Morbidity in children and adolescents after surgical correction of interrupted aortic arch

Michael L. O'Byrne*, Laura Mercer-Rosa, Huaqing Zhao, Xuemei Zhang, Wei Yang, Ronn E. Tanel, Bradley S. Marino, Amy Cassedy, Mark A. Fogel, Jack Rychik, Stephen Paridon, Elizabeth Goldmuntz

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

13 Scopus citations


Previous studies of outcome after operative correction of interrupted aortic arch (IAA) have focused on mortality and rates of reintervention. We sought to investigate the clinical status of children and adolescents after surgery for IAA. A cross-sectional study of subjects with IAA between the ages of 8 and 18 years was performed with the subjects undergoing simultaneous 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 postoperative use of medical care, including operative and transcatheter reinterventions, noncardiac surgeries, and hospitalizations. Twenty-one subjects with IAA with median age of 9 years were studied. Reintervention rates were 38 % for left-ventricular outflow tract, 33 % for AA, and 24 % for both. Rates of reintervention 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 decreased from age and sex norms (p < 0.0001). Health status and quality of life were both severely decreased. Subjects with IAA demonstrate a significant burden of operative and transcatheter intervention and large magnitude deficits in exercise performance, health status, and health-related quality of life.

Original languageEnglish (US)
Pages (from-to)386-392
Number of pages7
JournalPediatric cardiology
Issue number3
StatePublished - Mar 2014


  • Cardiac MRI
  • Cardiopulmonary exercise testing
  • Congenital heart disease
  • Quality of life

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health


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