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Interrupted Aortic Arch

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Surgery of Conotruncal Anomalies

Abstract

Interrupted aortic arch (IAA), usually associated with a ventricular septal defect (VSD), is a rapidly fatal congenital heart anomaly with ductus-dependent systemic circulation. Three types of IAA – A, B and C – are classicaly recognized. Only type B is, but not exclusively, a true cono-truncal anomaly. The critical condition of these neonates dictates early referral and surgical treatment. Careful preoperative assessment, and particularly the identification of a severe left ventricular outflow tract obstruction (LVOTO, <4 mm), is crucial for a successful outcome. DiGeorge syndrome is another frequently associated feature with important clinical implications. Many surgical options are available. Staged repair was popular in the past, but primary repair through a median sternotomy (using either deep hypothermic circulatory arrest or moderately hypothermic antegrade selective cerebral perfusion) is currently the preferred approach. Aortic arch reconstruction is accomplished by end-to-side anastomosis with the ascending aorta, more or less associated with patch augmentation of the anastomosis. A severe LVOTO may require more aggressive procedures, e.g. Yasui or Ross-Konno operations. Current early mortality for repair of IAA with VSD ranges from 4 to 10 %. Fifteen-year survival is about 60 %. Recurrence of arch obstruction has been reported from 10 to 30 %, while recurrence of LVOTO has averaged 20 %. Anatomic features affect mortality and initial LVOT procedures, whereas characteristics of the arch repair affect arch reintervention.

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Author information

Authors and Affiliations

  1. Pediatric Cardiac Surgery, Prince Sultan Cardiac Center, Riyadh, Saudi Arabia

    Roberto M. Di Donato MD

  2. Department of Pediatric Cardiac Surgery, National Heart and Lung Institute, Royal Brompton Hospital, Imperial College University, Sydney Street, London, SW3 6NP, UK

    Francois Lacour-Gayet MD

Authors
  1. Roberto M. Di Donato MD
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  2. Francois Lacour-Gayet MD
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Corresponding author

Correspondence to Roberto M. Di Donato MD .

Editor information

Editors and Affiliations

  1. Royal Brompton Hospital, London, United Kingdom

    Francois Lacour-Gayet

  2. C.S. Mott Children’s Hospital, University of Michigan, Ann Arbor, Michigan, USA

    Edward L. Bove

  3. German Pediatric Heart Center, Asklepios Klinik Sankt Augustin Gmb, Sankt Augustin, Germany

    Viktor Hraška

  4. University of Pittsburgh Medical School, Pittsburgh, Pennsylvania, USA

    Victor O. Morell

  5. Department of Cardiothoracic Surgery, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

    Thomas L. Spray

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Di Donato, R.M., Lacour-Gayet, F. (2016). Interrupted Aortic Arch. In: Lacour-Gayet, F., Bove, E., Hraška, V., Morell, V., Spray, T. (eds) Surgery of Conotruncal Anomalies. Springer, Cham. https://doi.org/10.1007/978-3-319-23057-3_33

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  • DOI: https://doi.org/10.1007/978-3-319-23057-3_33

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-23056-6

  • Online ISBN: 978-3-319-23057-3

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