title: Morbidity in children and adolescents after surgical correction of interrupted aortic arch.
abstract: 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.
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, 34th St and Civic Center Blvd, Philadelphia, PA, 19104, USA, obyrnem@email.chop.edu.
authors.forename: Michael L
authors.initials: ML
authors.lastname: O'Byrne
authors.forename: Laura
authors.initials: L
authors.lastname: Mercer-Rosa
authors.forename: Huaqing
authors.initials: H
authors.lastname: Zhao
authors.forename: Xuemei
authors.initials: X
authors.lastname: Zhang
authors.forename: Wei
authors.initials: W
authors.lastname: Yang
authors.forename: Ronn E
authors.initials: RE
authors.lastname: Tanel
authors.forename: Bradley S
authors.initials: BS
authors.lastname: Marino
authors.forename: Amy
authors.initials: A
authors.lastname: Cassedy
authors.forename: Mark A
authors.initials: MA
authors.lastname: Fogel
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
authors.forename: Stephen
authors.initials: S
authors.lastname: Paridon
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Goldmuntz
mesh_terms: D000293:Adolescent; D001017:Aortic Coarctation / Q000601:surgery*; D006328:Cardiac Catheterization; D002648:Child; D003430:Cross-Sectional Studies; D004562:Electrocardiography; D005080:Exercise Test; D005260:Female; D006305:Health Status Indicators; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009017:Morbidity; D011788:Quality of Life; D012086:Reoperation; D016896:Treatment Outcome
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