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Congenital Aortic Valve Stenosis and Regurgitation

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Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care

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Abstract

Congenital aortic valve disease ranges from newborn critical aortic stenosis and shock to teenage asymptomatic aortic regurgitation and progressive ventricular dilatation. Echocardiography provides essential information about the aortic valve and left ventricle, while cardiac magnetic resonance imaging provides additional information about regurgitant fraction and ventricular volume.

There is general consensus on the management of older patients with aortic valve disease; however, decision-making is more complex for newborns. A team-based approach may provide the best results. Treatment strategies should prioritize quality of life and preservation of left ventricular function. Minimizing re-intervention is an important secondary priority.

Both balloon and open interventions are established and effective treatments for newborns with critical aortic stenosis. Re-intervention rates depend on original morphology. Open valvotomy with tricuspidization may offer the best long-term results.

Older children with aortic stenosis or regurgitation have several options. Repair can restore function and maintain growth potential. Although repair is associated with limited durability, it does preserve all future replacement options. Replacement can be autologous (Ross procedure), mechanical, or bioprosthetic. Limited durability of bioprosthetic valves and aortic homografts renders them secondary replacement options for most children. Younger patients requiring replacement can be excellent candidates for the Ross procedure. The supported Ross procedure may offer excellent long-term durability in the older age group.

This chapter strives to provide the reader with a broad understanding of congenital aortic valve disease by highlighting the “standard” treatment as well as controversies in the surgical management of this disease.

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Author information

Authors and Affiliations

  1. Herma Heart Institute, Children’s Hospital of Wisconsin, Milwaukee, WI, USA

    Viktor Hraška, Ronald K. Woods, Michael E. Mitchell, Susan R. Foerster & Benjamin H. Goot

  2. Division of Pediatric Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, WI, USA

    Viktor Hraška, Ronald K. Woods & Michael E. Mitchell

  3. Division of Pediatric Cardiology, Medical College of Wisconsin, Milwaukee, WI, USA

    Susan R. Foerster & Benjamin H. Goot

Authors
  1. Viktor Hraška
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  2. Ronald K. Woods
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  3. Michael E. Mitchell
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  4. Susan R. Foerster
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  5. Benjamin H. Goot
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Corresponding author

Correspondence to Viktor Hraška .

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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Hraška, V., Woods, R.K., Mitchell, M.E., Foerster, S.R., Goot, B.H. (2020). Congenital Aortic Valve Stenosis and Regurgitation. In: da Cruz, E., 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_23-2

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  • DOI: https://doi.org/10.1007/978-1-4471-4999-6_23-2

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  • Print ISBN: 978-1-4471-4999-6

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