Background
Previous studies of outcome following operative correction of interrupted aortic arch (IAA) focus on mortality and rates of re-intervention. We sought to investigate the clinical status of children and adolescents following surgery for interrupted aortic arch (IAA).
Methods
A cross-sectional study of subjects with IAA between the ages of 8-18 years was performed with subject undergoing concurrent genetic testing, electrocardiogram, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and assessment of health status and health-related quality of life as well as concurrent retrospective cohort study reviewing their post-operative utilization of medical care including operative and trans-catheter reintervention, non-cardiac operations, and hospitalizations.
Results
21 subjects with IAA with median age of 9 years were studied. Re-intervention rates were 38% on the left ventricular outflow tract, 33% for the aortic arch, and 24% for both. Rates of re-intervention were highest in the first year of life and decreased in subsequent years. Left ventricular ejection fraction was preserved (72±6%). Maximal oxygen consumption, maximal work, and forced vital capacity were both significantly diminished from age and gender norms (p<0.0001). Health status and quality of life were both severely diminished.
Conclusion
Subjects with IAA demonstrate a significant burden of operative and trans-catheter intervention and large magnitude deficits in exercise performance, health status, and health related quality of life.