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01-01-2006 | Historical Perspective

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

Authors: Benjamin H. Taragin, Walter E. Berdon, Beth HTMLz

Published in: Pediatric Radiology | Issue 1/2006

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.

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Title: MRI assessment of bronchial compression in absent pulmonary valve syndrome and review of the syndrome
Authors: Benjamin H. Taragin
Walter E. Berdon
Beth HTMLz
Publication date: 01-01-2006
Publisher: Springer-Verlag
Published in: Pediatric Radiology / Issue 1/2006
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI: https://doi.org/10.1007/s00247-005-0018-9

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

Abstract

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