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Pediatric Radiology

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01.01.2006 | Historical Perspective

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

verfasst von: Benjamin H. Taragin, Walter E. Berdon, Beth HTMLz

Erschienen in: Pediatric Radiology | Ausgabe 1/2006

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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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Titel: MRI assessment of bronchial compression in absent pulmonary valve syndrome and review of the syndrome
verfasst von: Benjamin H. Taragin
Walter E. Berdon
Beth HTMLz
Publikationsdatum: 01.01.2006
Verlag: Springer-Verlag
Erschienen in: Pediatric Radiology / Ausgabe 1/2006
Print ISSN: 0301-0449
Elektronische ISSN: 1432-1998
DOI: https://doi.org/10.1007/s00247-005-0018-9

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