Pediatric Cardiology

, Volume 27, Issue 5, pp 628–632 | Cite as

Malalignment of the Septum Primum with Abnormally Positioned Left Atrial Appendage

Unusual Combination in a Shone’s Heart
  • Gregory H. KurioEmail author
  • Rajiv Gulati
  • David P. Bichell
  • James C. Perry


This case discusses a Shone’s variant with small left ventricle and mitral valve, bicuspid aortic valve, coarctation of the aorta, an unusual arrangement of atrial appendages, and partially anomalous pulmonary venous drainage due to a deviated atrial septum. The left atrial appendage could be directly entered from the right atrium at catheterization, but it was positioned behind the great arteries. The right atrial appendage was normal in origin and orientation. The implications of this anatomy in fetal cardiac development are reviewed.


Appendage Juxtaposed Shone’s Embryology Septum primum 



We thank Richard Van Praagh, MD, for his insights and comments.


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Copyright information

© Springer Science+Business Media, Inc. 2006

Authors and Affiliations

  • Gregory H. Kurio
    • 1
    Email author
  • Rajiv Gulati
    • 2
  • David P. Bichell
    • 3
  • James C. Perry
    • 4
  1. 1.Division of Pediatric CardiologyChildren’s Hospital Los AngelesLos AngelesUSA
  2. 2.Division of Cardiothoracic SurgeryChildren’s Hospital & Health CenterSan DiegoUSA
  3. 3.Pediatric and Congenital Cardiac SurgeryUniversity of Chicago, Comer Children’s HospitalChicago
  4. 4.Section of Pediatric CardiologyYale University Medical CenterNew HavenUSA

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