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Duplicated left pulmonary artery: an unknown disease? Three case reports and review of the literature

Published online by Cambridge University Press:  05 March 2015

Valentina Giudici ,
Mazyar Kanani ,
Nagarajan Muthialu ,
Michelle Carr ,
Alistair D. Calder ,
Catherine M. Owens ,
Andrew C. Cook  and
Jan Marek
Valentina Giudici*
Affiliation:
Great Ormond Street Hospital for Children, London, United Kingdom Paediatric Department Hospital Luigi Sacco, University of Milan, Milan, Italy
Mazyar Kanani
Affiliation:
Great Ormond Street Hospital for Children, London, United Kingdom
Nagarajan Muthialu
Affiliation:
Great Ormond Street Hospital for Children, London, United Kingdom
Michelle Carr
Affiliation:
Great Ormond Street Hospital for Children, London, United Kingdom
Alistair D. Calder
Affiliation:
Great Ormond Street Hospital for Children, London, United Kingdom
Catherine M. Owens
Affiliation:
Great Ormond Street Hospital for Children, London, United Kingdom
Andrew C. Cook
Affiliation:
Institute of Cardiovascular Sciences, University College London, London, United Kingdom
Jan Marek
Affiliation:
Great Ormond Street Hospital for Children, London, United Kingdom Institute of Cardiovascular Sciences, University College London, London, United Kingdom
*
Correspondence to: Dr V. Giudici, Paediatric Registrar, Paediatric Department Hospital Luigi Sacco, University of Milan, Via G.B. Grassi 74, 200174 Milan, Italy. Tel: +39 0239042253; Fax: +39 0239042254; E-mail: [email protected]
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Abstract

We report three cases of an abnormal finding of duplicated left pulmonary artery: two of these occurring in children with Kabuki syndrome and configuring the setting of a pseudo-pulmonary sling without any clinical or cardiac cross-sectional evidence of tracheal compression. The other case instead represents duplicated left pulmonary artery with pulmonary sling caused by the retro-tracheal course of the lower left pulmonary artery associated with “Christmas Tree” arrangement of the tracheo-bronchial system.

In both patients with pseudo-pulmonary sling and Kabuki syndrome, the abnormal finding was incidental during echocardiographic examination and neither of the patients required surgical repair for the condition. To the best of our knowledge, they represent the third and fourth cases in which such an anomaly of the pulmonary artery branches not forming a sling is seen in association with Kabuki syndrome. Another case represents our second experience and the second case reported in literature with duplicated left pulmonary artery in the setting of a complex tracheal anatomy. In this symptomatic patient, surgical repair of atrial septal defect and relief of the vascular ring were indicated, and the surgical repair was performed successfully at the age of 3 years.

Keywords

Kabuki syndromeaccessory left pulmonary arteryanomalous left pulmonary arterypulmonary artery sling
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 2 , February 2016 , pp. 340 - 346
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Copyright
© Cambridge University Press 2015 

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