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Interrupted Aortic Arch

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Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care

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Abstract

Interrupted aortic arch (IAA) is a rare congenital heart disease. It consists of a discontinuity between two segments of the aortic arch. The location of this interruption is the basis for a widely accepted classification in three types. IAA often has a genetic basis and usually occurs in association with other anomalies, especially ventricular septal defect. IAA is fatal in the neonatal period, if left untreated. The management was greatly improved with the introduction of prostaglandin E1, which allowed for preoperative stabilization. The surgical treatment has evolved from a staged approach to a primary surgical repair. The operative repair results have improved significantly in the last two decades. However, IAA remains a surgical challenge, in the acute outcomes and in the late recurrent aortic arch or left ventricular outflow tract obstruction.

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Notes

  1. 1.

    This chapter was based on our previous work: del Nido PJ, Geva T. Interrupted aortic arch. In: Gongora GD, Reyes NS, Velez Moreno JF, Angel GC. (Editors) Cardiologia Pediatrica. second Edition. McGraw-Hill 2018;489-501 (ISBN: 978-958-8813-70-7).

Abbreviations

CHD :

Congenital heart disease

CMR:

Cardiovascular magnetic resonance

CT:

Computed tomography

CV:

Cardiovascular

CVD:

Cardiovascular disease

ECG:

Electrocardiogram

HTN:

Hypertension

IAA:

Interrupted aortic arch

LV:

Left ventricle

LVOT:

Left ventricular outflow tract obstruction

VSD:

Ventricular septal defect

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Author information

Authors and Affiliations

  1. Department of Pediatric Cardiology, Hospital de Santa Marta, Centro Hospitalar de Lisboa Central, Rua de Santa Marta, Lisbon, Portugal

    José D. Martins & Conceição Trigo

  2. Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, MA, USA

    Ashwin Prakash & Tal Geva

  3. Department of Cardiothoracic Surgery & Transplantation, Hospital de Santa Marta, Centro Hospitalar de Lisboa Central, Rua de Santa Marta, Lisbon, Portugal

    José Fragata

Authors
  1. José D. Martins
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  2. Ashwin Prakash
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  3. Conceição Trigo
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  4. José Fragata
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  5. Tal Geva
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Editor information

Editors and Affiliations

  1. Children’s Hospital Colorado University of Colorado Denver, School of, Aurora, CO, USA

    Eduardo M. da Cruz

  2. Children's Hospital Colorado University of Colorado School of Medicin, Aurora, CO, USA

    Dunbar Ivy

  3. Department of Cardiac Surgery, German Pediatric Heart Center, Sankt Augustin, Germany

    Viktor Hraska

  4. Pediatric Cardiac Surgery, Children's Hospital Colorado University of Colorado, Aurora, CO, USA

    James Jaggers

Section Editor information

  1. The Heart Institute, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA

    Eduardo M. da Cruz

  2. The Heart Institute, Pediatric Cardiac Surgery, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA

    James Jaggers

  3. Cardiothoracic Surgery, University of Colorado School of Medicine The Children’s Hospital Pediatrics, Aurora, CO, USA

    James Jaggers

  4. Dept. Pediatric Cardiology, Children's Hospital Colorado, Univ. of Colorado, Denver, Aurora, Colorado, USA

    Dunbar Ivy

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Martins, J.D., Prakash, A., Trigo, C., Fragata, J., Geva, T. (2021). Interrupted Aortic Arch. In: da Cruz, E.M., Ivy, D., Hraska, V., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4999-6_28-2

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  • DOI: https://doi.org/10.1007/978-1-4471-4999-6_28-2

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  • Print ISBN: 978-1-4471-4999-6

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