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Cardiac, Aortic, and Pulmonary Vascular Involvement in Alagille Syndrome

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Alagille Syndrome

Abstract

Congenital heart disease is present in over 75% of patients with Alagille syndrome. In fact, stenosis and/or hypoplasia of the branch pulmonary arteries, the most common cardiovascular abnormality, is one of the five elements used in the clinical diagnosis of this syndrome. Other commonly occurring lesions include tetralogy of Fallot, pulmonary valve stenosis or atresia, valvar or supravalvar aortic stenosis, and aortic coarctation. The peripheral vasculature is frequently affected as well. In this chapter, we discuss in detail the spectrum and incidence of congenital cardiovascular disease in patients with Alagille syndrome, as well as recommended diagnostic approaches and findings, surveillance, management, and outcomes.

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Author information

Authors and Affiliations

  1. The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Justin T. Tretter

  2. Departments of Cardiothoracic Surgery and Pediatrics (Cardiology), Stanford University Medical School, Lucile Packard Children’s Hospital, Betty Irene Moore Children’s Heart Center, Clinical and Translational Research Program, Palo Alto, CA, USA

    Doff B. McElhinney

Authors
  1. Justin T. Tretter
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  2. Doff B. McElhinney
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Correspondence to Doff B. McElhinney .

Editor information

Editors and Affiliations

  1. Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, ON, Canada

    Binita M. Kamath

  2. Division of Gastroenterology, Hepatology and Nutrition, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Kathleen M. Loomes

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Tretter, J.T., McElhinney, D.B. (2018). Cardiac, Aortic, and Pulmonary Vascular Involvement in Alagille Syndrome. In: Kamath, B., Loomes, K. (eds) Alagille Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-319-94571-2_6

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  • DOI: https://doi.org/10.1007/978-3-319-94571-2_6

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-94570-5

  • Online ISBN: 978-3-319-94571-2

  • eBook Packages: MedicineMedicine (R0)

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