction of truncus arteriosus communis (TA) have focused on mortality and rates of reintervention. 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 reintervention and use 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 reintervention, and noncardiac operations was highest in the first year of life. Combined catheter-based and operative reintervention 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 subjects, and truncal valve replacement occurred in 8% of subjects. Maximal oxygen consumption (P = .0002), maximal work (P < .0001), and forced vital capacity (P < .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 comorbid disease throughout childhood, significant burden of operative and catheter-based reintervention, and deficits in exercise performance, functional status, and health-related quality of life.