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Pediatric Radiology

, Volume 36, Issue 1, pp 71–75 | Cite as

MRI assessment of bronchial compression in absent pulmonary valve syndrome and review of the syndrome

  • Benjamin H. TaraginEmail author
  • Walter E. Berdon
  • Beth HTMLz
Historical Perspective

Abstract

Absent pulmonary valve syndrome (APVS) is a rare cardiac malformation with massive pulmonary insufficiency that presents with short-term and long-term respiratory problems secondary to severe bronchial compression from enlarged central and hilar pulmonary arteries. Association with chromosome 22.Q11 deletions and DiGeorge syndrome is common. This historical review illustrates the airway disease with emphasis on assessment of the bronchial compression in patients with persistent respiratory difficulties post-valvular repair. Cases that had MRI for cardiac assessment are used to illustrate the pattern of airway disease.

Keywords

Absent pulmonary valve syndrome Airway abnormalities MRI 

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Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Benjamin H. Taragin
    • 1
    Email author
  • Walter E. Berdon
    • 1
  • Beth HTMLz
    • 2
  1. 1.Department of RadiologyChildren’s Hospital of New YorkNew YorkUSA
  2. 2.Department of CardiologyChildren’s Hospital of New YorkNew YorkUSA

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